Hematology and Oncology Book

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Autoimmune Hemolytic Anemia

Aka: Autoimmune Hemolytic Anemia, Autoimmune Hemolysis, Immune Hemolytic Anemia, AIHA, Warm Hemolysis, Cold Hemolysis, Cold Agglutinin Disease, Warm Agglutinin Disease, Warm Autoimmune Hemolytic Anemia, Cold Autoimmune Hemolytic Anemia
  1. Type: Warm Antibody (IgG mediated, more common)
    1. Mechanism
      1. IgG binds RBCs typically at Rh complex at 37 C (Body Temperature)
      2. Reticuloendothelial Macrophages ingest IgG-bound membrane
      3. Form microspherocytes which are trapped by Spleen (may result in Splenomegaly)
    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. Drug Induced Immune-Mediated Hemolysis
  2. 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 at polysaccharide antigens
      3. C3 bound RBCs are lysed on rewarming and 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
  3. Type: Paroxysmal (Cold Hemolysis mediated by IgG)
    1. See Paroxysmal Cold Hemoglobinuria
    2. Causes
      1. Tertiary Syphilis
      2. Acute Viral Infection (Mumps, Measles)
  4. Labs
    1. Direct Coombs Test positive
  5. Management
    1. General
      1. Consult with Hematology
      2. Blood product transfusions
        1. Use caution if pRBC transfusion needed
        2. Identify least cross-reacting pRBC match
    2. Cold Hemolysis
      1. Avoid cold exposure
      2. Supportive management
    3. Warm Hemolysis
      1. Corticosteroids (e.g. Prednisone 1-1.5 mg/kg/day)
      2. Splenectomy
      3. Immunosuppressants
      4. Danazol
      5. Intravenous Immunoglobulin
      6. Plasma exchange
  6. References
    1. Schreiber in Goldman (2000) Cecil Medicine, p. 876-82
    2. Dhaliwal (2004) Am Fam Physician 69:2599-606 [PubMed]
    3. Petz (2001) Curr Opin Hematol 8(6):411-6 [PubMed]
    4. Phillips (2018) Am Fam Physician 98(6): 354-61 [PubMed]

Autoimmune hemolytic anemia (C0002880)

