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Hematological Diseases

Hematological Diseases. A. Apirakaramwong, Ph.D. Department of Biopharmacy, Faculty of Pharmacy, Silpakorn University. Learning Objectives. State concepts of hematopoiesis and describe how hematopoiesis will change in abnormal states

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Hematological Diseases

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  1. Hematological Diseases A. Apirakaramwong, Ph.D. Department of Biopharmacy, Faculty of Pharmacy, Silpakorn University

  2. Learning Objectives • State concepts of hematopoiesis and describe how hematopoiesis will change in abnormal states • Describe types, causes, and pathophysilology of some importance disorders of red cells, white cells as well as pletelets • Explain how to identify such importance disorders by gathering data from laboratory tests as a role of pharmacists • State main symptoms and sign, also suggest suitable therapy for those disorders

  3. Hematopoiesis • Pluripotent stem cell = Myeloid stem cell + Lymphoid stem cell • Myeloid stem cell = erythrocyte, granulocyte, megakaryocyte • Lymphoid stem cell = B, T lymphocyte • Cytokines : ILs, CSF, EPO, TPO • รูปที่1

  4. Pluripotent stem cell SCF, IL-6, Flt3L Myeloid stem cell Lymphoid stem cell IL-3, IL-6, GM-CSF SCF, IL-7, Flt3L IL-5 GM-CSF TPO, IL-11 Pro-T E/Mega Pro-NK Pro-B CFU-Eo CFU-E CFU-G/M CFU-G CFU-Mega Monoblast Myeloblast CFU-M Megakaryoblast Proerythroblast IL-5 M-CSF G-CSF Flt3L IL-15 IL-7 TPO EPO Eosinophiloblast Plasma cell NK cell T cell Eosinophil Monocyte/Macrophage PMN Platelet Erythrocyte Hematopoiesis

  5. Bands Reticulocyte Howard,MR, and Hamilton, PJ, p.3, 2002.

  6. Peripheral thin blood smear (Wright stain) Neutrophil Eosinophil Platelete Neutrophil Lymphocyte Basophil Monocyte http://www-medlib.med.utah.edu/WebPath/HEMEHTML/HEME100.html

  7. Normal values- formed elements of blood(1) Morphology of Blood cell - Blood smear ( thin film) ดัดแปลงจาก Hutson,PR, p. 443, 2004 และ Lichtman, MA, et. al., p. 3-4, 2003

  8. RI and Anemia

  9. Normal values- formed elements of blood (2) • ESR: RBC เกาะกลุ่ม (agglutination) มีหลายภาวะที่มีผลเพิ่ม/ลด ESR (ตารางที่ 3 หน้า 9) eg.ESR  ในภาวะ macrocytosis (volume ของ RBC ) • Platelet (/microliter) :1.5-4.5 แสน • platelet เมื่อมีชิ้นส่วน RBC หรือ microcytic RBC •  เมื่อมี platelet clump • Serum ferritin: • Fe (store in 1. ferritin ซึ่งอยู่ใน macrophage and 2. Hemosiderin in cells ; transfer by transferrin) • serum ferritin ชี้ภาวะการขาด Fe (IDA)

  10. Normal values- formed elements of blood (3) • Serum iron, Total iron-binding capacity (TICB): • serum Fe วัด Fe-transferrin • serum iron = 1/3 TICB • IDA ค่า TICB จะเพิ่มเพราะสังเคราะห์ transferrin เพิ่ม ค่า %saturation transferrin จะลด เช่น • serum iron = 100 mcg/dL TICB = 300 mcg/dL • % sat trans = (100/300) x100 = 33 • ถ้า % sat trans < 15 % ---> IDA

  11. Normal values- formed elements of blood (4) • WBC diffential: • Granulocytes • Neutrophils : • seg, PMN, polys (หลาย lobes): ไขกระดูก--> endothelium until stimulated (ติดเชื้อ, glucocorticoid) • bans, stabs • Eosinophils/basophils : eos/parasite; baso/allergy • Monocytes/macrophages: T,B cell function • Lymphocytes (T, B, NK cells)

  12. Terms in blood smear (ตาราง 4 หน้า 12)

  13. Red cell Disorders • Anemia • 1. Acute/chronic Blood loss • 2. Hemolytic anemias: • Hereditary Spherocytosis • hemoglobinopathies: Sickle cell anemia, Thalassemias, • red cell enzyme defects: G6PD deficiency • Trauma • 3. Impaired production: Megaloblastic anemia, Iron deficiency anemia (IDA), anemia from chronic diseases(ACD) • 4. Aplastic (hypoplastic) anemia • Polycythemia

