Etiologies of hemolytic anemia
| Intrinsic hemolysis |
| Hemoglobin disorders |
| α-Thalassemias |
| β-Thalassemias |
| Sickle cell disease |
| Unstable hemoglobins |
| Membrane defects |
| Hereditary spherocytosis |
| Hereditary elliptocytosis, pyropoikilocytosis and related disorders |
| Hereditary stomatocytosis syndromes |
| Xerocytosis |
| Hydrocytosis |
| Rh null syndrome |
| GLUT1 deficiency |
| Tangier disease |
| Abetalipoproteinemia |
| Phytosterolemia |
| Enzymopathies |
| HMP shunt abnormality |
| Glucose-6-phosphate dehydrogenase |
| Embden-Meyerhof defect (glycolysis) |
| Pyruvate kinase |
| Hexokinase |
| Glucose phosphate isomerase |
| Phosphofructokinase |
| Triosephosphate kinase |
| Phosphoglycerate kinase |
| Aldolase |
| Glutathione metabolism defect |
| 5′ nucleotidase deficiency |
| Extrinsic hemolysis |
| Immune mediated |
| Primary |
| Warm-reactive autoimmune hemolytic anemia |
| Alloimmune hemolytic anemia |
| Acute hemolytic transfusion reaction |
| Delayed hemolytic transfusion reaction |
| Drug-induced hemolytic anemias (some types) |
| Secondary |
| Autoimmune or inflammatory disorders |
| Evans syndrome |
| Primary immunodeficiency |
| Wiskott-Aldrich syndrome |
| Common variable immune deficiency |
| Acquired immunodeficiency |
| HIV infection |
| Malignancy |
| Infection |
| Posttransplant |
| Cold agglutinins |
| Primary |
| Secondary |
| Infection (e.g., Mycoplasma, Epstein-Barr virus) |
| Malignancy |
| Lymphoid |
| Nonlymphoid |
| Paroxysmal cold hemoglobinuria |
| Immune |
| Postinfectious |
| Other |
| Recluse spider venom |
| Clostridial sepsis |
| Erythrocyte fragmentation |
| Primary thrombotic microangiopathy (TMA) |
| Inherited |
| ADAMTS13 deficiency/TTP (mutations in ADAMTS13) |
| Complement-mediated (mutations in CFH, CFI, CFB, C3, CD46, etc.) |
| Metabolism mediated (MMACHC mutations) |
| Coagulation-mediated (DGKE, PLG, THBD mutations) |
| Acquired |
| TTP (autoantibody) |
| Shiga toxin-mediated TMA (SH-TTP) |
| Drug-mediated (immune mediated) |
| Drug-mediated (toxic dose-related) |
| Complement-mediated (antibody) |
| Systemic disorders |
| DIC, many causes |
| HELLP syndrome |
| Malignancy |
| Malignant hypertension |
| Scleroderma renal crisis |
| Antiphospholipid syndrome |
| Infection |
| Complicated malaria |
| Clostridia or Haemophilus influenzae type b |
| Isolated intravascular sites of hemolysis |
| Kasabach-Merritt syndrome |
| Renal artery stenosis |
| Large vessel thrombi |
| Severe aortic coarctation |
| TIPS |
| Vasculitis |
| Dysfunctional cardiac valves or cardiac assist devices |
| March hemoglobinuria, extreme running |
| Other mechanical causes |
| Heat denaturation (blood warmer, thermal burns) |
| Osmotic stress |
| Drowning |
| Mechanical trauma |
| March hemoglobinuria |
| Marathon runners, etc. |
| Direct trauma |
| “Cell saver” devices |
| Thrombectomy |
| Cardiac bypass |
| Extracorporeal membrane oxygenation |
| Dialysis |
| Hypersplenism |
| Intrinsic hemolysis |
| Hemoglobin disorders |
| α-Thalassemias |
| β-Thalassemias |
| Sickle cell disease |
| Unstable hemoglobins |
| Membrane defects |
| Hereditary spherocytosis |
| Hereditary elliptocytosis, pyropoikilocytosis and related disorders |
| Hereditary stomatocytosis syndromes |
| Xerocytosis |
| Hydrocytosis |
| Rh null syndrome |
| GLUT1 deficiency |
| Tangier disease |
| Abetalipoproteinemia |
| Phytosterolemia |
| Enzymopathies |
| HMP shunt abnormality |
| Glucose-6-phosphate dehydrogenase |
| Embden-Meyerhof defect (glycolysis) |
| Pyruvate kinase |
| Hexokinase |
| Glucose phosphate isomerase |
| Phosphofructokinase |
| Triosephosphate kinase |
| Phosphoglycerate kinase |
| Aldolase |
| Glutathione metabolism defect |
| 5′ nucleotidase deficiency |
| Extrinsic hemolysis |
| Immune mediated |
| Primary |
| Warm-reactive autoimmune hemolytic anemia |
| Alloimmune hemolytic anemia |
| Acute hemolytic transfusion reaction |
| Delayed hemolytic transfusion reaction |
| Drug-induced hemolytic anemias (some types) |
| Secondary |
| Autoimmune or inflammatory disorders |
| Evans syndrome |
| Primary immunodeficiency |
| Wiskott-Aldrich syndrome |
| Common variable immune deficiency |
| Acquired immunodeficiency |
| HIV infection |
| Malignancy |
| Infection |
| Posttransplant |
| Cold agglutinins |
| Primary |
| Secondary |
| Infection (e.g., Mycoplasma, Epstein-Barr virus) |
| Malignancy |
| Lymphoid |
| Nonlymphoid |
| Paroxysmal cold hemoglobinuria |
| Immune |
| Postinfectious |
| Other |
| Recluse spider venom |
| Clostridial sepsis |
| Erythrocyte fragmentation |
| Primary thrombotic microangiopathy (TMA) |
| Inherited |
| ADAMTS13 deficiency/TTP (mutations in ADAMTS13) |
| Complement-mediated (mutations in CFH, CFI, CFB, C3, CD46, etc.) |
| Metabolism mediated (MMACHC mutations) |
| Coagulation-mediated (DGKE, PLG, THBD mutations) |
| Acquired |
| TTP (autoantibody) |
| Shiga toxin-mediated TMA (SH-TTP) |
| Drug-mediated (immune mediated) |
| Drug-mediated (toxic dose-related) |
| Complement-mediated (antibody) |
| Systemic disorders |
| DIC, many causes |
| HELLP syndrome |
| Malignancy |
| Malignant hypertension |
| Scleroderma renal crisis |
| Antiphospholipid syndrome |
| Infection |
| Complicated malaria |
| Clostridia or Haemophilus influenzae type b |
| Isolated intravascular sites of hemolysis |
| Kasabach-Merritt syndrome |
| Renal artery stenosis |
| Large vessel thrombi |
| Severe aortic coarctation |
| TIPS |
| Vasculitis |
| Dysfunctional cardiac valves or cardiac assist devices |
| March hemoglobinuria, extreme running |
| Other mechanical causes |
| Heat denaturation (blood warmer, thermal burns) |
| Osmotic stress |
| Drowning |
| Mechanical trauma |
| March hemoglobinuria |
| Marathon runners, etc. |
| Direct trauma |
| “Cell saver” devices |
| Thrombectomy |
| Cardiac bypass |
| Extracorporeal membrane oxygenation |
| Dialysis |
| Hypersplenism |