HEMOLYTIC ANEMIA OUTSIDE THE RED CELL WITHIN THE

HEMOLYTIC ANEMIA OUTSIDE THE RED CELL WITHIN THE

HEMOLYTIC ANEMIA OUTSIDE THE RED CELL WITHIN THE RED CELL 1. Membrane defects - HS AUTO-IMMUNE 1. Warm NON-IMMUNE 1. Hypersplenism - HE 2. Cold 2. Fragmentation syndromes

- grafts / valves / AS - Hereditary pyropoikilocytosis 3. Transfusion reactions - HTN / Pre-eclampsia - Hereditary stomatocytosis 4. Drug associated - March hemoglobinuria 2. Enzyme defects - MAHA - TTP/HUS -G6PD - DIC -Pyruvate kinase - hemangioma 3. Hemoglobin defects 2. Infections/Toxins (Malaria, Babeosis, Bartonella, - SCA

Clostridium welchii, snakes, - Thalassemias spiders) - Unstable hemoglobin 3. Drugs 4. Liver dz (Spur cell) HEMOLYTIC ANEMIA Intravascular Causes in Yellow OUTSIDE THE RED CELL WITHIN THE RED CELL 1. Membrane defects - HS AUTO-IMMUNE

1. Warm NON-IMMUNE 1. Hypersplenism - HE 2. Cold 2. Fragmentation syndromes - grafts / valves / AS - Hereditary pyropoikilocytosis 3. Transfusion reactions - HTN / Pre-eclampsia - Hereditary stomatocytosis 4. Drug associated - March hemoglobinuria 2. Enzyme defects - MAHA

- TTP/HUS -G6PD - DIC -Pyruvate kinase - hemangioma 3. Hemoglobin defects 2. Infections/Toxins (Malaria, Babeosis, Bartonella, - SCA Clostridium welchii, snakes, - Thalassemias spiders) - Unstable hemoglobin 3. Drugs 4. Liver dz (Spur cell)

Alf Alving, Scientist, U.S. Army (mid 1950s) RBC Extravascular Hemolysis RES HBG Fe Transferrin CO Storage and recycled in marrow

Lung Biliverdin Uncojugated bilirubin Conjugation Bile Intravascular Hemolysis RBC LYSIS HBG HAPTOGLOBIN REMOVED BY LIVER HBG TAKEN UP BY RENAL

TUBULAR CELLS HEMOGLOBINEMIA HEMOSIDERIN HEMOGLOBINURIA CELLS SLOUGHED IN URINE 1 WEEK LATER Classic Presentation 1. 2. 3. 3.

4. 5. 6. 7. New onset pallor and anemia Splenomegaly Jaundice Indirect bilirubin (<5) Reticulocyte percentage LDH (esp LDH-2) RBC life span Haptoglobin < 25 - 83% SENS - 96% SPEC

HEMOLYTIC ANEMIA OUTSIDE THE RED CELL WITHIN THE RED CELL 1. Membrane defects - HS AUTO-IMMUNE 1. Warm NON-IMMUNE 1. Hypersplenism - HE 2. Cold

2. Fragmentation syndromes - grafts / valves / AS - Hereditary pyropoikilocytosis 3. Transfusion reactions - HTN / Pre-eclampsia - Hereditary stomatocytosis 4. Drug associated - March hemoglobinuria 2. Enzyme defects - MAHA - TTP/HUS -G6PD - DIC -Pyruvate kinase - hemangioma 3. Hemoglobin defects 2. Infections/Toxins (Malaria, Babeosis, Bartonella,

- SCA Clostridium welchii, snakes, - Thalassemias spiders) - Unstable hemoglobin 3. Drugs 4. Liver dz (Spur cell) Hereditary Spherocytosis - Autosomal Dominant ~1:3000 Spectrin, Ankyrin, Protein 3 Dx: osmotic frag., neg. direct Coombs Tx: Splenectomy, cholecystectomy,

vaccine, folate Hereditary Eliptocytosis - Autosomal Dominant ~1:4500 Protein 4.1 Dx: >75% elliptic RBC Tx: Splenectomy Hereditary Stomatocytosis - Autosomal Dominant Lack of Protein 7.2 (stomatin)

