HEMOBLASTOSES.HEMOBLASTOSES.ANEMIASANEMIAS..

assistant of professorassistant of professor

Nechiporenko G.VNechiporenko G.V..

HEMOBLASTOSESHEMOBLASTOSES

HEMOBLASTOSESHEMOBLASTOSES are tumoral diseases are tumoral diseases of hemo-lymphopoietic tissue. of hemo-lymphopoietic tissue.

They are subdivided into: They are subdivided into:

1) Leukemias1) Leukemias

2) Peripheral lymphomas2) Peripheral lymphomas

Acute lymphoblastic leukemia (ALL)Acute lymphoblastic leukemia (ALL) L Lymphoymphoblasts are blasts are very immature cells with large nuclei that very immature cells with large nuclei that contain nucleoli. ALL is more common in children than adults. contain nucleoli. ALL is more common in children than adults. Many cases of ALL in children respond well to treatment, and Many cases of ALL in children respond well to treatment, and

many are curable.many are curable.

Necrotic Necrotic tonsillitis in tonsillitis in acute acute leukemialeukemia

Chronic lymphocytic leukemia (CLL)Chronic lymphocytic leukemia (CLL)MMature lymphocytes are increased markedly in number. ature lymphocytes are increased markedly in number. AA

disease disease is is most often seen in older adultsmost often seen in older adults;it;it responds poorly to responds poorly to treatment, but it is indolent.treatment, but it is indolent.

Lymph nodes in chronic Lymph nodes in chronic lympholeukemialympholeukemia

Liver in chronic Liver in chronic lympholeukemialympholeukemia

BBone marrow of a patient with acute myeloone marrow of a patient with acute myeloblasticblastic leukemialeukemia..

There are many large immature myelocytes andThere are many large immature myelocytes and one one megakaryocyte at the right centermegakaryocyte at the right center

Blood smear, initial presentation of chronic Blood smear, initial presentation of chronic myeloid leukemia (CML) - High powermyeloid leukemia (CML) - High power

Blood smears, chronic myelogenous Blood smears, chronic myelogenous leukemia (CML)leukemia (CML)

blast phase blast phase (left)(left)

Bone marrow in chronic Bone marrow in chronic myeloleukemiamyeloleukemia

Spleen in chronic Spleen in chronic myeloleukemiamyeloleukemia

Liver in Liver in chronic chronic

myeloleukemimyeloleukemiaa

The skull demonstrates the characteristic rounded The skull demonstrates the characteristic rounded

"punched out" lesions of multiple myeloma."punched out" lesions of multiple myeloma.

Round lesions filled with a soft reddish material are Round lesions filled with a soft reddish material are indicative of foci of myeloma in this section of indicative of foci of myeloma in this section of

vertebral bone.vertebral bone.

TThere are numerous plasma cells with eccentric here are numerous plasma cells with eccentric nuclei and a perinuclear halo of clearer cytoplasmnuclei and a perinuclear halo of clearer cytoplasm in in a smear of bone marrow aspirate from a patient a smear of bone marrow aspirate from a patient

with multiple myelomawith multiple myeloma. .

Cell with Cell with paraprotein in paraprotein in

multiple multiple myelomamyeloma

Hodgkin's Disease - Hodgkin's Disease - Classification Classification

Type Histologic Features

Frequency Prognosis

Nodular sclerosis

Bands of fibrosis, lacunar cells

Most frequent type, more common in women

Good, most are stage I or II

Mixed cellularity

Composed of many different cells

Most frequent in older persons, second most frequent overall

Fair, most are stage III

Lymphocyte predominance

Mostly B-cells and few Reed-Sternberg variant cells

Uncommon Good, most are stage I or II

Lymphocyte depletion

Many Reed-Sternberg cells and variants

Uncommon Poor, most are stage III or IV

Hodgkin's Disease - Staging Hodgkin's Disease - Staging Stage Characteristics

I Only a single lymph node site or extranodal site is involved

II Two or more lymph node sites on one side of the diaphragm are involved, or limited contiguous extranodal site involvement

III Lymph node sites on both sides of the diaphragm are involved, with splenic or limited contiguous extradodal site involvement, or both

IV Extensive involvement of extranodal sites, with or without lymph node involvement

Here is a 5 cm lymph node (obviously from a patient with Here is a 5 cm lymph node (obviously from a patient with lymphadenopathy). The node should normally be soft and pink and lymphadenopathy). The node should normally be soft and pink and less than 1 cm in size. This lymph node is involved with Hodgkin's less than 1 cm in size. This lymph node is involved with Hodgkin's

disease. disease.

