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Acute B Lymphoblastic Leukemia (B-ALL)
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Definition
Acute lymphoblastic leukemia (ALL) is a clonal expansion of the lymphoid blasts in bone
marrow, blood or other tissues. ALL can be either T or B lineage (see T ALL) . Pre-B ALL is the
proliferation of the blasts of the B lineage.
Sample Cases
Clickhere for instructions on how to download the free FCS Express Reader to view and manipulate the sample cases.
Epidemiology
The incidence of acute leukemia is approximately 4 cases per 100,000 per year. 30% of these are
ALL. ALL is generally seen in children 75% of cases are usually in children under 6 years of
age. There are approximately 3200 cases of ALL in the United States per year; 80-85% of theseare precursor B-ALL, the others areprecursor T-ALL. The overall cure rate in children is 85%,
and about 50% of adults have long-term disease-free survival.
Possible causes
Similar to AML, possible factors that have been associated with ALL are viruses, radiation,
cytotoxic chemotherapy and benzene exposure. Exposure to the atomic bomb of WWII increased
Case Name(click on case name to
open)
Comments Size
ALL-1
pre-B All
5 Mb
ALL-2
pre-B ALL with myeloid Antigens
7 Mb
ALL-3
pre B ALL
2.86 Mb
case 22 pre B ALLThis case was kindly provided by the ASCP Press. It is part ofFlow Cytometry in Clinical Diagnosisby
John Carey, Phil McCoy and David Keren.
7.54 MB
ALL case pre B ALL case submitted by UTMC. Compare withNormal PB. 2.8 MB
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the incidence of all leukemias including ALL. There is also evidence that may suggest a genetic
factor in some cases.
Morphology
Lymphoblasts (T or B) vary in size from similar to that of a mature lymphocyte to the size of aneutrophil. These blasts have scant to moderate basophillic cytoplasm with occassional
azurophillic granules (T ALL). The nuclear chromatin can be fine to clumped, with occasionally
prominant nucleoli.
Typical morphology of B lymphoblasts
WHO classification
The recent WHO International panel on ALL recommends that the FAB morphologic
classification (L1, L2, L3) be abandoned, since this classification has no clinical or prognostic
relevance. It instead advocates the use of the immunophenotypic classification. There are 2 mainimmunologic types: pre-B cell andpre-T cell. The mature B-cell ALL (L3) is now classified as
Burkitt leukemia/lymphoma. Subtyping helps determine the prognosis and most appropriate
treatment in treating ALL.
Immunophenotyping
Blasts in pre B ALL can be initially identified using a SSC vs CD45 plot. These blasts have low
SSC (many times smaller than normal lymphocytes) and dim to negative CD45. This differs from
blasts in AML where the SSC is generally higher.
http://clinicalflow.com/Cases/Case_List/Acute_Lymphoblastic_Leukemia_(ALL)_-_General_Information/Acute_T_Lymphoblastic_Leukemia%2F%2FLymphoma_(T_ALL)http://clinicalflow.com/Cases/Case_List/Acute_Lymphoblastic_Leukemia_(ALL)_-_General_Information/Acute_T_Lymphoblastic_Leukemia%2F%2FLymphoma_(T_ALL)http://clinicalflow.com/Cases/Case_List/Mature_B-Cell_Neoplasms_(general)/Burkitt's_Lymphoma_(BL)http://clinicalflow.com/Cases/Case_List/Acute_Myelogenous_Leukemia_(AML)_not_otherwise_categorized_-_General_Informationhttp://clinicalflow.com/Cases/Case_List/Acute_Lymphoblastic_Leukemia_(ALL)_-_General_Information/Acute_T_Lymphoblastic_Leukemia%2F%2FLymphoma_(T_ALL)http://clinicalflow.com/Cases/Case_List/Acute_Lymphoblastic_Leukemia_(ALL)_-_General_Information/Acute_T_Lymphoblastic_Leukemia%2F%2FLymphoma_(T_ALL)http://clinicalflow.com/Cases/Case_List/Mature_B-Cell_Neoplasms_(general)/Burkitt's_Lymphoma_(BL)http://clinicalflow.com/Cases/Case_List/Acute_Myelogenous_Leukemia_(AML)_not_otherwise_categorized_-_General_Information8/3/2019 Acute B Lymphoblastic Leukemia
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Example peripheral blood with CD45 negative Blymphoblasts and characteristically small (low SSC)
Example bone marrow with CD45 dim B lymphobla
Once the blasts are identified and gated, the following markers are useful in classification of pre
B ALL. Included are marking prevalence:
Marker Prevalence
CD10 89%
CD13 5%CD19 100%
CD20 24%
CD22 69%
CD33 31%
CD34 76%
CD45 (bright) 2%
CD45 (moderate) 33%
CD45 (dim) 36%
CD45 (negative) 29%
CD56 36%
CD79a 88%
CD117 0%
cytoplasmic IgM 22%
HLA Dr 98%
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TdT 91%
Example Dot plots in pre B ALL
pre B ALL cellscharacteristically coexpress
CD10 and CD19. In addition,
CD20 and CD23 (other B cellmarkers) are not expressed.
Expression of CD34 and TdTindicates immaturity and is
characteristic in pre B ALL.
cytoplasmic expression ofCD79 confirms B cell lineage.
CD38 is an expression ofimmaturity. The ploidy as seen
by the DNA Histogram,
indicates a hypodiploidy(DI=0.53) indicating poor
prognosis.
The phenotype of the blasts is an idependent prognostic parameter. B-ALL is subdivided into:
early pre B-ALL: TdT+, CD19+, CD10-
common ALL: CD19+, CD10+/CALLA+
pre B ALL: CD10+/-, CD19+, HLA Dr+, cytoplasmic IgM+
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mature B ALL: CD10+, CD19+, CD20+, CD22+, surface IgM+
Other relevant tests
Genetics: Some cytogenetic subtypes have a worse prognosis than others. These include:
A translocation between chromosomes 9 and 22, known as the Philadelphia chromosome,
occurs in about 20% of adult and 5% in pediatric cases of ALL.
A translocation between chromosomes 4 and 11 occurs in about 4% of cases and is most
common in infants under 12 months.
Not all translocations of chromosomes carry a poorer prognosis. Some translocations arerelatively favorable. For example, Hyperdiploidy (>50 chromosomes) is a good
prognostic factor.
Flow Diagnosis
To diagnose B ALL: CD45 downregulated, CD19+, TdT+, CD10+, surface light chainsnegative.
