wbc abnormality

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2. Normal Bone Marrow BM: Pluripotent stem cell Lymphoblast stem cell B , T Haemopoietic stem cell Myeloid Erythroid Megak. Mono Granulocyte RBC Plat Monocytes 3. Normal Bone Marrow Stem Cell Blast Cell Mature Cell. BM ErythroblastErythroblast Leucoblast Megakaryoblast Peripheral RBC WBC Platelets Blood Hb (O2) Defense Coagulation Infection Clotting ( Stop blood escape) Granulocyte Lymphocyte Monocyte Neutrophil Pre-B B Eosinophil Pre-T T Basophil 4. Normal Blood Cell Morphology Platelet RBC Lymphocyte Monocyte Eosinophil Neutrophi l Basophil 5. Leucocytosis and leucopenia Leucocytosis: NAD range 4 10x10/l Leucopenia: N 65% L 25% M 5% E 2% B 1% Neutrophil Neutrophilia Lymphocytes Lymphocytosis Monocytes Monocytosis. Eosinophil Oesinophilia Basophil Basophilia Neutropenia Lymphopenia Monocytopenia (Viral,drugs,aplastic A) oOr due to large no. of leucocyte not normally circulate e.g. Myeloblast or Lymphoblast as in Acute Leukemia. Leucocytosis: Neutrophilia: bacterial infection and other metabolic disorders, stresses (MI) (gout, ureamia) Ca.. Hge and haemolysis (BM hyperactivity) Monocytosis: chronic infection. eosinophilia: allergy, parasites, skin. Lymphocytosis: viral infection and chronic infection in TB, thyrotoid disease.. 6. Definition & Characteristics Normal WBC Count (4000 8000)x 10 LEUKEMIA : WHITE BLOOD Malignant disorder of WBC Uncontrolled growth of blast or more mature cells to replace normal cells in the bone marrow and escape into the blood. Failure of full maturation of cells. Invasion into the rest of the body organs (Liver, spleen, menings, testis, CNS) is mainly responsible for the devastating effect of the leukemia. 7. Normal B.M. BM invasion 8. Classification of Leukemia Division: Based on either cell type Lymphoid(ALL) lymphoblast a. Morphology Myloid (AML) Myeloblast Acute(AML, ALL) b. Clinical Chronic(CLL, CGL) Chronic lymphocytic leukemia Chronic Granulocytic leukemia Blast 9. The terms acute and chronic refer to the clinical behavior of the disease: In acute leukemias the history is usually brief and life expectancy, without treatment, is short, cells are blasts. ALL (L1, L2, L3) AML (M0 M7) In chronic leukemias the patient has been unwell for years, and survival is longer (In years). More mature cells. CML, Chronic Lymphocytic Leukemia CLL 10. Chronic Myeloid Leukemia (CML) Myeloproliferative disorders: CGL PRV Tthaemia (Granulocytes) (RBC) (Platelet) Pathogenesis: CGL results from the proliferation of the haemopoeitic pluripotent stem cell.This cell has got the capacity of unlimited proliferation for differentiation to granulocytic,megakaryocytic and RBC. 11. CML In CML the uncontrolled proliferation of the myeloid series, hence replacing or suppression of normal cells; i.e Progressive expansion of total mass of granulocytic tissue in the body. 12. Leukemoid reaction ( WBC ? CML) (Leukemia-like) e.g. WBC count 45 x 10 Inflammatory disorder & severe infection produce similar blood picture to CML. But careful history and examination should differentiate between the two possibilites. Special stain: Neutrophil alkaline phosphatase (NAP) Score: CML Score: leukamoid reaction. 13. CML Special Phenomenon: Philadelphia Chromosome (Ph.) (22- 9+) in 90% of cases. Discovered in Phil (USA). ABSENCE is the ONLY CONSISTENT sign of POOR prognosis. Ph. + CML follow a gradual pattern with a better prognosis. 14. CML CML : 15-20 % of Adult Leukemia. Median age 40-50 years. Uncommon below 18 years. Male > Female. CML Ph+: Has three phases. 1. Chronic Phase or Benign Phase. 2. Accelerated Phase. 3. Blast Phase. 15. CML (Chronic Phase) Ph+ 1. Chronic Phase: Clinical feature: Early stage, asymptomatic, 20 % chance of finding on routine blood count. Fullness of abdomen Splenomegaly: expansion of granulocytic mass,. Hyper metabolic state: Wt.loss, Night sweat, Anemia. Bleeding due to Tpenia Gouty arthritis due to high Uric Acid. O/E NAD except splenomegaly in 90% 16. CML (Chronic Phase) Ph + WBC Count < 50 x 10/l No spleen 100 Palpable 200 10cm below costal margin or normal Hb Ecchymosis, Retinal Hge Hepatosplenomegaly Sternal tenderness due to BM over expansion L N: Uncommon in Chronic Phase. 