II. Type: Warm Antibody (IgG mediated)

  1. Mechanism
    1. IgG binds RBCs at 37 C (Body Temperature)
    2. Macrophages ingest IgG-bound membrane
    3. Form microspherocytes which are trapped by Spleen
  2. Causes
    1. Idiopathic Hemolytic Anemia (most common)
    2. Non-Hodgkin's Lymphoma
    3. Chronic Lymphocytic Leukemia (CLL)
    4. Systemic Lupus Erythematosus (SLE)
    5. Human Immunodeficiency Virus (HIV)
    6. Medications
      1. See Drug-Induced Hemolytic Anemia

III. Type: Cold Antibody (IgM and complement mediated)

  1. Mechanism
    1. IgM transiently binds RBCs at 32 C (cold)
    2. Activates complement and C3 binds RBC membrane
    3. C3 bound RBCs are cleared by the liver
  2. Causes
    1. Human Immunodeficiency Virus (HIV)
    2. Mycoplasma pneumonia or other infection
    3. Infectious Mononucleosis
    4. Lymphoma
    5. Idiopathic

IV. Type: Paroxysmal (Cold Hemolysis mediated by IgG)

V. Labs

  1. Direct Coombs Test positive

VI. Management

  1. General
    1. Consult with Hematology
    2. Transfusing Blood Products
      1. Use caution if pRBC transfusion needed
      2. Identify least cross-reacting pRBC match
  2. Cold Hemolysis
    1. Avoid cold exposure
  3. Warm Hemolysis
    1. Corticosteroids (Prednisone 1-1.5 mg/kg/day)
    2. Splenectomy
    3. Immunosuppressants
    4. Danazol
    5. Intravenous Immunoglobulin
    6. Plasma exchange

VII. References

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Ontology: Cold Hemagglutinin Disease (C0175816)

Concepts Disease or Syndrome (T047)
MSH D000744
ICD10 D59.1
SnomedCT 1468004, 234382005, 398937006
English CHAD - Cold haemagglut disease, CRYOPATHIC AUTOIMMUNE HEMOLYTIC ANEMIA, COLD AGGLUTININ DIS, AIHA-Cold autoim haemol anaem, Cold autoimmun haemolyt anaem, Cold Hemagglutinin Disease, cold antibody hemolytic anemia, hemolytic anemia due to cold agglutinin disease (diagnosis), hemolytic anemia due to cold agglutinin disease, cold agglutinin disease, cold hemagglutinin disease, agglutinins cold disease, cold autoimmune hemolytic anemia, hemolytic anemia cold type, Cold Antibody Disease, Cold Antibody Hemolytic Anemia, Diseases, Cold Antibody, Anemia, Hemolytic, Cold Antibody, Disease, Cold Antibody, Cold Antibody Diseases, Cold haemagglutinin disease (disorder), Cold hemagglutinin disease, Cold agglutinin disease, Cold antibody hemolytic anemia, Cold hemolytic disease, Cryopathic hemolytic anemia, CHAD - Cold haemagglutinin disease, CHAD - Cold hemagglutinin disease, Cold antibody haemolytic anaemia, Cold haemagglutinin disease, Cold haemolytic disease, Cold type haemolytic anaemia, Cold type hemolytic anemia, Cryopathic haemolytic anaemia, AIHA - Cold autoimmune haemolytic anaemia, AIHA - Cold autoimmune hemolytic anemia, Cold autoimmune haemolytic anaemia, Cold autoimmune hemolytic anemia, Cold autoimmune hemolytic anemia (disorder), Haemolytic anaemia due to cold antibody, Hemolytic anemia due to cold antibody, Hemolytic anemia due to cold antibody, NOS, Agglutinin Disease, Cold, Agglutinin Diseases, Cold, Cold Agglutinin Disease, Cold Agglutinin Diseases, Disease, Cold Agglutinin, Diseases, Cold Agglutinin
Italian Anemia emolitica da emoagglutinine fredde, Malattia da agglutinina fredda, Malattia da agglutinine a frigore, Malattia da agglutinine fredda
Dutch koud type hemolytische anemie, koude-agglutinineziekte, hemolytische anemie koud type
French Anémie hémolytique de type froid, Maladie des agglutinines froides, Anémie hémolytique avec agglutinines froides
Japanese 冷式溶血性貧血, レイシキヨウケツセイヒンケツ, カンレイギョウシュウソショウ, 寒冷凝集素症
Spanish Enfermedad por Crioaglutininas, Enfermedad por aglutininas frías, anemia hemolítica criopática, anemia hemolítica autoinmune por crioaglutininas (trastorno), anemia hemolítica autoinmune por aglutininas frías, anemia hemolítica autoinmunitaria por crioaglutininas (trastorno), anemia hemolítica autoinmunitaria por crioaglutininas, anemia hemolítica autoinmune por crioaglutininas, anemia hemolítica autoinmune por anticuerpos fríos, anemia hemolítica tipo fría, enfermedad por aglutininas frías, anemia hemolítica por anticuerpos fríos (trastorno), hemoglobinuria por crioaglutininas, hemoglobinuria por aglutininas frías, anemia hemolítica por anticuerpos fríos, anemia hemolítica por crioaglutininas, enfermedad por crioaglutininas, Anemia hemolítica de anticuerpo frío
German Kälteagglutininkrankheit, Kaelteagglutinin-Krankheit, haemolytische Anaemie durch Kaelteagglutinine
Czech Hemolytická anémie s chladovými autoprotilátkami, Choroba studeného aglutininu, nemoc chladových aglutininů, nemoc chladových protilátek
Portuguese Doença de aglutininas frias, Doença por Crioaglutininas, Anemia hemolítica a frio
Hungarian Hideg típusú haemolyticus anaemia, Hideg agglutinin betegség, hideg típusú haemolytikus anaemia
Norwegian Kuldeagglutininsykdom, Kuldeagglutiniasjonssyndrom