Definition (MSHCZE) Zkr. AIHA – získaná hemolytická anemie způsobená autoprotilátkami proti membráně erytrocytů. Protilátky jsou nejč. IgG, jsou vázané na erytrocyty, popř. i volné a prokazují se Coombsovým testem. Zánik senzibilizovaných erytrocytů je zprostředkován komplementem nebo fagocytózou v retikuloendotelovém systému. Probíhá akutně zejm. u mladších osob nebo chroničtěji. AIHA je buď idiopatická, nebo symptomatická při jiném základním onemocnění např. malignity, systémové choroby. Léčebně se používají kortikoidy, imunosupresiva, danazol, někdy splenektomie, plasmaferéza (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI_NCI-GLOSS) A condition in which the body's immune system stops red blood cells from forming or causes them to clump together. Autoimmune hemolytic anemia can occur in patients who have chronic lymphocytic leukemia (CLL).
Definition (NCI) An acquired anemia caused by destruction of the red blood cells by autoantibodies. Causes include autoimmune disorders, lymphoproliferative disorders, and infections.
Definition (CSP) due to acquired antierythrocyte autoantibodies causing premature erythrocyte clearance by the spleen; antibodies can be warm or cold active; usually slow developing and treatable.
Definition (MSH) Acquired hemolytic anemia due to the presence of AUTOANTIBODIES which agglutinate or lyse the patient's own RED BLOOD CELLS.
Concepts Disease or Syndrome (T047)
MSH D000744
ICD9 283.0
SnomedCT 191209000, 25121006, 154803002, 191215000, 413603009
English Autoimmune hemolytic anemia, Anemia, Hemolytic, Autoimmune, AIHA - Autoimmune haemol anaem, AIHA - Autoimmune haemolytic anaemia, AIHA - Autoimmune hemolytic anemia, Autoimmune haemol.anaemia NOS, Autoimmune haemol.anemia NOS, Hemolytic anemia, autoimmune, ANEMIA, AUTOIMMUNE HEMOLYTIC, Autoimmun hemolytic anem, Anemia, Hemolytic, Autoimmune [Disease/Finding], autoimmune haemolytic anaemia, autoimmune haemolytic anaemias, anemia hemolytic autoimmune, hemolytic autoimmune anemia, anemia autoimmune hemolytic, autoimmune hemolytic anemia, anaemia haemolytic autoimmune, autoimmune hemolytic anemias, immunohemolytic anemia, Autoimmune haemolytic anaemia (disorder), Autoimmune hemolytic anemia NOS (disorder), Hemolytic anemia due to antibody, Haemolytic anaemia due to antibody, Autoimmune haemolytic anaemia NOS, Anemia hemolytic autoimmune, Autoimmune hemolytic anemia NOS, Autoimmune haemolytic anaemias, Anaemia haemolytic autoimmune, Anemia hemolytic autoimmune (NOS), Immune mediated hemolytic anemia, Hemolytic anemia, Autoimmune, hemolytic; anemia, autoimmune, anemia; hemolytic, autoimmune, Autoimmune hemolytic anemia, NOS, Hemolytic anemia due to antibody, NOS, Autoimmune haemolytic anaemia, Autoimmune hemolytic anemia (disorder), Autoimmune hemolytic anemias, Autoimmune Hemolytic Anemia, Anemias, Autoimmune Hemolytic, Autoimmune Hemolytic Anemias, Hemolytic Anemia, Autoimmune, Hemolytic Anemias, Autoimmune, Anemia, Autoimmune Hemolytic
Swedish Anemi, hemolytisk, autoimmun
Finnish Autoimmuunihemolyyttinen anemia
French AHAI, AHLAI, Anémie hémolytique auto immune SAI, Anémies hémolytiques autoimmunes, Anémie hémolytique autoimmune SAI, Anémie hémolytique autoimmune, Anémie hémolytique auto-immune
Russian ANEMIIA GEMOLITICHESKAIA IDIOPATICHESKAIA PRIOBRETENNAIA, ANEMIIA GEMOLITICHESKAIA AUTOIMMUNNAIA, KHOLODOVAIA AGGLIUTININOVAIA BOLEZN', АНЕМИЯ ГЕМОЛИТИЧЕСКАЯ АУТОИММУННАЯ, АНЕМИЯ ГЕМОЛИТИЧЕСКАЯ ИДИОПАТИЧЕСКАЯ ПРИОБРЕТЕННАЯ, ХОЛОДОВАЯ АГГЛЮТИНИНОВАЯ БОЛЕЗНЬ
Japanese 寒冷血球凝集素症, 特発性後天性溶血性貧血, 貧血-自己免疫性溶血性, 溶血性貧血-自己免疫性, 貧血-溶血性-特発性後天性, 自己免疫性溶血性貧血, 寒冷凝集素病, 貧血-特発性後天性溶血性, 本態性後天性溶血性貧血, 寒冷凝集素症, 貧血-溶血性-自己免疫性, 自己免疫性溶血性貧血NOS, ジコメンエキセイヨウケツセイヒンケツNOS, 自己免疫性溶血性貧血(NOS), ジコメンエキセイヨウケツセイヒンケツ
Croatian ANEMIJA, HEMOLITIČKA, AUTOIMUNA
Polish Niedokrwistość autoimmunohemolityczna, Niedokrwistość hemolityczna idiopatyczna nabyta
Czech anémie hemolytická autoimunitní, Autoimunitní hemolytická anémie, Autoimunitní hemolytická anémie (NOS), Autoimunitní hemolytická anémie NOS, Autoimunitní hemolytické anémie, AIHA, anemie hemolytická autoimunitní, autoimunitní hemolytická anemie
Norwegian Autoimmun hemolytisk anemi
Portuguese Anemia Hemolítica Autoimune, Anemias hemolíticas auto-imunes, Anemia hemolítica auto-imune NE, Anemia hemolítica auto-imune
Spanish anemia hemolítica producida por anticuerpos, anemia hemolítica autoinmunitaria, SAI (trastorno), anemia hemolítica autoinmunitaria, SAI, Anemia hemolítica autoinmune, Anemia hemolítica autoinmune (NEOM), Anemias hemolíticas autoinmunes, Anemia hemolítica autoinmune NEOM, anemia hemolítica autoinmune, anemia hemolítica autoinmunitaria (trastorno), anemia hemolítica autoinmunitaria, Anemia Hemolítica Autoinmune
Dutch anemie auto-immuunhemolytisch, auto-immune hemolytische anemie NAO, auto-immune hemolytische anemieën, auto-immuunhemolytische anemie, hemolytische auto-immune anemie (NAO), anemie; hemolytisch, auto-immuun, hemolytisch; anemie, auto-immuun, Anemie, auto-immuunhemolytische, Auto-immuunhemolytische anemie, Hemolytische anemie, auto-immuun-, Koudeagglutinineziekte
German Anaemie autoimmunhaemolytisch NNB, haemolytische Anaemie autoimmun, autoimmunhaemolytische Anaemien, Anaemie autoimmunhaemolytisch (NNB), autoimmunhaemolytische Anaemie, Anämie, autoimmunhämolytische, Autoimmunhämolytische Anämie, Hämolytische Anämie, autoimmune
Hungarian Autoimmun hemolítikus anémiák, haemolytikus autoimmun anaemia (k.m.n.), Autoimmun haemolytikus anaemiák, Haemolytikus autoimmun anaemia, Autoimmun haemolyticus anaemia, Autoimmun haemolytikus anaemia k.m.n.
Italian Anemie emolitiche autoimmunitarie, Anemia emolitica autoimmune NAS, Anemie autoimmuni emolitiche, Anemia emolitica autoimmune (NAS), Anemia emolitica autoimmune
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Warm autoimmune hemolytic anemia (C0272118)

