Hematology Hematology by Wikipedia Hematology is the branch

Hematology?

Hematology by Wikipedia… • Hematology is the branch of medicine concerned with the study, diagnosis, treatment, and prevention of diseases related to the blood. Hematology includes the study of etiology. It involves treating diseases that affect the production of blood and its components, such as blood cells, Hb, blood proteins, and the mechanism of coagulation. • Physicians specialized in hematology are known as hematologists or haematologists. Their routine work mainly includes the care and treatment of patients with hematological diseases. Physicians who work in hematology laboratories, and most commonly manage them, are pathologists specialized in the diagnosis of hematological diseases, referred to as hematopathologists or haematopathologists. Hematologists and hematopathologists generally work in conjunction to formulate a diagnosis and deliver the most appropriate therapy if needed. • •

An example of hematology…

I. Historical review for CML 1845년: first report (description) of CML by John Huges Bennett -Edinb Med Surg J 1845) 1951년: the concept of MPDs (Dr. William Dameshek) CML CMPD Phladelphia chromosome t(9; 22)(q 34; q 11. 2) BCR/ABL 1 Imatinib mesylate (IM) 1960년: Philadelphia chromosome (Drs. Nowell & Hungerford) 1973년: t(9; 22)(q 34; q 11. 2): Dr. Janet D. Rowley 1985년 : BCR/ABL 1 fusion transcript was subsequently identified by two different groups 1996: the discovery of imatinib, a paradigm shift in cancer treatment The 3 most popular CML drugs before the imatinib era were busulfan (1953), hydroxyurea ( 1965), and interferon-alpha (1983), respectively. 1996년: Brian Drucker discovered imatinib mesylate 2003년: IRIS study (NEJM)

Hematopoiesis: hemato- + -poiesis 조혈[造血] Blood (Cell) Formation

조혈 장소 태아기 0 -2개월 (1 st trimester): yolk sac 2 -7 개월 (2 nd trimester): liver, spleen 5 -9 개월 (3 rd trimster): bone marrow 유아기 Bone marrow (모든 뼈) 성인 척추, 늑골, 흉골, 두개골, 골반, 천골, 대퇴골의 전단 Extramedullary hematopoiesis BM aspiration and biopsy

2. 골수 http: //imgkid. com/red-bone-marrow-structure. shtml

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Normal hematopoiesis

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BM anatomy and microenvironment • Hematopoietic stem cell require cellular and soluble growth factor support • Cellular component : surrounding bone, stromal cells and microenvironment • Microenvironment : a vast network of vascular channels or sinusoids between which float fronds of hematopoietic cells, including fat cells. • Stromal cells : fibroblastoid cells, endothelial cells, and macrophages; these cells provide cell-cell contact support to HSC and growth factors • Stem cell niche in BM : – Endosteal niche : self-renewal – Vascular niche : proliferation, differentiation, and mobilization

Blood cells • 혈액세포 – RBC: – WBC: – Platelet: oxygen delivery host defense (bacteria) hemostasis • 수명 – – – RBC: 120 days Granulocyte: 6 -8 h; days in tissue Monocyte(macrophage): weeks to years Platelet: 10 days Lymphocyte: variable, hours up to several years

Hematopoiesis (조혈) • 조혈 자극 – RBC: – WBC: – Platelet: • 생산량 : – RBC: – Platelet: – Neutrophil: tissue oxygen level noxious foreign stimuli (bacteria) blood loss, tissue damage, platelet deficiency Over 500 billion cells per day 2 billion/Kg ; 10 billion/hr 7 billion/Kg 0. 85 billion/Kg • 성숙 조혈세포 Erythrocyte, neutrophil, eosinophil, basophil, monocyte, B cell, T cell, NK cell, dendritic cell • Circulating HSC : HSC mobilization

HGF • Erythropoiesis stimulating agent : Epo • Thrombopoiesis stimulating agents : – Tpo – Second generation : Romiplostim, Eltrombopag • Leucopoiesis stimulating agents – G-CSF, GM-CSF • Neutropenia, mobilization • bone pain, rash fever, fatigue, anorexia, phlebitis, thrombosis, capillary leak syndrome • Others : – II-3, IL-6, IL-1, Pixy-321(IL-3+GM-CSF) – In vitro expansion of HSC

Clinical use of HGF • • • Transient bone marrow failure following chemotherapy Hematopoietic stem cell and progenitor cell mobilization Recovery from hematopoietic cell transplantation Myelodysplastic syndrome Aplastic anemia Some forms of neutropenia Inherited bone marrow failure syndromes HIV infection-associated neutropenia Chronic anemias (eg, renal failure, prematurity, chronic disease/inflammation, HIV infection) • Reducing the need for perioperative blood transfus

Field of Hematology • • RBC WBC Platelet Bone marrow (stem cell) Coagulation factors Stem cell transplantation Transfusion

Erythroid • Anemia – Production defect : iron deficiency anemia – Destruction : hemolytic anemia – Loss : hemorrhage • Erythrocytosis – Primary : polycythemia vera : MPN – Secondary erythrocytosis

WBC • Leucocytosis – Primary : MPN, leukemia – Secondary : leukemoid reaction • Leucopenia – Primary : AA, MDS – Secondary : drug, infection • Functional defect – Congenital – Acquired

Platelets • Thrombocytosis – Primary : MPN – Secondary : infection, Fe deficiency • Thrombocytopenia – Production : aplastic anemia – Destruction : ITP, DIC • Functional defects : – Congenital : thrombasthenia – Acquired

Bone marrow (Stem cell) • Tumors of hematopoietic and lymphoid tissues (WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues (2008)) – – Myeloid neoplasm Acute leukemia of ambiguous lineage Lymphoid neoplasm Histiocytic and dendritic cell neoplasm • Hypoplastic anemia – Idiopathic – Drug – Myelophthisia

Others • Coagulation factors – Deficiency : • Congenital • Acquired – Thrombosis • Stem cell transplantation – Allogeneic – Autologous • Transfusion and Pheresis