Blood Diseases.ppt

INTRODUCTION Blood is the life-maintaining fluid that

circulates through the body's heart, arteries, veins, and capillaries.

Carries away waste matter and carbon dioxide, and brings nourishment, electrolytes, hormones, vitamins, antibodies, heat, and oxygen to the tissues.

Functions of blood are many and complex –many disorders that require clinical care

Conditions include benign (non-cancerous) disorders / cancers that occur in blood.

RELATIONSHIPS BETWEEN BLOOD TYPES AND ANTIBODIES

Blood Type

Antigens on Red Blood Cell

Can Donate Blood To

Antibodies in Serum

Can Receive Blood From

A A A, AB Anti-B A, O

B B B, AB Anti-A B, O

AB A and B AB None AB, O

O None A, B, AB, OAnti-A and

anti-BO

STANDARD TESTS PERFORMED IN LAB

Typing: ABO group (blood type) Rh typing (positive or negative antigen) screening for any unexpected red blood cell

antibodies Screening for current or past infections –

hepatitis viruses B and C; HIV; human T-lymphotrophic viruses (HTLV) I and II; syphilis

Irradiation to blood cells is performed to disable any T-lymphocytes present in the donated blood.

Blood typing is a laboratory test done to discover a person's blood type. If the person needs a blood transfusion, cross-matching is done following blood typing to locate

donor blood that the person's body will accept. (Illustration by Electronic Illustrators Group.)

(T-lymphocytes can cause a reaction when transfused, but can also cause what is called "graft-versus-host" problems with repeated exposure to foreign cells.)

"Leukocyte-reduced blood" has been filtered to remove the white blood cells which contain antibodies that can cause fevers in the recipient of the transfusion. (These antibodies, with repeated transfusions, may also increase a recipient's risk of reactions to subsequent transfusions.)

COMPONENTS OF BLOOD Red blood cells - carry oxygen to the tissues –

used in the treatment of anemia. Platelets - help the blood to clot – treatment of

leukemia and other forms of cancer. White blood cells - help to fight infection and

aid in the immune process. Plasma - helps to maintain blood pressure;

provides proteins for blood clotting; balances the levels of sodium and potassium

Cryoprecipitate AHF - portion of the plasma contains clotting factors that help to control bleeding.

Albumin, immune globulins, and clotting factor concentrates may also be separated and processed for transfusions.

BLOOD DISORDERS Anemia – Short of RBC Hemophilia – defect in the blood coagulating

mechanism Thrombocythemia – abnormal small number of

platelets in the circulating blood. Hemochromatosis – disorder of iron metabolism

characterized by excessive absorption Hodgkin's Disease – marked by chronic enlargement

of the lymph nodes Leukemias – Progressive proliferation of abnormal

leukocytes Non-Hodgkin's Lymphoma – lymphoma other than

Hodgkin disease

ANEMIA Occurs due to significant shortage of healthy

red blood cells. RBC serve as transporters of oxygen to

different tissues and organs of the body. Body is not getting sufficient iron – indicates

deficiency of hemoglobin in the RBCs Detected or confirmed through CBC and this

often sort the disorder is quite common. Many kinds of anemia depending on the causes

and can be classified through different ways depending on the morphology of RBCs.

SYMPTOMS Feels restless and loss of energy after a little

physical activity. Because of the shortage of oxygen in the organs

fatigue begins to occur. Unusual fast heart rate especially during

exercise. Panting and headache while doing exercise. Not be able to concentrate properly

ANEMIA

TYPES OF ANEMIA Macrocytic anemia: Megaloblastic anemia and

non-megaloblastic macrocyctic anemia. Primary cause of this sort of anemia is collapse of DNA synthesis with kept RNA synthesis that occurs due to the division of the divisional cells.

Microcytic anemia: Sort of anemia occurs due to hemoglobin synthesis shortage or collapse.

Normcytic anemia: Occurs when Hb levels decreases overall. Size of RBC is often normal.

Heinz Body anemia: Considered a cell abnormality that usually occurs in cells under anemia.

Iron-deficiency anemia – hypochromic microcytic anemia characterized by low serum iron, increased serum iron-binding capacity, decreased serum ferritin, and decreased marrow iron stores.

Megaloblastic (pernicious) anemia – predominant number of megaloblastic erythroblasts, and relatively few normoblasts, among the hyperplastic erythroid cells in the bone marrow

Hemolytic anemia – increased rate of erythrocyte destruction.