References
1. World Health Organization Classification of Tumors. Tumors of Haematopoetic and Lymphoid
Tissue. Jaffe, E., Harriss, N., Stein, H., Vardiman, J. IARC Press 2001
2. Flow Cytometry in Neoplastic Hematology-Morphologic-Immunophenotypic Correlation.Gorczyca W., Informa Healthcare 2007
3.2006 Bethesda International Consensus Recommendations on Immunophenotypic Analysis ofHematolymphoid Neoplasia by Flow Cytometry. Cytometry Part B, 2007
4. Clinical Flow Cytometric Analysis of Hematolymphoid Cells; Approved Guideline - SecondEdition H43-A2 Clinical and Laboratory Standards Institute (CLSI) 2007
Contributors to this page
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ALL UTMC (1).fey
Pre-B cell ALL submitted by UTMC.2.77 MB23:04, 10 Apr 2009 Tanya TolmachoffActions
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pre-B ALL4.97 MB19:30, 4 Apr 2008 David Novo Actions
B-ALL2.feypre-B ALL with myeloid Antigens
7.08 MB19:30, 4 Apr 2008 David Novo Actions
B-ALL3.fey
pre-B ALL2.86 MB19:30, 4 Apr 2008 David Novo Actions
case_22.fey
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Acute lymphoblastic leukemia
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From Wikipedia, the free encyclopedia
Acute lymphoblastic leukemia (ALL)Classification and external resources
ICD-10 C91.0
ICD-9 204.0
ICD-O: M9835/3
DiseasesDB 195
eMedicine med/3146ped/2587
MeSH D054198
Acute lymphoblastic leukemias (ALL), is a form ofleukemia, orcancer of the white blood
cells characterized by excess lymphoblasts.
Malignant, immature white blood cellscontinuously multiply and are overproduced in thebonemarrow. ALL causes damage and death by crowding out normal cells in the bone marrow, and
by spreading (infiltrating) to other organs. ALL is most common in childhood with a peak
incidence at 25 years of age, and another peak in old age. The overall cure rate in children isabout 80%, and about 45%-60% of adults have long-term disease-free survival. [1]
Acute refers to the relatively short time course of the disease (being fatal in as little as a few
weeks if left untreated) to differentiate it from the very different disease ofChronic Lymphocytic
Leukemia which has a potential time course of many years. It is interchangeably referred to asLymphocytic or Lymphoblastic. This refers to the cells that are involved, which if they were
normal would be referred to aslymphocytes but are seen in this disease in a relatively immature(also termed 'blast') state.
Symptoms The signs and symptoms of ALL are variable but follow from bone marrowreplacement and/or organ infiltration.
Generalized weakness and fatigue
Anemia
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Frequent or unexplained [fever] and [infection]
Weight loss and/or loss of appetite
Excessive and unexplained bruising
Bone pain, joint pain (caused by the spread of "blast" cells to the surface of the bone or
into the joint from the marrow cavity)
Breathlessness Enlarged lymph nodes,liver and/or spleen
Pitting edema (swelling) in the lower limbs and/or abdomen
Petechia, which are tiny red spots or lines in the skin due to lowplatelet levels
The signs and symptoms of ALL result from the lack of normal and healthy blood cells becausethey are crowded out by malignant and immature leukocytes (white blood cells). Therefore,
people with ALL experience symptoms from malfunctioning of their erythrocytes (red blood
cells), leukocytes, and platelets. Laboratory tests which might show abnormalities include bloodcount tests, renal function tests, electrolyte tests and liver enzyme tests.
Diagnosis
Diagnosing ALL begins with a medical history,physical examination,complete blood count, and
blood smears. Because the symptoms are so general, many other diseases with similar symptomsmust be excluded. Typically, the higher the white blood cell count, the worse the prognosis.[2]
Blast cells are seen onblood smearin majority of cases (blast cells are precursors (stem cells) to
all immune cell lines). Abone marrow biopsy is conclusive proof of ALL.[3] A lumbar puncture
(also known as a spinal tap) will tell if the spinal column andbrainhas been invaded.
Pathological examination, cytogenetics(particularly the presence ofPhiladelphia chromosome)andimmunophenotyping, establish whether the Myeloblastic (neutrophils, eosinophils or
basophils) or Lymphoblastic (B lymphocytes orT lymphocytes) cells are the problem. RNAtesting can establish how aggressive the disease is; different mutations have been associated with
shorter or longer survival. Immunohistochemical testing may reveal TdT or CALLA antigens on
the surface of leukemic cells. TdT is a protein expressed early in the development of pre-T and
pre-B cells while CALLA is an antigen found in 80% of ALL cases and also in the "blast crisis"ofCML.
Medical imaging (such as ultrasoundorCT scanning) can find invasion of otherorgans
commonly the lung, liver, spleen, lymph nodes, brain, kidneys and reproductive organs.[4]
[edit] Pathophysiology
In general, cancer is caused by damage to DNA that leads to uncontrolled cellular growth andspread throughout the body, either by increasing chemical signals that cause growth, or
interrupting chemical signals that control growth. Damage can be caused through the formation
offusion genes, as well as the dysregulation of aproto-oncogene via juxtaposition of it to the
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promoter of another gene, e.g. theT-cell receptorgene. This damage may be caused by
environmental factors such as chemicals, drugs or radiation.
ALL is associated with exposure toradiationand chemicals in animals and humans. Theassociation of radiation and leukemia in humans has been clearly established in studies of
victims of the Chernobyl nuclear reactor andatom bombs in Hiroshima andNagasaki. Inanimals, exposure tobenzeneand other chemicals can cause leukemia. Epidemiological studies
have associated leukemia with workplace exposure to chemicals, but these studies are not asconclusive. Some evidence suggests that secondary leukemia can develop in individuals who are
treated for other cancers with radiation and chemotherapy as a result of that treatment. [5]
[edit] Cytogenetics
Cytogenetic translocations associated with specific molecular genetic abnormalities in ALL
Cytogenetic
translocation
Molecular genetic
abnormality%
cryptic t(12;21) TEL-AML1 fusion[6]25.4%[
7]
t(1;19)(q23;p13) E2A-PBX (PBX1) fusion[8] 4.8%[7]
t(9;22)(q34;q11) BCR-ABL fusion(P185)[9] 1.6%[7]
t(4;11)(q21;q23) MLL-AF4 fusion[10] 1.6%[7]
t(8;14)(q24;q32) IGH-MYC fusion[11]
t(11;14)(p13;q11) TCR-RBTN2 fusion [12]
12:21 is the most common translocation and portends a good prognosis. 4:11 is the most
common in children under 12 months and portends a poor prognosis.
