II. Definitions

  1. Hemolytic Anemia
    1. Premature Red Blood Cell destruction prior to their normal 120 day life span

III. Types

  1. Extravascular Hemolysis (most common)
    1. Splenic and hepatic clearance of defective RBCs
    2. Mechanisms
      1. Splenic Sequestration and Phagocytosis due to poorly deformable RBCs
      2. Antibody mediated Hemolysis by Phagocytosis or complement mediated destruction
  2. Intravascular Hemolysis (Microangiopathic Hemolytic Anemia)
    1. RBC membrane injury (Trauma, autoimmune, infection)
    2. Mechanisms
      1. Direct cellular obstruction (toxins, Trauma, lysis)
      2. Fragmentation Hemolysis by external sheearing forces
      3. Oxidative Hemolysis

IV. Pathophysiology

  1. Mechanisms of RBC destruction

VII. Symptoms

  1. See Anemia
  2. Fatigue
  3. Dyspnea on exertion
  4. Back pain
  5. Fatigue
  6. Dark Red Urine (appears as Gross Hematuria)

IX. Labs

  1. Complete Blood Count
    1. Normocytic Anemia is most common
  2. Direct Antibody Test (Direct Coombs test)
  3. Reticulocyte Index >3% (Reticulocytosis)
    1. Normal response within 3-5 days of Anemia onset
  4. Serum Haptoglobin decreased
  5. Liver Function Tests
    1. Lactate Dehydrogenase (LDH) elevated
    2. Serum Unconjugated Bilirubin (Indirect Bilirubin) elevated
  6. Urinalysis
    1. Hemosiderinuria (requires prussian blue staining)
    2. Hemoglobinuria
  7. Peripheral Smear
    1. Spherocytes (very difficult to identify on Peripheral Smear)
      1. See osmotic fragility with reflex Band 3 Protein below
      2. Congenital spherocytosis (Negative Direct Coombs)
      3. Immune Hemolytic Anemia (Positive Direct Coombs)
    2. Schistocytes
      1. Microangiopathic Anemia (eg. Heart Valve Hemolysis)
    3. Hypochromic Microcytic Anemia
      1. Thalassemia
    4. Sickle Cells
      1. Sickle Cell Anemia
    5. Bite and Blister Cells or Heinz Bodies (oxidative Hemolysis resulting in partial Phagocytosis)
      1. G6PD Deficiency
  8. Other labs
    1. G6PD Activity
    2. Radionuclide RBC survival study
    3. Hemoglobin electrophoresis
      1. Indicated for Sickle Cell Anemia or Thalassemia
    4. Infection evaluation (if fever or travel)
      1. Blood Cultures for Clostridium perfringens
      2. Babesia Serology
      3. Blood smears for Malarial Hemoglobinuria
    5. Osmotic fragility with reflex to Band 3 Protein (much more sensitive than Spherocytosis)
      1. Available as panel from Mayo
      2. Identifies Hereditary Spherocytosis

X. Diagnosis

  1. Acute Jaundice or Hematuria AND
  2. Anemia AND
  3. Other Lab criteria (see above)
    1. Reticulocytosis
    2. Lactate Dehydrogenase increased
    3. Unconjugated Bilirubin increased
    4. Serum Haptoglobin decreased

XI. Management

  1. Treat underlying cause
  2. Corticosteroids (if not contraindicated)
  3. Splenectomy (refractory to steroids)
  4. IV Immunoglobulin

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Related Studies

Ontology: Anemia, Hemolytic (C0002878)