Sickle cell anemia – autosomal recessive anemia characterized by crescent- or sickle-shaped erythrocytes and accelerated hemolysis, due to substitution of a single amino acid - chromosome 11

Aplastic anemia – greatly decreased formation of erythrocytes and hemoglobin, usually associated with pronounced granulocytopenia and thrombocytopenia

Chronic anemia Anemia of folate deficiency

Cooley's anemia (beta thalassemia) – syndrome of severe anemia resulting from the homozygous state of one of the thalassemia genes or one of the hemoglobin Lepore genes with onset, in infancy or childhood, of pallor, icterus, weakness, splenomegaly, cardiac enlargement, thinning of inner and outer tables of skull, microcytic hypochromic anemia with poikilocytosis, anisocytosis, stippled cells, target cells, and nucleated erythrocytes

CAUSES Accurate blood loss Anemia of chronic disease Bone marrow failure and Plastic anemia.

TREATMENT A successful treatment of anemia depends on

successful diagnosis of the cause that brings about the disease.

There are several causes of anemia including blood loss, cancer, a nutritional deficiency, chronic illness, bone marrow infiltration, lower response to erythropoietin and inflammation.

These causes can be determined with laboratory test results and physical examination.

Specific treatment for anemia will be determined by the physician based on:

Age, overall health, and medical history Extent of the disease Tolerance for specific medications, procedures,

or therapies Expectations for the course of the disease Opinion or preference of the patient

Treatment of the causative disease Vitamin and mineral supplements Change in diet Medication Blood transfusion Bone marrow transplant Surgery (to remove the spleen, if related to

hemolytic anemia) Antibiotics (if an infection is the causative

agent)

HEMOPHILIA Inherited bleeding or coagulation, disorder. Persons with hemophilia lack the ability to stop

bleeding because of the low levels, or complete absence, of specific proteins, called "factors," in their blood that are necessary for clotting.

Proper clotting of blood helps prevent excessive bleeding.

Types of hemophilias – hemophilia A - lack of factor VIII hemophilia B - lack of factor IX

CAUSES Hemophilia types A and B are inherited

diseases passed on from a gene located on the X chromosome. Females carrier of hemophilia has the hemophilia gene on one of her X chromosomes, and there is a 50 percent chance that she may pass the defective gene to her male offspring.

Males who inherit the defective gene will develop hemophilia. Males with hemophilia do not pass the gene to their sons; however, they do pass the gene to their daughters.

Females who inherit the defective gene will become carriers who may, in turn, have a 50 percent chance of passing it on to their children. Although females who inherit the gene generally have no active problems related to hemophilia, some may have other problems associated with bleeding, such as excessive menstrual bleeding, frequent or severe nosebleeds, or bleeding after dental procedures or surgery.

In about 1/3rd of hemophilia cases, there is no family history of the disease. These cases are due to a new or spontaneous development of the defective gene in the female.

SYMPTOMS Excessive, uncontrollable bleeding Bleeding may occur even if there is no injury. Often occurs in the joints and in the head. Bruising - Occur from small accidents, which

can result in a large hematoma. Bleeds easily - Tendency to bleed. Bleeding into a joint - Hemarthrosis can cause

pain, immobility, and eventually deformity if not medically managed properly.

Bleeding into the muscles - Bleeding into the muscles can cause swelling, pain, and redness.

Bleeding from injury or bleeding in the brain - Bleeding from injury or spontaneously in the brain, is the most common cause of death in children with hemophilia and the most serious bleeding complication.

Other sources of bleeding - Blood found in the urine or stool may also be a symptom of hemophilia.

The symptoms of hemophilia may resemble other blood disorders or medical problems.

DIAGNOSIS & EFFECTS Complete medical history and physical

examination Clotting factor levels Complete blood count (CBC) Assessment of bleeding times DNA testing. Most common cause of disability from hemophilia

is chronic joint disease or arthropathy, which is caused by uncontrolled bleeding into the joints.

Hemorrhage – severe internal or external discharge of blood, is a continuing problem.

TREATMENT

Blood transfusions

Prophylactic (preventive) treatment with infused clotting factors

Immune Thrombocytopenic Purpura (Thrombocytopenia)

Blood disorder characterized by an abnormal decrease in the number of blood platelets, which results in internal bleeding.

Acute thrombocytopenic purpura – Most common in young children, the symptoms may follow a virus infection and disappears within a year - usually disorder does not recur.

Chronic thrombocytopenic purpura – Onset of the disorder can happen at any age, and symptoms can last six months or longer. Adults have this form more often than children, and females have it 3 times more often than males.