[edit] Prognosis
The survival rate has improved from zero four decades ago, to 20-75 percent currently, largelydue to clinical trials on new chemotherapeutic agents and improvements in stem cell
transplantation(SCT) technology.
Five-year survival rates evaluate older, not current, treatments. New drugs, and matching
treatment to the genetic characteristics of the blast cells, may improve those rates. The prognosisfor ALL differs between individuals depending on a variety of factors:
Sex: females tend to fare better than males.
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Ethnicity: Caucasians are more likely to develop acute leukemia than African-Americans, Asians and Hispanics and tend to have a better prognosis thannon-Caucasians.
Age at diagnosis: children between 110 years of age are most likely todevelop ALL and to be cured of it. Cases in older patients are more likely toresult from chromosomal abnormalities (e.g. the Philadelphia chromosome)
that make treatment more difficult and prognoses poorer. White blood cell count at diagnosis of less than 50,000/l Cancer spread into the Central nervous system (brain or spinal cord) has
worse outcomes. Morphological, immunological, and genetic subtypes Patient's response to initial treatment Genetic disorders such as Down's Syndrome
Cytogenetics, the study of characteristic large changes in the chromosomes ofcancer cells, is animportant predictor of outcome.[13]
Some cytogenetic subtypes have a worse prognosis than others. These include:
A translocation between chromosomes 9 and 22, known as the Philadelphiachromosome, occurs in about 20% of adult and 5% in pediatric cases of ALL.
A translocation between chromosomes 4 and 11 occurs in about 4% of casesand is most common in infants under 12 months.
Not all translocations of chromosomes carry a poorer prognosis. Sometranslocations are relatively favorable. For example, Hyperdiploidy (>50chromosomes) is a good prognostic factor.
Genome-wide copy number changes can be assessed by conventionalcytogenetics or virtual karyotyping. SNP array virtual karyotyping can detectcopy number changes and LOH status, while arrayCGH can detect only copy
number changes. Copy neutral LOH (acquired uniparental disomy) has beenreported at key loci in ALL, such as CDKN2A gene, which have prognosticsignificance.[14][15][16] SNP array virtual karyotyping can readily detect copyneutral LOH. Array CGH, FISH, and conventional cytogenetics cannot detectcopy neutral LOH.
Cytogenetic changeRisk
category
Philadelphia chromosomePoor
prognosis
t(4;11)(q21;q23)Poorprognosis
t(8;14)(q24.1;q32)Poor
prognosis
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Complex karyotype (more than four
abnormalities)
Poor
prognosis
Low hypodiploidy or near triploidyPoor
prognosis
High hyperdiploidy (specifically, trisomy
4, 10, 17)
Good
prognosis
del(9p)Good
prognosis
Correlation of prognosis with bone marrow cytogenetic finding in acute lymphoblastic
leukemia
Prognosi
sCytogenetic findings
Favorable Hyperdiploidy > 50 ; t (12;21)
Intermedia
te
Hyperdiploidy 47 -50; Normal(diploidy); del (6q);
Rearrangements of 8q24
Unfavorabl
e
Hypodiploidy-near haploidy; Near tetraploidy; del (17p); t
(9;22); t (11q23)
Unclassified ALL is considered to have an intermediate prognosis.[17]
[edit] Classification
As ALL is not a solid tumour, the TNM notation as used in solid cancers is of little use.
[edit] The FAB classification
Subtyping of the various forms of ALL used to be done according to the French-American-
British (FAB) classification,[18] which was used for all acute leukemias (including acute
myelogenous leukemia, AML).
ALL-L1: small uniform cells ALL-L2: large varied cells ALL-L3: large varied cells with vacuoles (bubble-like features)
Each subtype is then further classified by determining the surface markers of the abnormallymphocytes, called immunophenotyping. There are 2 main immunologic types: pre-B cell and
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pre-T cell. The mature B-cell ALL (L3) is now classified as Burkitt's lymphoma/leukemia.
Subtyping helps determine the prognosis and most appropriate treatment in treating ALL.
[edit] WHO proposed classification of acute lymphoblastic
leukemia
The recent WHO International panel on ALL recommends that the FAB classification be
abandoned, since the morphological classification has no clinical or prognostic relevance. It
instead advocates the use of the immunophenotypic classification mentioned below.
1- Acute lymphoblastic leukemia/lymphoma Synonyms:Former Fab L1/L2
i. Precursor B acute lymphoblastic leukemia/lymphoma. Cytogeneticsubtypes:[19]
o t(12;21)(p12,q22) TEL/AML-1o t(1;19)(q23;p13) PBX/E2A
o t(9;22)(q34;q11) ABL/BCRo T(V,11)(V;q23) V/MLL
ii. Precursor T acute lymphoblastic leukemia/lymphoma
2- Burkitt's leukemia/lymphoma Synonyms:Former FAB L3
3- Biphenotypic acute leukemia
[edit] Variant Features of ALL
1- Acute lymphoblastic leukemia with cytoplasmic granules 2- Aplastic presentation of ALL 3- Acute lymphoblastic leukemia with eosinophilia 4- Relapse of lymphoblastic leukemia 5- Secondary ALL
[edit] Immunophenotyping in the diagnosis and classification
of ALL
The use of a TdT assay and a panel of monoclonal antibodies (MoAbs) to T cell and B cellassociated antigens will identify almost all cases of ALL.
Immunophenotypic categories of acute lymphoblastic leukemia (ALL)
TypesFAB
Class
Td
t
T cell associate
antigen
B cell associate
antigen
c
Ig
s
Ig
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Precursor
BL1,L2 + - +
-/
+-
Precursor
TL1,L2 + + - - -
B-cell L3 - - + - +
[edit] Treatment
The earlier acute lymphocytic leukemia is detected, the more effective the treatment. The aim isto induce a lasting remission, defined as the absence of detectable cancer cells in the body
(usually less than 5% blast cells on the bone marrow).
Treatment for acute leukemia can includechemotherapy,steroids, radiation therapy, intensivecombined treatments (includingbone marroworstem celltransplants), and growth factors.[20]
[edit] Chemotherapy
Chemotherapy is the initial treatment of choice. Most ALL patients will receive a combination ofdifferent treatments. There are no surgical options, due to the body-wide distribution of the
malignant cells. In general, cytotoxic chemotherapy for ALL combines multiple antileukemic
drugs in various combinations. Chemotherapy for ALL consists of three phases: remission
induction, intensification, and maintenance therapy.