This is a liver that is involved with Hodgkin's disease. The This is a liver that is involved with Hodgkin's disease. The staging of Hodgkin's disease is very important in staging of Hodgkin's disease is very important in

determining therapy. determining therapy.

Spleen in Spleen in Hodgkin’s Hodgkin’s diseasedisease

Mixed Mixed cellularity type of Hodgkin’s Hodgkin’s

diseasedisease

Hodgkin's disease, nodular sclerosis type.Hodgkin's disease, nodular sclerosis type. Note the bands of pink collagenous tissue dividing Note the bands of pink collagenous tissue dividing

the field in this lymph node.the field in this lymph node.

These are the lacunar cellsThese are the lacunar cells characteristic for the characteristic for the nodular sclerosis type of Hodgkin's disease.nodular sclerosis type of Hodgkin's disease.

This is a high power view of a Reed-Sternberg cell This is a high power view of a Reed-Sternberg cell seen with Hodgkin's disease. Note the large, seen with Hodgkin's disease. Note the large,

prominent nucleoli. prominent nucleoli.

Non-Hodgkin's Lymphomas Non-Hodgkin's Lymphomas TypeType Histologic Histologic

Features Features ImmunogeneticsImmunogenetics Clinical Clinical

FeaturesFeatures

Small Small Lymphocytic Lymphocytic Lymphoma Lymphoma

Small and well-Small and well-differentiated B differentiated B lymphocytes, lymphocytes, with diffuse with diffuse effacement of effacement of nodal nodal architecture and architecture and no follicles no follicles

CD19, 5; Bcl-2 CD19, 5; Bcl-2 and Bcl-6 and Bcl-6 expression expression

Seen in older Seen in older adults, it is adults, it is

essentially the essentially the solid tissue solid tissue

(lymph nodal) (lymph nodal) component of component of

chronic chronic lymphocytic lymphocytic leukemia; leukemia;

disease tends disease tends to be to be

generalized but generalized but with indolent with indolent course and course and prolonged prolonged

survival; some survival; some may transform may transform

to more to more aggressive aggressive

lymphomas lymphomas

Follicle Center Follicle Center Lymphoma Lymphoma (predominantly (predominantly small cell) small cell)

Nodal Nodal architecture is architecture is effaced by effaced by monotonous, monotonous, crowded follicles crowded follicles composed of composed of monomorphous monomorphous small cleaved B-small cleaved B-lymphocytes lymphocytes

CD19, 20, 79a; CD19, 20, 79a; t(14:18); Bcl-2 t(14:18); Bcl-2 expression expression

Most common Most common type, seen in type, seen in adults, often adults, often involves involves multiple lymph multiple lymph nodes, course is nodes, course is indolent, with indolent, with prolonged prolonged survival, though survival, though some may some may transform to a transform to a large cell large cell lymphoma lymphoma

Diffuse Large B-Diffuse Large B-cell Lymphoma cell Lymphoma

Cells are large, Cells are large, with prominent with prominent nucleoli and nucleoli and abundant abundant cytoplasm and cytoplasm and many mitoses. many mitoses. Most are B-cell, Most are B-cell, but 20% are T-but 20% are T-cell phenotype cell phenotype

CD19, 20, 79a; CD19, 20, 79a; some have some have t(14;18); some t(14;18); some have Bcl-2 and have Bcl-2 and Bcl-6 expression; Bcl-6 expression; linked to EBV linked to EBV infection; infection; negative TdT negative TdT

Though often Though often localized, they localized, they tend to be tend to be aggressive aggressive extranodal extranodal masses; seen in masses; seen in adults and adults and children, can be children, can be seen in HIV seen in HIV infection infection

Burkitt Burkitt Lymphoma Lymphoma

Intermediate Intermediate sized B-sized B-lymphocytes lymphocytes (small-(small-noncleaved cells) noncleaved cells)

CD10, 19, 20, CD10, 19, 20, 79a; t(8:14) is 79a; t(8:14) is characteristic; characteristic; African form African form linked to EBV linked to EBV infection; infection; negative TdT negative TdT