CAUSES Medications - including over-the-counter Infection Pregnancy Immune disorders However, about half of all cases are classified

as idiopathic.

SYMPTOMS Internal bleeding, which may cause: ecchymosis

- bruising , petechiae - tiny red dots on skin or mucous membranes

Occasionally, bleeding from the nose, gums, digestive tract, urinary tract

Rarely, bleeding within the brain Symptoms may resemble other blood disorders

or medical problems.

DIAGNOSIS Complete medical history and physical

examination Additional blood and urine tests Other evaluation procedures Careful review of patient's medications Bone marrow examination

TREATMENT Treatment of the causative disease Discontinuation of causative drugs Treatment with corticosteroids Treatment with medications Lifestyle changes, such as: use of protective

gear , avoidance of certain activities

HEMOCHROMATOSIS Also called iron overload

disease, is the most common genetic disorder.

It is a metabolic disorder that causes increased absorption of iron, which is deposited in the body tissues and organs.

The iron accumulates in the body where it may become toxic and cause damage.

HODGKIN'S DISEASE Type of lymphoma, a cancer in the lymphatic

system. Rare disease usually occurs most often in

people between the ages of 15 and 34, and in people over age 55.

Hodgkin's disease causes the cells in the lymphatic system to abnormally reproduce, eventually making the body less able to fight infection.

Hodgkin's disease cells can also spread to other organs.

Lymphadenoma – Hodgkin's Disease (Pseudo-leukemia of German authors)

SIGNS AND SYMPTOMS Painless swelling of lymph nodes in neck,

underarm, and groin Fever Night sweats Fatigue Weight loss Itching of the skin It may resemble other blood disorders or

medical problems, such as influenza or other infections.

RISK FACTORS Past infection with infectious mononucleosis History of infectious mononucleosis (caused by

an infection with the Epstein-Barr virus) Acquired immunodeficiency syndrome (AIDS)

DIAGNOSIS Additional blood tests X-rays of the chest, bones, liver, and spleen Biopsy of the lymph nodes

TREATMENT Radiation therapy Chemotherapy

LEUKEMIA Cancer of the blood cells, usually the white

blood cells. Leukemic cells look different than normal cells

and do not function properly.

Acute Lymphocytic Leukemia Acute Myelogenous Leukemia Chronic Lymphocytic Leukemia Chronic Myelogenous Leukemia

TYPES OF LEUKEMIA

Lymphocytic or myelogenous leukemia Cancer can occur in either the lymphoid or

myeloid white blood cells. When the cancer develops in the lymphocytes

(lymphoid cells), it is called lymphocytic leukemia.

Cancer develops in the granulocytes or monocytes (myeloid cells) – myelogenous leukemia.

Acute or chronic leukemia Acute leukemia - The new or immature cells,

called blasts, remain very immature and cannot perform their functions. The blasts increase in number rapidly, and the disease progresses quickly.

Chronic leukemia - There are some blast cells present, but they are more mature and are able to perform some of their functions. The cells grow more slowly, and the number increases less quickly, so the disease progresses gradually.

LEUKEMIA IS CLASSIFIED INTO ONE OF THE FOUR MAIN TYPES OF LEUKEMIAS Acute myelogenous leukemia (AML) Chronic myelogenous leukemia (CML) Acute lymphocytic leukemia (ALL) Chronic lymphocytic leukemia (CLL)

SIGNS AND SYMPTOMS More frequent infections and fevers Anemia and its symptoms: pale skin, fatigue,

weakness, bleeding, bruising, fever, chills, loss of appetite, loss of weight, swollen or tender lymph nodes, liver, or spleen, petechiae (tiny red spots under the skin), swollen or bleeding gums, sweating, bone or joint pain.

Acute leukemia: headaches, vomiting, confusion, loss of muscle control, seizures, swollen testicles, sores in the eyes or on the skin.

Chronic leukemia may affect the skin, central nervous system, digestive tract, kidneys, and testicles.

The symptoms of acute and chronic leukemias may resemble other blood disorders or medical problems

DIAGNOSIS Physician examination for swelling in the: liver,

spleen, lymph nodes under the arms, in the groin, and in the neck

Blood tests and laboratory tests Blood tests to examine the blast (immature)

blood cells Bone marrow aspiration and biopsy Lymph node biopsy Spinal tap Imaging procedures, such as x-ray, ultrasound,

and computed tomography (CT)

TREATMENT Chemotherapy Radiation therapy Bone marrow stem cell transplantation Biological therapy Platelet transfusion Red blood cell transfusion Medications to prevent or treat damage to other

systems of the body caused by leukemia treatment

ACUTE LYMPHOCYTIC LEUKEMIA [ALL]

ALL is a cancer of the blood in which too many lymphocytes, a type of white blood cell, are produced by the bone marrow and by organs of the lymph system.