Phase Description Agents
Remission induction The aim of remission induction is
to rapidly kill most tumor cells
and get the patient into
remission. This is defined as the
presence of less than 5%
leukemic blasts in the bone
marrow, normal blood cells and
absence of tumor cells from
blood, and absence of other signs
and symptoms of the disease.
CNS prophylaxis should beginduring this phase of treatment
and continue during the
consolidation/intensification
period. The rationale is based on
the presence of CNS involvement
in 10%-40% of adult patients at
Combination of
Prednisolone or
dexamethasone,
vincristine, asparaginase
(better tolerance in
pediatric patients), and
daunorubicin (used in
Adult ALL) is used to
induce remission.
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diagnosis.
Consolidation/Intensific
ation
Intensification uses high doses of
intravenous multidrug
chemotherapy to further reducetumor burden. Since ALL cells
sometimes penetrate the Central
Nervous System (CNS), most
protocols include delivery of
chemotherapy into the CNS fluid
(termed intrathecal
chemotherapy). Some centers
deliver the drug through
Ommaya reservoir (a device
surgically placed under the scalpand used to deliver drugs to the
CNS fluid and to extract CNS fluid
for various tests). Other centers
would perform multiple lumbar
punctures as needed for testing
and treatment delivery.
Typical intensification
protocols use vincristine,
cyclophosphamide,
cytarabine, daunorubicin,etoposide, thioguanine or
mercaptopurine given as
blocks in different
combinations. For CNS
protection, intrathecal
methotrexate or
cytarabine is usually used
combined with or without
cranio-spinal irradiation
(the use of radiation
therapy to the head and
spine). Central nervous
system relapse is treated
with intrathecal
administration of
hydrocortisone,
methotrexate, and
cytarabine.
Maintenance therapy
The aim of maintenance therapy
is to kill any residual cell that was
not killed by remission induction,
and intensification regimens.
Although such cells are few, they
will cause relapse if not
eradicated.
For this purpose, daily oral
mercaptopurine, once
weekly oral methotrexate,
once monthly 5-day
course of intravenous
vincristine and oral
corticosteroids are usually
used. The length of
maintenance therapy is 3
years for boys, 2 years for
girls and adults.[21]
As the chemotherapy regimens can be intensive and protracted (often about 2 years in case of theGMALL UKALL, HyperCVAD or CALGB protocols; for ALL about 3 years, 2 months for
males on COG protocols; 2 years, 2 months for females- longer for males as testicles are a
potential reservoir), many patients have anintravenous catheter inserted into a large vein (termed
acentral venous catheteror a Hickman line), or a Portacath, a cone-shaped port with a siliconenose that is surgically planted under the skin, usually near the collar bone, and the most effective
product available, due to low infection risks and the long-term viability of a portacath.
http://en.wikipedia.org/wiki/CNShttp://en.wikipedia.org/w/index.php?title=Intrathecal_chemotherapy&action=edit&redlink=1http://en.wikipedia.org/w/index.php?title=Intrathecal_chemotherapy&action=edit&redlink=1http://en.wikipedia.org/wiki/Ommaya_reservoirhttp://en.wikipedia.org/wiki/Cyclophosphamidehttp://en.wikipedia.org/wiki/Cytarabinehttp://en.wikipedia.org/wiki/Etoposidehttp://en.wikipedia.org/wiki/Thioguaninehttp://en.wikipedia.org/wiki/Mercaptopurinehttp://en.wikipedia.org/wiki/Hydrocortisonehttp://en.wikipedia.org/wiki/Mercaptopurinehttp://en.wikipedia.org/wiki/Methotrexatehttp://en.wikipedia.org/wiki/Vincristinehttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-20http://en.wikipedia.org/wiki/Chemotherapy_regimenshttp://en.wikipedia.org/wiki/Intravenoushttp://en.wikipedia.org/wiki/Intravenoushttp://en.wikipedia.org/wiki/Central_venous_catheterhttp://en.wikipedia.org/wiki/Central_venous_catheterhttp://en.wikipedia.org/wiki/Hickman_linehttp://en.wikipedia.org/wiki/Portacathhttp://en.wikipedia.org/wiki/CNShttp://en.wikipedia.org/w/index.php?title=Intrathecal_chemotherapy&action=edit&redlink=1http://en.wikipedia.org/w/index.php?title=Intrathecal_chemotherapy&action=edit&redlink=1http://en.wikipedia.org/wiki/Ommaya_reservoirhttp://en.wikipedia.org/wiki/Cyclophosphamidehttp://en.wikipedia.org/wiki/Cytarabinehttp://en.wikipedia.org/wiki/Etoposidehttp://en.wikipedia.org/wiki/Thioguaninehttp://en.wikipedia.org/wiki/Mercaptopurinehttp://en.wikipedia.org/wiki/Hydrocortisonehttp://en.wikipedia.org/wiki/Mercaptopurinehttp://en.wikipedia.org/wiki/Methotrexatehttp://en.wikipedia.org/wiki/Vincristinehttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-20http://en.wikipedia.org/wiki/Chemotherapy_regimenshttp://en.wikipedia.org/wiki/Intravenoushttp://en.wikipedia.org/wiki/Central_venous_catheterhttp://en.wikipedia.org/wiki/Hickman_linehttp://en.wikipedia.org/wiki/Portacath8/3/2019 Acute B Lymphoblastic Leukemia
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[edit] Radiation therapy
Radiation therapy (or radiotherapy) is used on painful bony areas, in high disease burdens, or aspart of the preparations for abone marrow transplant (total body irradiation). Radiation in the
form of whole brain radiation is also used for central nervous system prophylaxis, to prevent
recurrence of leukemia in the brain. Whole brain prophylaxis radiation used to be a commonmethod in treatment of childrens ALL. Recent studies showed that CNS chemotherapy provided
results as favorable but with less developmental side effects. As a result, the use of whole brain
radiation has been more limited. Most specialists in adult leukemia have abandoned the use o
[edit] Epidemiology
In the US, the incidence of ALL is roughly 6000 new cases per year (as of 2009),[22]orapproximately 1 in 50,000. ALL accounts for approximately 70 percent of all childhood
leukemia cases (ages 0 to 19 years), making it the most common type of childhood cancer.[22] It
has a peak incident rate of 25 years old, decreasing in incidence with increasing age before
increasing again at around 50 years old. ALL is slightly more common in males than females.There is an increased incidence in people with Down Syndrome, Fanconi anemia,Bloom
syndrome,Ataxia telangiectasia,X-linked agammaglobulinemiaandSevere combined
immunodeficiency.
