Hematology (1) The blood and bone marrow, abnormal blood count, anemias: an overview

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1. 2. Hematology Course: an overview of Clinical Hematology Dr. Ahmed Elshebiny , MD Lecturer of Internal Medicine Faculty of Medicine, Menoufyia University Former Clinical Research Fellow, Joslin Diabetes Center, Harvard University 3. Course includes Hematology an Overview : The blood and bone marrow Clinical Hematology : The full blood count Anemia : a Clinical approach Deficiency anemias Hemolytic anemia : Acquired Hemolytic anemia : inherited Bone marrow failure syndromes ( aplastic, myeloysplastic, PNH) Hypoproliferative anemias (of chronic disease) Myeloproliferative disorders Leukemias Lymphomas & MyelomasPorphyria & Hemochromatosis Leukocyte disorders Interpretation of standard hematologic tests 4. Structure and Function The blood and bone marrow 5. Blood as a circulatory fluid A protein rich fluid called plasma in which cellular elements are suspended Carrier of O 2and CO 2 Carries nutrients Carries waste Hormones Immunity Hemostasis 6. 7. Blood is continuously renewed 8. The Bone Marrow is the blood Factory May be exposed to damage or failure 9. Spleen? Blood filter Immunity 10. Red blood cells A nucleated Diameter and thickness Membrane Metabolism 11. Red cell membrane Phospholipid bilayer Intracellular protein network 50%cholestrol Membrane proteins(glycophorin c and band 3) Reticuloproteins ( spectrin , actin, ankyrin) 12. Hemoglobin Tetramer of 4 globin chains (proteins) Each with a heme group containing iron Can be distinguished by electrophoresis Chain types Alpha Beta Gamma Delta Zeta and epsilon are embryonic 13. 14. Fetal and neonatal hemoglobins 15. HB and MCVvalues with age & sex 16. Haemopooiesis Yolk sac ( 3 rd week) Liver and spleen ( 6 wk to 7 months) Bone marrowRed marrow & yellow marrow Extramedullary ---- When? 17. 18. Aplastic Anemia 19. Bone marrow aspiration and biopsy 20. Bone marrow aspiration 21. P.N.H 22. Hematology Course (2): Clinical Hematology Abnormal Blood count 23. Manifestations of blood diseases Anemia High hemoglobin Bleeding Thrombosis Infections Lymphadenopathy Splenomegaly 24. Full blood count Common investigation Can point to hematological disorders Cornerstone of hematological diagnosis Manual or automated Automated counting and sizing by different methods e.g.( Aperture impedance) They also measure the hemoglobin byspectrophotometry Some other parameters are calculated ( MCH, MCHC) The automated analyzers can diffrentiate leukocyte 25. N.B. 5% of the normal population lie outside the normal range Race may affect the CBC Be careful of artifacts 26. Anemia Definition Morphological approach Etiological( kinetic ) approach RBCs in the blood film 27. Polycythemia True Polycythemia( Primary and secondary) Relative ( decreased plasma volume) 28. Leucocytosis Examine the differential count Examine a blood film Commonest is neutrophilia 29. WBCs 5 WBCs line ( diffrential) Neutrophils Lymphocytes MonocytesEosinophilsBasophils 30. Neutropenia Clinically evident when ANC < 500 Congenital and acquired Congenital ( e.g Kostman;s syndrome and cyclic neutropenias) Most cause of acquired neutropenia is drugs 31. Drugs associated with neutropenia Anticonvulsants---- phenytoin Antithyropid------- carbimazole Phenothiazines ------ carbamazepine Antibacterial -------- phenylbutazone Anti-inflammatory ------ co trimoxazole Cytotoxic Others ----- gold, penicillamine, imipramine 32. Neutrophil disorders Infections( viral, salmonella, protozoal, malaria) Drugs Autoimmune Alcohol Congenital Infections ( bact, fungal) Surgery, Burns Infarctions Inflammation ( gout,RA, IBD) Malignancy (CML) PhysiologicalNeutroppenia Netrophilia 33. Lymphocytic count disorders Infections( recent viral) (immunosuppressive, cortisone) Autoimmune & CT diseases Sarcoidosis Chronic renal failure Congenital : severe combined immunodifficiency Infections ( viral, pertussis,glandular fever) Lymphoproliferative ( CLL, lymphoma ) Post splenectomy Lymphopenia Lymphocytosis 34. Eosinophils Acute inflammation Steroids Catecholamines Allergy ( asthma, eczema) Infections ( Helminths, viral) Skin disease C.T disease e.g PAN Malignancy ( solid tumors and lymphomas) Gold therapy Eosinopenia Eosinophilia 35. Basophils Hyperthyroidism Inflammation ( Acute hypersensitivity, IBD) Iron difficiency Myeloproliferative disorders ( Polythycemia, CML) basopenia Basophilia ( rare) 36. Monocytosis Viral and fungal infections T.B infection C.T diseases Inflammatory bowl disease Myeloproliferative Chronic Myelomonocytic leukemia ( one of MDS) Malignancy e.g solid tumors 37. Platelets (thrombocytopenia) Spurious Increased consumption Decreased production 38. Platelets (thrombocytosis) Reactive Primary 39. Blood film Abnormal shape ( poikilocytosis) Spherocytes Target cells Polychromasia Dimorphic film Lobulation and toxic granulation A typical cells Blast cells Malaria others 40. Hematology Course (3): Clinical Hematology Anemia :a clinical approach! 41. Anemia is operationally defined as a reduction in one or more of the major RBC measurements:hemoglobin concentration, hematocrit, or RBC countKeep in mind these are all concentration measures most accurately measured by obtaining a RBC mass via isotopic dilution methods.Definition: 42. Kinetic approach to anemias Decreased erythrocyte productionDecreased erythropoietin productionInadequate marrow response to erythropoietinErythrocyte lossHemorrhageHemolysis 43. Normal blood film 44. Morphological approach 45. Reticulocytes Increased reticulocytes (greater than 2-3% or 100,000/mm3 total) are seen in blood loss and hemolytic processes, although up to 25% of hemolytic anemias will present with a normal reticulocyte count due to immune destruction of red cell precursors.Retic counts are most helpful if extremely low (