Definition (MSHCZE) Anemie vznikající v důsledku zvýšené hemolýzy. Obecně jsou charakterizovány zvýšeným nepřímým bilirubinem v séru až ikterem, může být splenomegalie. V krevním obrazu bývá retikulocytóza, v kostní dřeni je aktivována červená krevní řada. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) Anemia resulting from the premature destruction of the peripheral blood red cells. It may be congenital or it may be caused by infections, medications, or malignancies.
Definition (CSP) due to premature destruction of erythrocytes in the spleen or peripheral vessels; commonly associated with glucose-6-phosphate dehydrogenase deficiencies, splenomegaly, and malarial or other infections.
Definition (MSH) A condition of inadequate circulating red blood cells (ANEMIA) or insufficient HEMOGLOBIN due to premature destruction of red blood cells (ERYTHROCYTES).
Concepts Disease or Syndrome (T047)
MSH D000743
ICD10 D55-D59.9 , D55-D59
SnomedCT 191231008, 191416001, 61261009
English Anemia, Hemolytic, ANAEMIA HAEMOLYTIC, ANEMIA HEMOLYTIC, Haemolytic anaemias, Haemolytic anaemias NOS, Hemolytic anemias, Hemolytic anemias NOS, Hemolytic anemia NOS, [X]Haemolytic anaemias, [X]Hemolytic anemias, Hemolytic Anemia, hemolytic anemia (diagnosis), hemolytic anemia, Anaemia haemolytic, Haemolytic anaemia NOS, Anemia hemolytic, Anemia hemolytic (NOS), Hemolytic anemia (NOS), Anemia, Hemolytic [Disease/Finding], Anaemia;haemolytic, hemolytic anemias, haemolytic anaemias, Hemolytic anemias (D55-D59), [X]Hemolytic anemias (disorder), Hemolytic anemias NOS (disorder), ANEMIA, HEMOLYTIC, HEMOLYTIC ANEMIA, Hemolytic anemia, Haemolytic anaemia, Hemolytic anemia (disorder), hemolytic; anemia, anemia; hemolytic, Hemolytic anemia, NOS, Anemia;hemolytic, haemolytic anaemia
French ANEMIE HEMOLYTIQUE, Anémie hémolytique SAI, Anémie hémolytique
Portuguese ANEMIA HEMOLITICA, Anemia hemolítica NE, Anemia hemolítica, Anemia Hemolítica
Dutch anemie hemolytisch, hemolytische anemie NAO, anemie hemolytisch (NAO), hemolytische anemie (NAO), anemie; hemolytisch, hemolytisch; anemie, hemolytische anemie, Anemie, hemolytische, Hemolytische anemie
German haemolytische Anaemie (NNB), Anaemie, haemolytisch, Anamie haemolytisch NNB, ANAEMIE HAEMOLYTISCH, Anaemie haemolytisch (NNB), haemolytische Anaemie, Anämie, hämolytische
Italian Anemia emolitica NAS, Anemia emolitica (NAS), Anemia emolitica
Spanish Anemia hemolítica (NEOM), Anemia hemolítica NEOM, ANEMIA HEMOLITICA ENZIMOPATICA, [X]anemias hemolíticas, anemias hemolíticas, SAI (trastorno), [X]anemias hemolíticas (trastorno), anemias hemolíticas, SAI, anemia hemolítica (trastorno), anemia hemolítica, Anemia hemolítica, Anemia Hemolítica
Japanese 溶血性貧血(NOS), 溶血性貧血NOS, ヨウケツセイヒンケツNOS, ヨウケツセイヒンケツ, 黄疸性貧血, 黄疸-溶血性, 後天性溶血性貧血, 貧血-溶血性-後天性, 溶血性貧血, 貧血-後天性溶血性, 溶血性黄疸, 細血管性貧血, 貧血-細血管性, エヤン-ヴィダル症候群, 貧血-溶血性
Swedish Anemi, hemolytisk
Finnish Hemolyyttinen anemia
Czech Anémie hemolytická, Hemolytická anémie NOS, Hemolytická anémie, Hemolytická anémie (NOS), anémie hemolytická, hemolytická anemie
Polish Niedokrwistość hemolityczna, Niedokrwistość hemolityczna nabyta
Hungarian haemolytikus anaemia (k.m.n.), anaemia, haemolytikus, Haemolytikus anaemia, Haemolyticus vérszegénység, haemolytikus anaemia k.m.n., anaemia haemolytica, Haemolyticus anaemia (k.m.n.)
Norwegian Hemolytisk anemi

Ontology: Hemolytic disorder (C1263988)

Concepts Disease or Syndrome (T047)
SnomedCT 157130001, 268878003, 157133004, 288278000, 128086004
English Haemolytic disease NOS, diseases haemolytic, hemolytic disorder, disorders haemolytic, disease hemolytic, hemolytic disease, haemolytic disease, Hemolytic disease, Haemolytic disease, Haemolytic disease NOS (disorder), Hemolytic disease NOS (disorder), Haemolytic disorder, Hemolytic disorder (disorder), Hemolytic disorder, disease (or disorder); hemolytic, Hemolytic disease NOS
Spanish enfermedad hemolítica, SAI (trastorno), Hemolytic disease NOS, Haemolytic disease NOS, enfermedad hemolítica, SAI, trastorno hemolítico (trastorno), trastorno hemolítico
Dutch aandoening; hemolytisch