[edit] Additional images
acute lymphoblastic leukemia (ALL), peripheral blood of a child, Pappenheim stain,magnification x100
bone marrow smear (large magnification) from a patient with acute lymphoblastic
leukemia
http://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit§ion=12http://en.wikipedia.org/wiki/Radiation_therapyhttp://en.wikipedia.org/wiki/Bone_marrow_transplanthttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit§ion=13http://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Childhood_leukemiahttp://en.wikipedia.org/wiki/Childhood_leukemiahttp://en.wikipedia.org/wiki/Childhood_leukemiahttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Down_Syndromehttp://en.wikipedia.org/wiki/Fanconi_anemiahttp://en.wikipedia.org/wiki/Fanconi_anemiahttp://en.wikipedia.org/wiki/Bloom_syndromehttp://en.wikipedia.org/wiki/Bloom_syndromehttp://en.wikipedia.org/wiki/Ataxia_telangiectasiahttp://en.wikipedia.org/wiki/Ataxia_telangiectasiahttp://en.wikipedia.org/wiki/X-linked_agammaglobulinemiahttp://en.wikipedia.org/wiki/X-linked_agammaglobulinemiahttp://en.wikipedia.org/wiki/X-linked_agammaglobulinemiahttp://en.wikipedia.org/wiki/Severe_combined_immunodeficiencyhttp://en.wikipedia.org/wiki/Severe_combined_immunodeficiencyhttp://en.wikipedia.org/wiki/Severe_combined_immunodeficiencyhttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit§ion=14http://en.wikipedia.org/wiki/File:ALL-KM-2.jpghttp://en.wikipedia.org/wiki/File:ALL_-_Peripherial_Blood_-_Diagnosis_-_01.jpghttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit§ion=12http://en.wikipedia.org/wiki/Radiation_therapyhttp://en.wikipedia.org/wiki/Bone_marrow_transplanthttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit§ion=13http://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Childhood_leukemiahttp://en.wikipedia.org/wiki/Childhood_leukemiahttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Down_Syndromehttp://en.wikipedia.org/wiki/Fanconi_anemiahttp://en.wikipedia.org/wiki/Bloom_syndromehttp://en.wikipedia.org/wiki/Bloom_syndromehttp://en.wikipedia.org/wiki/Ataxia_telangiectasiahttp://en.wikipedia.org/wiki/X-linked_agammaglobulinemiahttp://en.wikipedia.org/wiki/Severe_combined_immunodeficiencyhttp://en.wikipedia.org/wiki/Severe_combined_immunodeficiencyhttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit§ion=148/3/2019 Acute B Lymphoblastic Leukemia
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bone marrow smear from a patient with acute lymphoblastic leukemia
LeukemiaNote: This section has health/medical information. It was not written by a health care
professional. The medical references are:
Childhood Leukemias, edited by Ching-Hon Pui, 1999, Cambridge UniversityPress
NCI web site sections on the different types of childhood leukemias, accessed2007
Childhood Leukemia: A Guide for Families, Friends, and Caregivers, 4th ed.,by Nancy Keene, 2010.
On this site: Warning Signs of Childhood Cancer: Leukemia
Leukemia is a cancer of the bone marrow, the spongy center of the bones that makes blood cells.In leukemia, abnormal white blood cells divide out of control and crowd out the normal cells in
the bloodstream. The abnormal white blood cells are not mature, and therefore cannot carry out
their infection-fighting function in the blood. These cells crowd out healthy white blood cells, as
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well as the red blood cells which carry oxygen to the body and the platelets which cause the
blood to clot.
What are the different types of childhood leukemia?
The most common type of leukemia in children is acute lymphocytic (or lymphoblastic)leukemia orALL, which is further characterized as pre-B, B, or T-cell ALL. Childhood acute
myeloid leukemia orAML is less common. "Acute" means that the diseases progress rapidly.
The chronic forms of these leukemias, CLL and CML respectively, are seen almost solely in
adults. In general, acute leukemias are most prevalent in children and are therefore often referredto as "childhood leukemias".
ALL: acute lymphocytic leukemia (pre-B, B, or T-cell). ALL NCI PDQ AML: acute myeloid leukemia AML NCI PDQ
About 5% of childhood leukemias are distinct types of chronic myeloid leukemias. Juvenile
myelomonocytic leukemia (JMML,NCI PDQ) occurs primarily in children aged 2 or under.Acute promyelocytic leukemia (APL,NCI PDQ) is a distinct subtype of AML. A good startingpoint for research into these and other less common childhood leukemias is on the cancer.gov
myeloid leukemias page.
A rare type of leukemia in children is Anaplastic Large Cell Lymphoblastic Leukemia, or
ALCL. Follow this link for more information on pediatric ALCL.
Types of white blood cells
White blood cells - the blood cells that grow out of control in leukemia - are the cells that fight
infection. Blood contains three types of cells:
red blood cells (RBCs, or erythrocytes): these cells carry oxygen to all partsof your body and give the blood its red color
platelets (thrombocytes): these cells cause your blood to clot when youbleed
white blood cells (WBCs, or leukocytes): these cells defend your body frominfections
All blood cells originate in the bone marrow. In fact, they all develop from one special type of
cell, called a stem cell.
White blood cells come in several types, including:
granulocytes: fight bacteria by surrounding them and "eating" them. monocytes: fight germs, but aren't as specific as granulocytes. B-lymphocytes: these cells attach antibodies on germs (or anything they
don't think belongs) with antibodies, which in turn signal other WBCs to getthe tagged germ.
http://www.cancer.gov/cancertopics/pdq/treatment/childALL/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/HealthProfessional/page10http://www.cancer.gov/cancertopics/pdq/treatment/childAML/HealthProfessional/page7http://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.ped-onc.org/diseases/ALCL.htmlhttp://www.cancer.gov/cancertopics/pdq/treatment/childALL/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/HealthProfessional/page10http://www.cancer.gov/cancertopics/pdq/treatment/childAML/HealthProfessional/page7http://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.ped-onc.org/diseases/ALCL.html8/3/2019 Acute B Lymphoblastic Leukemia
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T-lymphocytes: these cells signal orders to other WBCs to come to a germ,and they make those other WBCs stay at the battle sight.