17. CML (Chronic Phase) Ph+ Peripheral Blood Picture: Neutrophilia: Peak Myelocytes + Neutrophils Basophilia + Eosinophilia Normal RBC, Plat. Giant ones. Bone Marrow: Reflection of PB Hyper cellular. Special stain: Neutrophil Alkaline Phosphatase (NAP) score. Low score: Characteristic of CML. High score: in leukomoid reaction (leukemia-like) Chronic phase: median survival 40 50 month. 18. Chronic Myelocytic Leukemia 19. CML (ACC. PHASE) CML(CHRONIC PHASE ) 20. CML (Accelerated Phase) Chronic Phase Median survival 40-50 ms. In the absence of curative treatment the disease will progress to: ACCELERATED PHASE: This is a gradual acceleration phase 6/12 Fullblown Leukemia. Gradual or Abrupt. Characterized by fever, fatigue, bone pain. Progressive painful splenomegaly, lymphadenopathy. 21. CML (Accelerated Phase) WBC difficult to control with conventional treatment (Unresponsive to therapy). > or = 20% blast in PB or BM (like acute L.) Basophelia and Eosinophilia. Anemia and Thrombocytopenia. Increase splenomegaly Myelofibrosis Newcytogenitic in addition to Ph. chrom. Median survival Ph+ 2-26 m 22. CML (Blast Crisis) 3.BLAST CRISIS Chronic Phase Ph + 40-50 m median Accelerated Phase 2-26 m survival Blast crisis 2- 6 m 30% die during accelerated phase 70% die during blast crisis 23. CML Blast crisis Symptoms of Acute Leukemia (BM failure): Anemia, TPenia, Granulcytopenia; Resulting in bleeding and infection. Extra Medullary Transformation : Skin, node, epidural, bone. Progressive Splenomegaly Malaise, Unexplained fever, > = 30 Blast (AML) Lymphoid crisis or ALL ONLY BMT can prevent evolution to Blast crisis. 24. Normal B.M. ALL 25. Treatment of CML: 1. Cytotoxic drugs to irradicate (malignant cells) 2. Blood component, if needed (RBC, platelets) 3. Splenoctomy if hugely enlarged 4. Treatment of acute leukemia (blast crisis) 5. Bone marrow and cord blood transplant( stem cell) 6. No evidence that early treatment prolong survival. 26. Management of CML Explain diagnosis and treatment patient in simple terms + short and long term risk. Support psychological, social, financial. Recently very effective drug imatinib mesylate (Gleeven, Glivec) is now the initial choice (expensive) Other cytoxic drug (Hydroxyurea) interferon. Stem cells transplant HLA matched donor from cord blood, BM or peripheral blood. HLA matched donor registery in some countries. Blast crisis and acc. Phase no posibility of maintaining patien in good health for longer time. Local irradiation. 27. Chronic Lymphocytic Leukemia (CLL) Abnormal, uncontrolled proliferation of mature B lymphocytes (cf from ALL) invading lymph node, spleen (splenomegaly and later bone marrow replacement of normal haemopoietic cells causing anaemia; (fatigue, tiredness and thrombocytopenia; bleeding (late stages) Disease of the elderly (above 45 yrs) Sex ratio male to female ratio 2:1 Mostly asymptomatic (lymphocytosis + LN) Lymphadenophy H/ spleenomegaly Infections ( due to low NAD immunoglobulin). BM failure. 28. Clinical symptoms of CLL 25% of patients are asymptomatic at diagnosis. Common symptoms can include malaise, low-grade fever, and night sweats. Weakness, fatigue, anorexia, and weight loss. Crevical and supreclavicular adenopathy. Hepatosplenomegaly is also present. 29. Laboratory data of CLL 1. Leukocytosis may be observed. The total leukocyte counts can range from 30 to 200 x 109 /L. 2. Absolute lymphocytosis is a usual finding. 3. Smudge cells are highly characteristic. 4. Serum electrophoresis studies usually show a hypogammaglobulinemia. 30. Treatment depending on the stage. - Stages of CLL: 0 lymphocytosis (10 x 10/l) persistant for 3/12. 1.lymph nodes 2.spleen 3.Hb < 10 4.Plat < 100 x 10/l 31. Chronic lymphocytic leukemia (CLL) General characteristics - The typical length of survival from the time of diagnosis is 2 to 10 years. - The principal cause of death is usually infection. - The high risk of infection in patients with CLL is the result of altered humoral (antibody) immunity caused by suppression of immunoglobulin synthesis that leads to hypogammaglobulinemia. - Patients may develop autoimmune disease eg. Autoimmune hemolytic anemia. - Classic CLL is usually a B cell disease. - Hairy-cell leukemia: B-cell chronic lymphocytic leukemia