Permeability to Na, K Stomatocytes, xerocytes target cells Tx: Splenectomy Stateville Penitentiary, near Joliet, IL (mid 1950s) Warm Immunohemolyis - Most common in adult women (CT, SLE) IgG bound to RBC activate phagocytes and complement Dx: + direct Coombs Tx: -

1. 2. 3. 4. 5. 6. Prednisone 1mg/kg ( RES, Ab production) Splenectomy Cyclophosphamide Azathioprine IV gamma globulin Transfusions (cross-matching impossible) Cold Immunohemolyis -

monoclonal production of cold agglutinins response to infection (M. pneumoniae, EBV) IgM mediated complement attack (no Fc) Tx: - 1. Underlying cause 2. Splenectomy, Steroids, have minimal role Otto Warburg - 1931 Nobel prize for discovery of hexosemonophosphate shunt

and glycolytic pathway (Otto Meyerhof, Gustav Embden students) - 1931-1944 discovery of cytochromes, flavin adenine dinucleotide, nicotinamide adenine dinucleotide. - 1944 offered 2nd Nobel prize, but prevented from

accepting it by Hitler (1883-1970) TTP - Ab inhibits protease that normally cleaves vWF - - 1. 2. 3. 4.

5. Intravascular hemolysis Thrombocytopenia Non focal neurologic findings Renal function Fever Dx - - Negative direct Coombs Fragmented RBC, but no spherocytes Normal coagulation tests

Tx - Plasmapheresis, glucocorticoids, dipyridamole, dextran, ASA HUS - O157:H7 Shiga-like verotoxins that damage renal vascular endothelial cells Clinically similar to TTP, no neuro manifestations Tx -

Plasmapheresis, dialysis, transfusions Role of glucocorticoids, dextran, heparin uncertain Liver disease: Spur Cell Anemia - cholesterol to phospholipid ratio Splenic traffic jam Clinically similar to TTP, no neuro manifestations Limited Treatment PNH -

Somatic mut. on X-chromosome Gene makes GPI anchor Many proteins cant attach to RBC No DAF and membrane inhibitor of reactive lysis (MIRL), RBC sensitive to complement Hypercoaguable state Dx: pancytopenia, LAP, sucrose hemolysis, Hams test (lysis in acidified serum) Tx: Transfusion, glucocorticoids, Fe BM transplant usually effective G6PD - X chromosome, recessive

11% African American males sensitivity to oxidative stress Heinz bodies, bite cells Triggers: infection, drugs, met. acidosis, moth balls, fava beans Dx: enzyme assay (fluorescent spot) after acute episode Tx: avoid triggers DRUGS THAT CAUSE HEMOLYTIC ANEMIA OXIDANT 1. Antibiotics - PENICILLIN TYPE

(Ab + DrugMembrane) nitrofurantoin 1. Penicillins sulfa 2. Cephalosporins dapsone 3. Synthetic penicillins nalidixic acid IMMUNE COMPLEX AUTOIMMUNE (Ab-Drug + Membrane) (AutoAb to Rh Ag

1. methyldopa (Most common type) 1. quinidine 2. rifampin 2. Primaquine 3. Pyridium 4. Doxorubicin 5. Methylene blue MISCELLANEOUS 1. Vitamin K (water soluble) Cinchona plant, Costa Rica, containing both quinine and quinidine, named for the Countess of the Spanish town of

Hematuria, Hemoglobinuria, and Myoglobinuria Causes of Intravascular Hemolysis 1. Transfusion reactions 2. Infections - Clostridium welchi - Malaria, Babeosis - Bartonella - Mycoplasma pneumonia 3. Fragmentation syndromes - grafts / valves / AS - HTN / Pre-eclampsia - March hemoglobinuria - TTP/HUS

- DIC - hemangioma 4. G6PD deficiency with oxidant stress 5. PNH 6. Infusion of hypotonic solutions 7. Snake and Spider venoms 8. Some autoimmune hemolytic anemias (RhoD)

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