In acute lymphocytic leukemias, the B- or T-lymphocytes are growing out of control. In acutemyelogenous leukemias, the granulocytes are growing out of control.
lymphocytic (ALL): uncontrolled growth of B- or T-lymphocytes myelogenous (AML) (granulocytic): uncontrolled growth of granulocytes
In all of the leukemias, immature white cells crowd out the good cells. Since they crowd out thered blood cells, a person with leukemia is anemic, without enough red blood cells to carry the
necessary oxygen or energy to the body. That's why fatigue is a sign of leukemia. The leukemia
cells also crowd out the platelets, so if a person with leukemia is cut, the bleeding does not stopas readily. They also bruise easier. Since the blasts are immature, non-functioning infection
fighting cells, a person with leukemia is easily susceptible to infection.
If you are interested in more information on blood cells, follow the links below for in-depth,
technical information.
University of Virginia's site on blood cells - a good tutorial from the Universityof Virginia.
University of Washington REAL classification of leukemia cells, flow cytometrypanels, diagnosis of acute leukemia, tdt, descriptions of many diagnostictests used in leukemia treatment. From the University of Washington,Department of Laboratory Medicine, Hematopathology Laboratory.
Treatment for childhood leukemias
ALL. The primary treatment for newly diagnosed ALL is combination chemotherapy. Radiationand bone marrow transplantation may be used in some cases. Treatment begins with an intense
treatment called "induction" with a combination of several chemotherapy drugs, usually cytosinearabinoside, vincristine, prednisone, L-asparaginase, and daunorubicin. The goal of induction is
to kill most of the leukemia cells; most patients do not have any leukemic cells in the bone
marrow at the end of induction. (At least, not detectable in a light-microscopical examination ofstained bone marrow smear.) The next phase is called "consolidation" in which a different
combination of drugs is administered, usually methotrexate, cyclophosphamide, cytosine
arabinoside, mercaptopurine, and prednisone. "Maintenance" follows, in which thechemotherapy is lessened to a few of the drugs administered less frequently. Maintenance is
generally well tolerated by the patient. Often a period of maintenance is followed by another
cycle of induction-consolidation, called "re-intensification". Total therapy lasts from two to threeyears. Detailed information on this web site:
ALL clinical trials page and ALL main page on this ped-onc site
AML. In general, newly diagnosed AML is initially treated more aggressively than is ALL.Intensive chemotherapy followed by bone marrow transplantation is becoming the first treatment
chosen, especially when a suitable donor is available. After the intensive chemotherapy and/or
http://www.med-ed.virginia.edu/courses/path/innes/wcd/http://depts.washington.edu/labweb/Divisions/Hema/Flow.htmhttp://www.ped-onc.org/diseases/ALLclintrial.htmlhttp://www.ped-onc.org/diseases/ALL.htmlhttp://www.med-ed.virginia.edu/courses/path/innes/wcd/http://depts.washington.edu/labweb/Divisions/Hema/Flow.htmhttp://www.ped-onc.org/diseases/ALLclintrial.htmlhttp://www.ped-onc.org/diseases/ALL.html8/3/2019 Acute B Lymphoblastic Leukemia
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bone marrow transplant, children with AML do not go on maintenance; studies have shown that
AML children in remission have had as much chemotherapy as their bodies can tolerate, and
additional maintenance chemotherapy does not benefit them.
Chronic myeloid leukemias. As in AML, intensive chemotherapy and/or BMT are generally
employed. Currently (2005), imatinib mesylate (Gleevec) is being studied in clinical trials.Chronic leukemias have three clinical phases: chronic, accelerated, and blast crisis. Prognosis
depends on the clinical phase of the disease.
Relapsed leukemia. Relapse, or recurrence of leukemia, can occur anytime during therapy or
after completion of treatment. Generally, it is more difficult to cure a child after relapse of the
leukemia; relapse during or soon after the completion of treatment is considered less favorable
than relapse a year or several years after treatment. Treatment depends on the site of relapse,whether it is in the bone marrow, central nervous system, testes, or other locations. Aggressive
chemotherapy and radiation treatment, often followed bone marrow transplantation, are used to
treat relapse of childhood leukemia.
New Treatments
What's on the horizon for leukemia treatment? The following organization talks about new
treatments:
Research updates from the Leukemia and Lymphoma Society
The big news (early 2000s) for the treatment of CML (and Ph+ ALL) is STI-571. Brian Druker
(Oregon Health Sciences University in Portland) is the Leukemia and Lymphoma Society doctor
prominent in this research.
Gleevec web site
ALL: New ideas for treatmentbys for ALL are also listed on the Clinical Trials for ALL page.
In 2004, ara G entered the treatment plans for T-cell ALL. Clofarabine (2005) shows promise forrefractory (relapsed) ALL. More information in an essay on your author's private web site:
from sea to ara c
Statistics
Leukemia accounts for approximately 35% of all childhood cancers Approximately 1 in 1000 children will be diagnosed with leukemia by the age
of 19 It is more common in children under the age of 10 The five year survival rate for children diagnosed with leukemia and
subsequently treated is approximately 70% 2500 cases of leukemia are diagnosed per year in the US
http://www.lls.org/http://www.gleevec.com/http://www.ped-onc.org/diseases/ALLclintrial.html#newtreatmentshttp://www.ped-onc.org/diseases/ALLclintrial.html#newtreatmentshttp://www.pfeist.net/ALL/arac/sea2arac7.htmlhttp://www.lls.org/http://www.gleevec.com/http://www.ped-onc.org/diseases/ALLclintrial.html#newtreatmentshttp://www.pfeist.net/ALL/arac/sea2arac7.html8/3/2019 Acute B Lymphoblastic Leukemia
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Ped-Onc Resources for Leukemia
The following ped-onc resource lists have appropriate sections for parents of children with
leukemia:
childhood cancer e-mail lists - the ACOR ALL-kids list and the generalchildhood cancer list, ped-onc, are both appropriate
support organizations - Leukemia and Lymphoma books and printed materials - leukemias and lymphomas (and don't miss the
book for children, I'm Still Me) young people with leukemia - personal home pages Kidz With Leukemia. Computer program for kids. Read my in-depth review for
details.
Links to More Information
The following web sites provide good, general information on lymphomas cancers and theirtreatment.
General Information on Leukemia and Leukemia Research
Leukemia Education Series on the Leukemia and Lymphoma site. A variety ofinformation.
Childhood Leukemia Center. This site is the online version ofChildhoodLeukemia by Nancy Keene (listed above under books).
