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Minimal Requirement for Concepts of Anemia 林建廷 Dec 12, 2009.

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Presentation on theme: "Minimal Requirement for Concepts of Anemia 林建廷 Dec 12, 2009."— Presentation transcript:

1 Minimal Requirement for Concepts of Anemia 林建廷 Dec 12, 2009

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

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

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

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

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

7 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)

8

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

10 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

11 Iron and IDA

12 Distribution of Iron in Adults

13 Storage iron  Circulating iron  RBC iron  Tissue iron

14 Harrison’s Principles of Internal Medicine 15th Ed. 2001

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

16 Causes of IDA Increased iron requirement Hypermenorrhea GI blood loss– hemorrhoid, PUD, GI cancer, angiodysplasia Factitious removal Hemolysis Hemodialysis Hookworm infestation Rapid growth of teenage Pregnancy and lactation Inadequate iron supply 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%)

18 Thalassemia

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

21 Thalassemia Presentation Presentation Constant low MCV Constant high RBC Typically low RDW Typically low MCV/RBC Typically iron storage ↗ Diagnosis 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

25 Comparison of Iron Chelators

26 26 Deferasirox (Exjade) Common adverse effect: –Cre elevation (40%), (ref. 14% in DFO) –Proteinuria (18%) –GI (abd pain, N/V, diarrhea, 10-15%) –Hepatitis (8%) –Headache (15%), skin rash (11%) –No agranulocytosis, neutropenia or arthralgia Pregnancy safety: –FDA category B

27 Megaloblastic Anemia

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29 Megaloblastic Anemia Presentation Presentation 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 Diagnosis ??Schilling test Low Vit B12 or folic acid –Vit B12: 270~400: empirical try –Vit B12< 270: definite deficiency

30 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 30

31 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

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

33 Hemolytic Anemia

34 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

35 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

36 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

37 AIHA and DAT reactions IgMIgG Cold CAD ( 第二常見 ) 分為 primary 及 secondary— infection, WM, AILD, lymphoma, CLL, malignancy PCH (DL-Ab) WarmRare 最常見, 分為 Idopathic 及 secondary –lymphoma, autoimmune, viral, other malignancy, HIV Anti-IgGAnti-C3d%Note I+-35 因 RBC 上的 Ag 不夠多,無法接上補體 II++56Typical case III 可能因為 Ab 不是 IgG ,如 IgM or IgA 2.Ab 可能是 cold-reacting, ex. DL Ab (PCH) 3.Low affinity Ab IV--<1

38 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

39 Hepcidin and Anemia of Chronic Disease

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

42 ACD Treatment


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