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血液腫瘤科 廖浚凱 Aug 15, 2012. Outline  Anemia  Blood smear.

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Presentation on theme: "血液腫瘤科 廖浚凱 Aug 15, 2012. Outline  Anemia  Blood smear."— Presentation transcript:

1 血液腫瘤科 廖浚凱 Aug 15, 2012

2 Outline  Anemia  Blood smear

3 Erythrocyte Development EPO: 90% from kidney 10% from liver

4 Hemoglobin Fe + protoporphyrin α2β2 ↓ heme + globin ↓ Hb

5 Two main approaches for anemia that are not mutually exclusive: 1. Kinetic approach 2. Morphological approach

6 Kinetic approach of Anemia Production? Survival/ Destruction? The key test is the …..

7 Reticulocyte Production Index— an estimate of marrow production relative to normal Corrected Ret= Ret(%) * Corrected Ret= Ret(%) * Hct 45 RPI= Corrected Ret Maturation index

8 RPI (kinetic approach)  RPI are the most helpful:  extremely low (<0.1%)  AA/ PRCA are the first considerations  extremely high (>3%)  Blood loss/ hemorrhage  Hemolytic anemia (although 25% AIHA have normal RPI)

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10 Morphological Approach-1 MCV>115  Vit B12, Folate  Drugs that impair DNA synthesis (AZT, chemotherapy, azathioprine)  MDS MCV  Ditto  Reticulocytosis  Hypothyroidism  Alcoholism/ liver dz

11 Morphological Approach-2 Normocytic (MCV )  Anemia of chronic disease (ACD)  Mixed deficiencies  Renal failure  BM dz (ex AA, MDS, MM…) Microcytic (MCV<80)  Iron deficiency anemia  Thalassemia  Anemia of chronic disease (ACD, 30-40%)  Sideroblastic anemia  Pb intoxification

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13 Distributio n of Iron in Adults

14 Storage iron  Circulating iron  RBC iron  Tissue iron

15 IDA Presentation PresentationDiagnosis  Progressive MCV ↘  Progressive RBC ↘  Typically high RDW  Typically high MCV/RBC  Typically mild PLT ↑  Ferritin: low (N=20-300)  Iron/TIBC <16% (N=~33%)  BM iron stain

16 Causes of IDA Increased iron requirementInadequate iron supply  Hypermenorrhea  GI blood loss– hemorrhoid, PUD, GI cancer, angiodysplasia  Factitious removal  Hemolysis  Hemodialysis  Hookworm infestation  Rapid growth of teenage  Pregnancy and lactation  Poor nutritional intake  Malabsorption  Gastrectomy (may also has Vit B12 deficiency)

17 Iron Supply  Oral: usually 3 months to restore iron storage  Ferrum chewable (Fe 3+) 100mg/tab  Hematonic (Fe 2+) 50mg/tab  IV  Atofen (!! Risk of allergy and anaphylaxis—0.6%)

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20 Harrison’s Principles of Internal Medicine 15th Ed Hb Gene

21 Thalassemia Presentation PresentationDiagnosis  Constant low MCV  Constant high RBC  Typically low RDW  Typically low MCV/RBC  Typically iron storage ↗  Exclude IDA  Hb electrophoreis:  Hb A2>3.5% or HbF>2%  β- thalassemia  HbH(+)  α-thalassemia with ¾ defect (HbH dz)  HbA2 and HbF: N  α-thalassemia with ¼ or 2/4 defect  Gene diagnosis

22 Gene Level of Thalassemia in Taiwan  α-thalassemia: Prevalence 4%  --SEAα0 deletion  -α3.7 deletion  -α4.2 deletion  HbQS or HbCS  β-thalassemia: Prevalence 2%  Codon 41/42 TCTT deletion β0  IVS II 654 C-> T point-mutation β0  Codon 17 A-> T β0  Promoter 28 A-> G β+  HbE (Codon 26 G-> A) β+  ??% α + β-thalassemia (Severity?)