The lymphocytes fight infection by making antibodies that attack harmful elements. But, in ALL, the cells are immature and overabundant. They crowd out other blood cells, and may collect in the blood, bone marrow, and lymph tissue.

Acute leukemia can occur over a short period of days to weeks. Chromosome abnormalities (extra chromosomes and structural changes in the chromosome material) are present in the majority of all patients.

ALL is the most common type of leukemia in young children. This type of leukemia may also affect adults, especially those age 65 and older.

SIGNS AND SYMPTOMS Anemia Bleeding Bruising Fever Persistent weakness Fatigue Aches in bones and joints Swollen lymph nodes

DIAGNOSIS Blood tests and other evaluation procedures Bone marrow aspiration and biopsy Spinal tap/lumbar puncture - A small amount of

cerebral spinal fluid (CSF) removed. CSF is the fluid that bathes the brain and spinal cord.

TREATMENT Chemotherapy Radiation therapy Bone marrow transplantation

ACUTE MYELOGENOUS LEUKEMIA [AML]

AML cancer of the blood in which too many granulocytes are produced in the bone marrow.

Bone marrow cells mature into several different types of blood cells.

AML affects the young blood cells (called blasts) that develop into a type of white blood cell (called granulocytes).

Main function of granulocytes is to destroy bacteria.

Blasts, do not mature & become too numerous, remain in the bone marrow and blood.

Acute leukemia can occur over a short period of days to weeks.

Chromosome abnormalities (extra chromosomes and structural changes in the chromosome material) are present in the majority of ALL patients.

AML occurs in both children and adults.

SIGNS AND SYMPTOMS Anemia Bleeding Bruising Fever Persistent weakness Fatigue Aches in bones and joints Swollen lymph nodes

DIAGNOSIS Complete medical history and physical

examination Blood tests Bone marrow aspiration and biopsy Spinal tap/lumbar puncture

TREATMENT Chemotherapy Radiation therapy Bone marrow transplantation

CHRONIC LYMPHOCYTIC LEUKEMIA [CLL]

CLL cancer of the blood in which too many lymphocytes, a type of white blood cells, are produced by the bone marrow and by organs of the lymph system.

Lymphocytes fight infection by making antibodies that attack harmful elements, but in CLL, the cells are immature and over abundant. They crowd out other blood cells, and may collect in the blood, bone marrow and lymph tissue.

CLL usually occurs in people 60 years of age or older. It is a slowly progressing disease.

SIGNS AND SYMPTOMS Persistent weakness Swollen lymph nodes Enlarged spleen Enlarged liver Anemia

DIAGNOSIS Complete medical history Physical examination Blood tests Bone marrow biopsy

TREATMENT Chemotherapy Radiation therapy

Treatment for complications – infection or anemia

Leukapheresis, a procedure to remove excessive lymphocytes

Bone marrow transplantation Splenectomy, surgery to remove the spleen

CHRONIC MYELOGENOUS LEUKEMIA [CML]

CML cancer of the blood in which too many granulocytes, a type of white blood cell, are produced in the bone marrow.

Bone marrow cells mature into several different types of blood cells.

CML affects the young blood cells (called blasts) that develop into a type of white blood cell (called granulocytes).

Main function of granulocytes is to destroy bacteria. The blasts, which do not mature and become too numerous, remain in the bone marrow and blood.

CML can occur over a period of months or years. Specific chromosome rearrangement is found in

patients with CML. Part of chromosome #9 breaks off and attaches itself to chromosome #22, so that there is an exchange of genetic material between these two chromosomes. This rearrangement changes the position and functions of certain genes, which results in uncontrolled cell growth.

Other chromosome abnormalities can also be present.

CML occurs mainly in adults and is rare in children.

SIGNS AND SYMPTOMS Anemia Bleeding Bruising Fever Persistent weakness Fatigue Aches in bones and joints Swollen lymph nodes

DIAGNOSIS Blood tests Bone marrow aspiration and biopsy Spinal tap/lumbar puncture

TREATMENT Chemotherapy Biological therapy - using the body's immune

system to fight cancer Radiation therapy Stem cell transplantation Splenectomy

NON-HODGKIN'S LYMPHOMA Type of lymphoma, which is a cancer in the

lymphatic system. Non-Hodgkin's disease causes the cells in the

lymphatic system to abnormally reproduce eventually causing tumors to grow and can also spread to other organs.