Endemic in Endemic in Africa with Africa with mandibular and mandibular and abdominal abdominal involvement; involvement; sporadic sporadic elsewhere with elsewhere with abdominal abdominal involvement; involvement; affects mainly affects mainly children and children and young adults young adults

High-grade B-High-grade B-cell Lymphoma cell Lymphoma (small non-(small non-cleaved) cleaved) Burkitt-like Burkitt-like LymphomaLymphoma

Intermediate Intermediate sized B-sized B-lymphocytes lymphocytes (small non-(small non-cleaved cells)cleaved cells)

CD19, 20CD19, 20 Sporadic; may Sporadic; may be seen with be seen with HIV infectionHIV infection

Precursor T or Precursor T or B-cell B-cell Lymphoblastic Lymphoblastic Lymphoma/LeuLymphoma/Leukemia kemia (Lymphoblastic (Lymphoblastic Lymphoma) Lymphoma)

Intermediate Intermediate sized sized lymphocytes in lymphocytes in a diffuse a diffuse pattern pattern

B-cells are B-cells are CD19, 20, CD19, 20, sometimes sometimes CD10; T-cells CD10; T-cells are CD3 and 8; are CD3 and 8; all are TdT all are TdT positive positive

Seen in children Seen in children and and adolescents; T-adolescents; T-cell type often cell type often in mediastinum; in mediastinum; very aggressive very aggressive and can and can progress to progress to acute acute lymphocytic lymphocytic leukemia leukemia

Mantle Cell Mantle Cell LymphomaLymphoma

Small to Small to medium sized B medium sized B cellscells

CD 19, 20, 43; CD 19, 20, 43; t(11;14); Bcl-1 t(11;14); Bcl-1 (Cyclin D1) (Cyclin D1) expressionexpression

Seen in adults Seen in adults in middle age; in middle age; often advanced often advanced at diagnosis at diagnosis and may be and may be extranodal, extranodal, including including multifocal multifocal submucosal submucosal nodules in nodules in bowel bowel

Marginal Zone Marginal Zone LymphomaLymphoma

Small to Small to medium sized B medium sized B cellscells

CD19, 20, 79a; CD19, 20, 79a; negative CD5 negative CD5 and 10and 10

Seen in middle Seen in middle aged adults; aged adults; typically arises typically arises in areas of in areas of immune immune activation activation (Hashimoto (Hashimoto thyroiditis, thyroiditis, Sjogren Sjogren syndrome, H. syndrome, H. pylori gastritis); pylori gastritis); similar lesions similar lesions asociated with asociated with mucosal mucosal lymphoid tissue lymphoid tissue are called are called MALTomas MALTomas (mucosa-(mucosa-associated associated lymphoid tissue lymphoid tissue tumors); may tumors); may transform to transform to diffuse large B-diffuse large B-cell lymphoma cell lymphoma

Lymph node, follicular Lymph node, follicular lymphoma, low grade lymphoma, low grade

This is a malignant This is a malignant lymphoma, small cleaved lymphoma, small cleaved cell type, follicular (also cell type, follicular (also known as: malignant known as: malignant lymphoma, poorly lymphoma, poorly differentiated lymphocytic differentiated lymphocytic type, nodular). type, nodular).

Here is a lymph node Here is a lymph node involved by lymphoma. involved by lymphoma. The capsule of the node The capsule of the node has been invaded and the has been invaded and the lymphomatous cells lymphomatous cells extend into the extend into the surrounding adipose surrounding adipose tissue. Note that the tissue. Note that the follicles are numerous and follicles are numerous and irregularly sized. irregularly sized.

Small bowel, Burkitt Small bowel, Burkitt lymphoma lymphoma

Malignant lymphoma is typically extranodal in AIDS. Malignant lymphoma is typically extranodal in AIDS. Seen here in small intestine are two mass lesions on Seen here in small intestine are two mass lesions on

the mucosal surface.the mucosal surface.

Extranodal malignant lymphoma inExtranodal malignant lymphoma in AIDS is often AIDS is often multifocal. Seen here in liver are two mass lesions multifocal. Seen here in liver are two mass lesions on the cut surface. The color can range from white on the cut surface. The color can range from white

to tan to red, often intermixed.to tan to red, often intermixed.