Cancer.gov site. Excellent. MedLine Plus. Links to a lot of useful information.
The Leukemia and Lymphoma Society. This US society web site providesgood basic information on leukemias. They have booklets to download as pdffiles (or to send for), disease descriptions, treatments, patient services, etc.Especially recommended: NewsLine articles
Leukaemia and Lymphoma Research. Information on leukemia from thisBritish society. There are a lot of "goodies" on this site, links to reviews aswell as good basic information.
Leukemia Links: Granny Barb and Art's. This classic site provides useful andinformative information with great links.
St. Jude's Research Hospital page on Leukemia. This page has information onleukemia and the latest research at St. Jude's.
ALL-KIDS web site, the reference site for the ALL-kids mail list.
JMML Foundation. Excellent resource, specific for JMML, JuvenileMyelomonocytic Leukemia. Founded by parents, covers all aspects of JMML. AML: Caylee's Hope. Good resources for AML, another parent site. Children's Cancer Web. Links and descriptions of leukemia. Excellent. Medicine Online. Information on leukemia and links to more information.
Although this is a dot-com site, it has some good links, and may acquire moregood links in the future. I like the atlas of acute leukemia and the leukemialinks.
http://www.ped-onc.org/cfissues/maillist.htmlhttp://www.ped-onc.org/cfissues/maillist.html#leukemialistshttp://www.ped-onc.org/resources/supportorg.html#leuklymphsupporghttp://www.ped-onc.org/cfissues/books.html#leukemiahttp://www.ped-onc.org/cfissues/books.html#stillmehttp://www.ped-onc.org/hp/leukpages.htmlhttp://www.kidzwithleukemia.com/http://www.ped-onc.org/cfissues/kidz.htmlhttp://www.lls.org/resourcecenter/freeeducationmaterials/#ALLhttp://www.childhoodcancerguides.org/leukemia.htmlhttp://www.cancer.gov/cancerinfo/types/childhoodcancershttp://www.nlm.nih.gov/medlineplus/leukemiachildhood.htmlhttp://www.lls.org/http://www.lls.org/#/resourcecenter/enewsletters/http://www.beatbloodcancers.org/http://www.acor.org/leukemia/http://www.stjude.org/leukemiahttp://www.stjude.org/leukemiahttp://www.all-kids.org/http://www.jmmlfoundation.org/http://www.cayleeshope.com/http://www.cancerindex.org/ccw/guide2l.htmhttp://www.meds.com/leukemia/leukemia.htmlhttp://www.ped-onc.org/cfissues/maillist.htmlhttp://www.ped-onc.org/cfissues/maillist.html#leukemialistshttp://www.ped-onc.org/resources/supportorg.html#leuklymphsupporghttp://www.ped-onc.org/cfissues/books.html#leukemiahttp://www.ped-onc.org/cfissues/books.html#stillmehttp://www.ped-onc.org/hp/leukpages.htmlhttp://www.kidzwithleukemia.com/http://www.ped-onc.org/cfissues/kidz.htmlhttp://www.lls.org/resourcecenter/freeeducationmaterials/#ALLhttp://www.childhoodcancerguides.org/leukemia.htmlhttp://www.cancer.gov/cancerinfo/types/childhoodcancershttp://www.nlm.nih.gov/medlineplus/leukemiachildhood.htmlhttp://www.lls.org/http://www.lls.org/#/resourcecenter/enewsletters/http://www.beatbloodcancers.org/http://www.acor.org/leukemia/http://www.stjude.org/leukemiahttp://www.all-kids.org/http://www.jmmlfoundation.org/http://www.cayleeshope.com/http://www.cancerindex.org/ccw/guide2l.htmhttp://www.meds.com/leukemia/leukemia.html8/3/2019 Acute B Lymphoblastic Leukemia
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National Children's Leukemia Foundation. Provides a range of services, fromhelp in finding donors to referrals to fundraising.
Technical Information
University of Virginia's site on blood cells - a good tutorial from the Universityof Virginia. University of Washington REAL classification of leukemia cells, flow cytometry
panels, diagnosis of acute leukemia, tdt, descriptions of many diagnostictests used in leukemia treatment. From the University of Washington,Department of Laboratory Medicine, Hematopathology Laboratory.
ASCO online, links to abstracts on pediatric lymphoma and leukemia. ASH: American Society of Hematology. Links to the annual meetings and
abstracts and to journals. A good way to follow the latest research inleukemia.
Acute Lymphoblastic Leukemia (ALL)
Acute lymphoblastic leukemia (ALL) is a fast-growing cancer of the white blood cells.
Lymphocytes are a type of white blood cell that the body uses to fight infections. In ALL, thebone marrow makes lots of unformed cells called blasts that normally would develop into
lymphocytes. However, the blasts are abnormal. They do not develop and cannot fight infections.
http://www.leukemiafoundation.org/http://www.leukemiafoundation.org/http://www.leukemiafoundation.org/http://www.med-ed.virginia.edu/courses/path/innes/wcd/http://depts.washington.edu/labweb/Divisions/Hema/Flow.htmhttp://www.asco.org/ASCOv2/Meetings/Abstractshttp://www.hematology.org/http://www.leukemiafoundation.org/http://www.med-ed.virginia.edu/courses/path/innes/wcd/http://depts.washington.edu/labweb/Divisions/Hema/Flow.htmhttp://www.asco.org/ASCOv2/Meetings/Abstractshttp://www.hematology.org/8/3/2019 Acute B Lymphoblastic Leukemia
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The number of abnormal cells (or leukemia cells) grows quickly. They crowd out the normal red
blood cells, white blood cells and platelets the body needs.
Acute lymphoblastic leukemia symptoms and diagnosis
There are about 4,000 new cases of ALL in the United States each year. It appears
most often in children younger than age 10. ALL is the most common leukemia in
children. However, it can appear in people of any age about one-third of cases
are adults. Acute lymphoblastic leukemia may also be called acute lymphocytic
leukemia or acute lymphoid leukemia.
Signs and symptoms
The symptoms a person with ALL has depend on how many normal blood cells he or
she has. Symptoms also depend on how many leukemia cells there are and where
they collect in the body.
Red blood cells carry oxygen throughout the body. Low numbers of red bloodcells can lead to anemia -- feeling tired or weak, being short of breath andlooking pale.
White blood cells fight infections. Low numbers of white blood cells can leadto fever and frequent infections that are hard to treat.