Etiology is idiopathic

SYMPTOMS Painless swelling of lymph nodes in neck,

underarm, and groin Fever Night sweats Fatigue Weight loss Itching of the skin Recurring infections

RISK FACTORS Genetic disease of the

immune system Unprotected exposure to

strong sunlight High-fat, low-fiber diet Smoking Excessive alcohol

consumption Environmental factors –

radiation, chemicals, and infections

Organ transplantation Infections with HIV or

HTLV-1 Infections with malaria History of infectious

mononucleosis Helicobacter pylori

bacterium – stomach ulcers

DIAGNOSIS Blood tests X-rays of the chest, bones, liver, and spleen Biopsy of the lymph nodes, bone marrow, and

other sites Lymphangiograms - lymphatic system x-rays CT scan Ultrasonography scan

TREATMENT Radiation therapy Chemotherapy

THROMBOCYTHEMIA It is a myeloproliferative blood disorder. It is characterized by the production of too

many platelets in the bone marrow. Too many platelets make normal clotting of

blood difficult Etiology is idiopathic

SYMPTOMS Increased blood clots in arteries and veins Bleeding Bruising easily Bleeding from the nose, gums, gastrointestinal

tract Bloody stools Hemorrhaging after injury or surgery Weakness Enlarged lymph nodes

DIAGNOSIS Complete medical history and physical

examination Blood counts and elevated platelet levels Bone-marrow biopsy

TREATMENT Chemotherapy Plateletpheresis - a procedure to remove extra

platelets from the blood

BONE MARROW TRANSPLANTATION [BMT]

BMT is a special therapy for patients with cancer or other diseases which affect the bone marrow.

A bone marrow transplant involves taking cells that are normally found in the bone marrow (stem cells), filtering those cells, and giving them back either to the patient or to another person.

The goal of BMT is to transfuse healthy bone marrow cells into a person after their own unhealthy bone marrow has been eliminated.

Bone marrow transplantation is not yet a standard treatment therapy, but has been used successfully to treat diseases such as leukemias, lymphomas, aplastic anemia, immune deficiency disorders, and some solid tumor cancers since 1968.

Bone marrow is the soft, spongy tissue found inside bones. It is the medium for development and storage of about 95

percent of the body's blood cells. Blood cells that produce other blood cells are called stem

cells. The most primitive of the stem cells is called the

pluripotent stem cell, which is different than other blood cells Renewal - able to reproduce another cell identical to

itself. Differentiation - able to generate one or more subsets

of more mature cells. It is the stem cells that are needed in bone marrow

transplantation.

NORMAL ANATOMY

INDICATION

PROCEDURE

AFTER CARE

GOAL OF BMT Cure many diseases and types of cancer.

When a person's bone marrow has been damaged or destroyed due to a disease or intense treatments of radiation or chemotherapy for cancer, a marrow transplant may be needed.

A bone marrow transplant can be used to: Replace diseased, non-functioning bone marrow

with healthy functioning bone marrow Replace the bone marrow and restore its normal

function after high doses of chemotherapy or radiation are given to treat a malignancy – process called "rescue".

Replace bone marrow with genetically healthy functioning bone marrow to prevent further damage from a genetic disease process (such as Hurler's syndrome, and adrenoleukodystrophy).

BMT BENEFITS Leukemias Severe aplastic anemia Lymphomas Multiple myeloma Immune deficiency disorders Solid-tumor cancers – like breast or ovarian

DIFFERENT TYPES OF BMT Autologous bone marrow transplant Allogeneic bone marrow transplant

A parent - a haploid-identical match is when the donor is a parent and the genetic match is at least half identical to the recipient.

An identical twin - a syngeneic transplant is an allogeneic transplant from an identical twin. Identical twins are considered a complete genetic match for a marrow transplant.

Unrelated bone marrow transplants (UBMT or MUD for matched unrelated donor)

Umbilical cord blood transplant

Stem Cell Transplantations [SCT] In bone marrow - there is 1 stem cell in every

100,000 blood cells. Breast bone, skull, hips, ribs, and spine contains

the stem cells. Harvesting stem cells from bone marrow

requires a surgical procedure. Harvesting stem cells from the blood stream is

accomplished by a process called apheresis.

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