  14. White blood cell disorders • Leukopenia: • Neutrophenia • Lymphocytopenia • Leukocytosis • Granulocytosis • Lymphocytosis • Neoplastic proliferations of white cells • Malignant lymphoma • Leukemias

  15. Quantitative disorders thrombocytopenia (40,000/ mL) thrombocytosis (1,000,000/ mL ) Qualitative disorders Inherited Acquired Platelet disorders

  16. Coagulation disorders • Hemophilias • Coagulation disorders • Von Willebrand disease • Vitamin K deficiency • DIC • Afrinogenemia • Liver diseases

  17. Red cells • Production (erythropoiesis) • 120 days • produce in bone marrow: 1 week • renal interstitial tubular cell :EPO(stimulated by hypoxia) • hemoglobin: HbA (alpha2 + beta2) • HbA2 (alpha2 + delta 2), HbF (alpha2 + gamma2) • Fe (80%) =Hb; (13%) =Mb ; rest = ferritin and hemosiderin; absorption in heme iron (Fe 2+) or noneheme iron with vit C • produce rapidly when Vit B12, folate and Fe adequate

  18. Red cells (cont.) • Membrane • elasticity, deformibility, ATP from glycolysis , NADPH (PPP) & glutathione • spectrin-actin, ankyrin • Degradation • spleen : phagocytosis • Hb--heme oxygenase-->Fe, biliverdin--> bilirubin --> bilt salt

  19. Red cell membrane Aster J., p 399, 2003

  20. Anemia • จำนวน, MCV  • รูปร่าง ลักษณะ • Microcytic • Macrocytic • Poikilocytosis (shape) • Anisocytosis (size) • การติดสี • Hypochromic • Hyperchromic http://www-medlib.med.utah.edu/WebPath/HEMEHTML

  21. Anemia Hb or Hct < normal range hypoxia compensation by EPO, erythropoiesis, cardiac output อาการ: ซีดอ่อนเพลียเป็นลมหน้ามืดใจสั่นปวดศีรษะหอบไวต่อความเย็น ส่วนอาการอื่นๆขึ้นกับสาเหตุเช่นkailonychia (เล็บช้อน) ในIDA; jaundice in hemolytic anemia; bone demormities in thalassemia จำแนกชนิดตามตารางที่5 หน้า16

  22. Classification of Anemia • Anemia of blood loss • Hemolytic anemia • Hereditary Spherocytosis (HS) • Hemoglobinopathies: sickle cell anemia and thalassemia syndrome • Red cell enzyme defect (G6PD) • Trauma to red cells • Impaired of red cell production • Iron deficiency anemia (IDA) • Megaloblastic anemia • Anemia of chronic disease • Aplastic anemia

  23. Anemia of blood loss • Acute or chronic blood loss (3-4 wk to recover) • Iron deficiency anemia • Malnutrition or pregnancy

  24. Hemolytic anemia • Normocytic, normochromic • Bilirubin  • Erythropoiesis lead toreticulocyte • Intrinsic (Intracorpuscular): defect of RBC membrane, Hb, metabolism, enzyme • Extrinsic : membrane defect: autoimmune, oxidants

  25. Hemolytic anemia • อาการเลือดจางรวดเร็วดีซ่านตรวจไม่พบbile ในปัสสาวะ • ชนิดเฉียบพลันHgb ลดลง 1 g/dL ต่อ 1 สัปดาห์ • ใน G6PD, Thalassemia, HS อาจถูกกระตุ้น โดยไข้ติดเชื้อยาหรือสารเคมี hemolysis crisis • ชนิดเรื้อรังมีซีดเพลียยาวนานเช่นbeta-thalassemia • อาจพบม้ามโต, hemoglobinuria, นิ่วในถุงน้ำดีหรือbone deformities

  26. Treatment • กำจัดสาเหตุที่ทำให้RBC แตก • แก้ไขcomplication เช่นhyperkalemia, kidney failure • รักษาตามอาการ เช่นให้เลือด (hypertransfusion) • ใช้ยาบางกรณี เช่นantimalarial agents • ควรให้folic acid supplement : 5 mg/d

  27. Hemolytic anemia • Hereditary Spherocytosis (HS) • Hemoglobinopathies • sickle cell anemia • thalassemia syndrome • Red cell enzyme defect (G6PD) • Trauma to red cells

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