Platelets control bleeding. Low numbers of platelets can lead to cuts that healslowly, easy bruising or bleeding and tiny red spots under the skin(petechiae).
High numbers of leukemia cells can cause pain in the bones or joints, lack ofappetite, headache or vomiting. These symptoms are less common.
Diagnosis
ALL is diagnosed when blood and bone marrow samples show a large number of
abnormal lymphocyte blasts. To find out the type of ALL and how well it might
respond to treatment, doctors test samples taken from the blood and bone marrow
to learn:
The size and number of leukemia cells. The type of lymphocyte affected the leukemia cells can begin from one of
two types of lymphocytes, B cells or T cells. What changes appear in the chromosomes of the leukemia cells. This is
called cytogenetics.
Doctors also use a test called a lumbar puncture (or spinal tap) to find out whether there areleukemia cells in the fluid around the brain and spinal cord.
Based on these tests, doctors may categorize ALL into one of the following types:
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Early pre-B ALL Common ALL Pre-B-cell ALL Mature B-cell ALL (Burkitt leukemia) Pre-T-cell ALL Mature T-cell ALL
The type of ALL is one of several factors doctors use to plan treatment.
reatment options for acute lymphoblastic leukemia
ALL can get worse quickly, so doctors usually begin treatment right away. To plan
the treatment, doctors look at a patient's risk factors (also called prognostic
factors). Risk factors are patient and disease traits that clinical research studies
have linked to better or poorer outcomes from treatment. Examples of risk factors
are a patient's age and the type of ALL he or she has. For more details, see Risk
Factors for Planning ALL Treatment.
For a patient with ALL, the treatment plan may include:
Chemotherapy drugs that destroy cancer cells or stop them from growing(discussed further below). Some form ofchemotherapy will be part of thetreatment plan for all patients with ALL.
Radiation therapy most patients do not receive radiation therapy.However, children who have signs of disease in the central nervous system(brain and spinal cord) or have a high risk of the disease spreading to thisarea may receive radiation therapy to the brain.
Bone marrow or cord blood transplant (also called a BMT) a transplant
(discussed further below) offers some patients the best chance for a long-term remission of their disease. Because transplants can have serious risks,this treatment is used for patients who are less likely to reach a long-termremission with chemotherapy alone.
Chemotherapy for acute lymphoblastic leukemia
There are three phases of chemotherapy treatment for ALL: induction, consolidation
and maintenance. Many patients also receive treatment called intrathecal
chemotherapy to prevent leukemia from spreading to the central nervous system.
Induction chemotherapy
Most patients with ALL are given induction chemotherapy. The goal of induction
therapy is to bring the disease into remission. Remission is when the patient's blood
counts return to normal and bone marrow samples show no sign of disease.
Induction therapy achieves a remission in more than 95% of children and in about
75% to 89% of adults. [1, 2] Induction therapy is usually very intense and lasts
about one month. After induction chemotherapy, the next step may be a transplant
or consolidation chemotherapy, depending on the treatment plan.
http://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Lrn_about_Disease/ALL/Risk_Factors_for_Planning_ALL_/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Lrn_about_Disease/ALL/Risk_Factors_for_Planning_ALL_/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Undrstnd_Treat_Opt/Lrn_Other_Treatment/Chemotherapy/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Undrstnd_Treat_Opt/Lrn_Other_Treatment/Radiation/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Lrn_about_Disease/ALL/Risk_Factors_for_Planning_ALL_/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Lrn_about_Disease/ALL/Risk_Factors_for_Planning_ALL_/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Undrstnd_Treat_Opt/Lrn_Other_Treatment/Chemotherapy/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Undrstnd_Treat_Opt/Lrn_Other_Treatment/Radiation/index.html8/3/2019 Acute B Lymphoblastic Leukemia
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When a patient is diagnosed with ALL, he or she may have 100 billion leukemia
cells. If induction therapy destroys at least 99% of these cells, the patient is in
remission. However, that could still leave 100 million leukemia cells in the body. If
these cells are not destroyed, they can grow and multiply and cause a relapse of
the disease.
Consolidation therapy
Consolidation therapy, the second phase of chemotherapy, is also intense. It lasts
about four to eight months. The goal of consolidation therapy is to reduce the
number of disease cells left in the body. The drugs and doses used during
consolidation therapy depend on the patient's risk factors.
Maintenance therapy
If a patient stays in remission after induction and consolidation therapy,
maintenance therapy begins. The goal is to destroy any disease cells that remain sothat the leukemia is completely gone. Maintenance therapy is less intense than the
other two phases. It may last two to three years.
Intrathecal chemotherapy
During all three phases of chemotherapy treatment, many patients receive extra chemotherapy to
destroy leukemia cells that may have spread to the central nervous system (the brain and spinal
cord). This chemotherapy is injected right into the spinal fluid using a lumbar puncture (spinaltap) or an Omaya reservoir (a device placed under the scalp). It is called intrathecal
chemotherapy.
Children with ALL who have a high risk of the disease spreading to the central nervous system
may receive higher or more frequent doses of intrathecal chemotherapy. Some of these childrenmay also be given radiation therapy to the brain. However, radiation to the brain can cause
problems with growth and mental development in children, so doctors try to avoid this treatment.
Chemotherapy success rates for ALL
One way to measure the success of a treatment is tracking how many patients
survive five years or more after treatment.
For children, the overall survival rate after chemotherapy is nearly 80%. [3]This includes children with all levels of risk factors. Survival rates are muchlower for children with high-risk disease, while children with low-risk diseasehave even higher rates of survival.
For adults, the overall survival rate after chemotherapy is about 40%. [3] Thisincludes adults with all levels of risk factors. For adults with high-risk disease,survival rates are much lower, while survival rates are higher for some adultswith low-risk disease.
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Relapse
Induction therapy brings about a remission in most patients, but over time some
patients relapse. A relapse is when the disease returns after a remission. Patients
who relapse after chemotherapy may be treated with different chemotherapy drugs
and/or more intense doses. Patients who relapse soon after remission or while they
are receiving chemotherapy have high-risk disease. For these patients,
chemotherapy is less likely to achieve a long-term remission, but a bone marrow or
cord blood transplant may be effective.
Bone marrow or cord blood transplant for acute
lymphoblastic leukemia
For some patients, a bone marrow or cord blood transplant may offer the best
chance for a long-term remission. A transplant is a strong treatment with risks of
serious side effects, so it is not used for all patients with ALL. Transplants are usedwhen chemotherapy alone is unlikely to provide a long-term remission.
Allo