23 Hb H disease ( 黃疸 ) Hydrops fetalis ( 死胎 ) 優生學的理由 β –Major ( 終身輸血 )

24 Thalassemia Treatment  Mild: None  Severe:  Folic acid  RBC transfusions + iron chelators  Allo-SCT

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27 Megaloblastic Anemia Presentation PresentationDiagnosis  High high MCV  Pancytopenia  Hypersegmented PMN  Typically high RPI, hemolysis (LDH ↗ ), indirect hyperbilirubinemia  Tinnitus or other neurological presentation  Vegetarism ( 奶, 蛋, 維他命 )  Gastrectomy or Pernicious anemia  ??Schilling test  Low Vit B12 or folic acid  Vit B12: 270~400: empirical try  Vit B12< 270: definite deficiency

28 Remember Pancytopenia  P: PNH  A: Asplatic anemia  N: Neoplasm/ Near neoplasm (including MDS)  C: Cirrhosis/ Connective tissue disease  Y: Vit B12/ Folic acid  T: Toxin/ Drug  O: Overwhelming sepsis (Hemophagocytic syndrome)/ Others 28

29 Vit B12/Folate Deficiency Anemia Treatment  Vit B12 deficiency:  Vit B12 1 mg im qd *5  q1wk *4  q1m*3  q6m  R/I pernicious anemia (Anti-parietal Ab, anti-IF Ab and PES)  Thyroid dz  Gastric cancer  Folate deficiency: Improved diet, folic acid

30 Harrison’s Principles of Internal Medicine 15th Ed Usually rapid response Most important F/U  Reticulocyte, LDH

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32 Is there hemolysis?  Damaged RBCs on smear  spherocytes (immune hemolysis, HS)  RBCs fragments (microangiopathic anemias)  Marrow response to hemolysis  polychromasia on smear  reticulocytosis  erythroid hyperplasia in marrow  Biochemical evidence of RBCs destruction  Indirect hyperbilirubinemia  increased LDH  decreased/absent haptoglobin

33 Extravascular vs Intravascular hemolysis ExtravascularIntravascular Test HemolysisHemolysis LD  bilirubin   haptoglobin N to absent absent hemoglobinuria absent present free Hb in plasma absent present urine hemosiderin absent present

34 An approach to hemolytic anemia ImmuneNon-immune CongenitalAcquired Defects of: Membrane/ skeleton (eg. Hereditary spherocytosis) Enzymes (eg. G6PD deficiency) Hemoglobinopathy PNH Infections sepsis malaria Mechanical P rosthetic heart valve Microangiopathic HA (TTP, HUS, DIC) Hypersplenism Autoimmune Alloimmune Drug-induced Hemolytic anemia

35 D/D of AIHA Warm AIHACold AIHA RBC ’ s coated with IgG, Fix complement poorly (usually C3 only) RBC ’ s coated with IgM which fixes complement Younger ageOlder age extravascular hemolysis (Spleen is primary site of destruction) Intravascular hemolysis (Liver is primary site of destruction) Spherocytes on PB smearAgglutination on PB smear 70% associated with other illnesses90% associated with other illnesses Treatment: steroids, Splenectomy Rituximab Immunosuppressants Treatment: avoidance of cold Poor response to steroids, splenectomy Rituximab Plasmapheresis in refractory cases

36 D/D of ACD and IDA ACD may concurrently coexist with true iron deficiency (In this situation, iron/TIBC is more reliable than ferritin)

37 貧血  分類:  依血球大小及形態:  紅血球偏小性貧血:海洋性貧血、缺鐵性貧血、慢性疾病相關之貧血  正常紅血球大小之貧血  紅血球偏大性貧血:維他命 B12 缺乏或葉酸缺乏性貧血、網狀紅血球增加之貧血 〈溶血急性出血〉  依製造功能  製造不足:  骨髓造血功能不足、再生不良性貧血、造血元素缺乏性貧血、缺鐵性貧血、純紅血球製造不 良、其他疾病合併骨髓侵犯  紅血球生成素〈 Erythropoietin 〉不足:腎衰竭、內分泌失調、營養 不良、 蛋白質不足  破壞或流失增加:溶血性貧血、出血、脾臟腫大性貧血  重要指標:  網狀性紅血球值〈 Reticulocyte count 〉  乳酸脫氫脢〈 LDH 〉  膽紅素〈 Bilirubin 〉  血紅素結合素〈 Heptoglobin 〉

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39 Burr cells  Altered lipid in cell membrane  artifact  Uremia  gastric CA  transfused old blood