Non-Hodgkin's lymphomas seen in the central nervous system Non-Hodgkin's lymphomas seen in the central nervous system with AIDS are essentially clonal expansions of Epstein-Barr with AIDS are essentially clonal expansions of Epstein-Barr

virus infected lymphocytes. These lymphomas are high grade virus infected lymphocytes. These lymphomas are high grade (immunoblastic) and agressive, with a poor prognosis. (immunoblastic) and agressive, with a poor prognosis.

AnemiaAnemia

Anemia is a reduction in the Anemia is a reduction in the concentration of the hemoglobin in the concentration of the hemoglobin in the blood. It is usually accompanied by blood. It is usually accompanied by reduction in the number of red blood reduction in the number of red blood cells (with the exception of iron-cells (with the exception of iron-deficiency types, thalassemia). deficiency types, thalassemia). Poikilocytosis (different size), Poikilocytosis (different size), anisocytosis (different shape) of anisocytosis (different shape) of erythrocytes can develop in blood. erythrocytes can develop in blood. Erythroblasts, normoblasts, Erythroblasts, normoblasts, megaloblasts appear also.megaloblasts appear also.

Classification of Classification of Anemias.Anemias.

A.A. PATHOPHYSIOLOGICPATHOPHYSIOLOGICI. Anemia due to increased blood I. Anemia due to increased blood

lossloss Acute post-haemorrhagic anemiaAcute post-haemorrhagic anemia Chronic blood lossChronic blood lossII. Anemias due to increased red cell II. Anemias due to increased red cell

destruction destruction (Hemolytic (Hemolytic anemias) anemias) A.A. Extrinsic (extracorpuscular) red cell Extrinsic (extracorpuscular) red cell

abnormalitiesabnormalitiesB.B. Intrinsic (intracorpuscular) red cell Intrinsic (intracorpuscular) red cell

abnormalitiesabnormalities

III. Anemias due to impaired red cell productionIII. Anemias due to impaired red cell production a)a) Cytoplasmic maturation defectsCytoplasmic maturation defects Deficient haem synthesis:Deficient haem synthesis:

Iron deficiency anemiaIron deficiency anemia Deficient globin synthesis:Deficient globin synthesis:

Thalassaemic syndromesThalassaemic syndromes b)b) Nuclear maturation defectsNuclear maturation defects Vitamin B12 and/or folic acid deficiency: Vitamin B12 and/or folic acid deficiency:

Megaloblastic anaemiaMegaloblastic anaemia c)c) Defect in stem cell proliferation and Defect in stem cell proliferation and

differentiationdifferentiation Aplastic anemiaAplastic anemia Pure red cell aplasiaPure red cell aplasia Anemia of chronic disordersAnemia of chronic disorders Bone marrow infiltrationBone marrow infiltration Congenital anemiaCongenital anemia

B.B. MORPHOLOGICMORPHOLOGIC

II.. Microcytic, hypochromicMicrocytic, hypochromic

II.II. Normocytic, normochromicNormocytic, normochromic

III.III. Macrocvtic. normochromicMacrocvtic. normochromic

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IschemiaIschemiaof kidney in acute posthemorrhagic anemiaof kidney in acute posthemorrhagic anemia

Extramedullar hemopoiesis in liver Extramedullar hemopoiesis in liver with chronic posthemorrhagic anemiawith chronic posthemorrhagic anemia

Pernicious anemia (B12 or/and Pernicious anemia (B12 or/and folic acid deficiency)folic acid deficiency)

Cause is absence of gastromucoprotein Cause is absence of gastromucoprotein secretion by parietal cells due to secretion by parietal cells due to heredital dysfunction of fundal glands heredital dysfunction of fundal glands in stomach. Autoimmune processes in stomach. Autoimmune processes take place: autoantibodies blockade take place: autoantibodies blockade gastromucoprotein and vit. B 12, so gastromucoprotein and vit. B 12, so erythropoiesis changes in erythropoiesis changes in megaloblastic type. Hemolysis megaloblastic type. Hemolysis predominates over hemopoiesis.predominates over hemopoiesis.

MMacro-ovalocytes in a case of acro-ovalocytes in a case of PPernicious ernicious AAnemianemia..

AAplastic anemiaplastic anemia Hematopo Hematopoiietic elements in this bone marrow etic elements in this bone marrow

biopsy are markedly reduced. biopsy are markedly reduced.

Hemosiderosis of SpleenHemosiderosis of Spleen

Hemolytic Hemolytic anemia.anemia.

HemoglobinurHemoglobinuric nephrosisic nephrosis