40 Elliptocytes/ovalocytes  Abnormal cytoskeletal proteins  Hereditary elliptocytosis

41 Howell Jolly body  Nuclear remnant - DNA  hemolytic anemia  Absent or hypofunction spleen

42 Schistocyte/helmet cells  Fragmented (mechanical or phagocytosis)  DIC  TTP  HUS  Vasculitis  prosthetic heart valve  severe burns

43 Sickle cells  Molecular aggregation of Hgb-S  SS, SC, S-thal  rarely S-trait

44 NRBC  Common in newborn  severe degree of hemolysis

45 Spherocyte  Absent central pallor  look smaller  Hereditary spherocytosis  immune hemolytic anemia

46 Target cells  Increased redundancy of membrane  hemoglobinopathies  thalassemia  liver disease

47 Tear drop cells  Distorted drop shaped  Smear artifact  myelofibrosis  promyeloblastic leukemia  space occupying lesions of marrow

48 Rouleaux formation  Increased serum protein levels  Multiple myeloma

49 Red cell Agglutination

50 血球成熟之變化 性質成熟之變化 細胞 大小大->小 (megakaryoblas 除外 ) 細胞質 量 顏色 顆粒 少->多 深藍->淡藍->橘紅 ( 如 RBC) 多->少或消失 細胞核 染色質 顏色 圓,卵圓->內凹,變小->分葉->消失 細->粗 紅紫->藍紫 核 / 質比比例減少

51 血球染色特徵 細胞成分顏色細胞成分顏色 核仁 染色質 紅血球 淡藍 紫色 粉紅色 網狀紅血球顆粒 芽球細胞質 血小板 灰藍色 深藍色 紫色 初級顆粒 嗜中性顆粒 嗜酸性顆粒 嗜鹼性顆粒 毒性顆粒 紅紫色 紫色 橘紅色 黑紫色 藍黑色 Auer 小體 Cabot 氏環 Howell-Jolly 小體 Dohl 小體 紫色 鮮藍色

52 WBC series  Granulocytes  Neutrophil  Eosinophil  Basophil  Monocyte  Lymphocyte  T cell, B cell  Neutrophil  Blast  Promyelocyte  Myelocyte  Metamyelocyte  Band  Segment

53 Granulocyte 0-5%50-65%

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56 Faggot cell Auer RodsFaggot Cells

57 Pancytopenia with macrocytic anemia  Causes  megaloblastic anemia  aplastic anemia  acute leukemia  myelodysplastic syndrome  chronic liver disease (esp. liver cirrhosis)  Work-up  Serum VitB12 & folic acid  reticulocytes  bone marrow study

58 Macrocytic anemia with unmeasurable RBC  RBC & Hct : unmeasurable  RBC agglutination  Causes : autoimmune hemolytic anemia, mixed type or cold type  Work-up  peripheral blood smear  serum bilirubin (T/D), AST, LDH, haptoglobin, urine analysis, reticulocytes  Coombs’ test (direct and indirect)  ANA, VDRL, cold hemagglutinin antibody

59 有獎徵答

60   檢驗項目 檢驗值 單位 H/L 參考值  =======================================================  WBC /CMM L M F  RBC 2.06 MILON/CMM L M F  HGB 8.5 g/dL L M F12-16  HCT 25.8 % L M41-53 F36-46  MCV FL H  MCH 41.3 pg/Cell H  MCHC 32.9 g/dL  RDW-SD 97.0 % H  PLATELET /CMM H  RDW-CV 24.8 % H  SEGMENT 41.5 % L  LYMPHOCYTE 51.5 %  MONOCYTE 5.3 % 0-12  EOSINOPHIL 1.7 % 0-5  BASOPHIL 0.0 % 0-1

61 IDA

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66 Leukoerythroblastosis  Immature RBC (nucleated RBC) and myeloid cells in peripheral blood  Causes  Bone morrow disorders :  Infiltrative disorders or hematologic malignancy  Peripheral disorders :  Severe infection, major stress or operation, acute blood loss

67 Thrombocytosis  Primary: Bone marrow disease  Myeloproliferative disease  Secondary (Reactive)  Malignancy  Inflammation or infection  Connective tissue disorder  Blood loss or anemia  Splenectomy

68 THANKS


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