Concepts Disease or Syndrome (T047)
SnomedCT 3978000
English AIHA - Warm autoim haemol anae, AUTOIMMUNE HEMOLYTIC ANEMIA INVOLVING WARM REACTING ANTIBODY, Warm autoimm haemol anaemia, Warm autoimm haemol anemia, warm antibody hemolytic anemia, acquired warm antibody hemolytic anemia, acquired warm antibody hemolytic anemia (diagnosis), warm antibody immunohemolytic anemia, warm autoimmune hemolytic anemia, AIHA - Warm autoimmune haemolytic anaemia, AIHA - Warm autoimmune hemolytic anemia, Warm autoimmune haemolytic anaemia, Warm autoimmune hemolytic anemia, Warm type haemolytic anaemia, Warm type hemolytic anemia, Haemolytic anaemia due to warm antibody, Hemolytic anemia due to warm antibody, Warm autoimmune hemolytic anemia (disorder), Hemolytic anemia due to warm antibody, NOS, Symptomatic warm type hemolytic anemia, Warm type autoimmune hemolytic disease
Spanish anemia hemolítica por anticuerpos autoinmunitarios calientes, Anemia hemolítica tipo tibio, anemia hemolítica por anticuerpos autoinmunitarios calientes (trastorno), anemia hemolítica por anticuerpos calientes, anemia hemolítica tipo caliente, Anemia hemolítica de anticuerpo caliente
Italian Anemia emolitica di tipo caldo
Dutch warm type hemolytische anemie, hemolytische anemie warm type
French Anémie hémolytique de type chaud, Anémie hémolytique à anticorps chauds
Japanese 温式溶血性貧血, オンシキヨウケツセイヒンケツ
Czech Hemolytická anémie s tepelnými autoprotilátkami
Hungarian Meleg típus haemolyticus anaemia, meleg típusú haemolytikus anaemia
Portuguese Anemia hemolítica a quente
German haemolytische Anaemie durch Waermeagglutinine
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Immune Hemolytic Anemia (C0852336)

Definition (NCI_NCI-GLOSS) A condition in which the body's immune system stops red blood cells from forming or causes them to clump together. Immune complex hemolytic anemia can occur in patients who have chronic lymphocytic leukemia (CLL).
Definition (NCI) An acquired anemia resulting from immune-mediated destruction of the red blood cells. Causes include autoimmune disorders, blood transfusions, and drugs.
Concepts Disease or Syndrome (T047)
Italian Anemie emolitiche autoimmuni
Japanese 免疫性溶血性貧血, メンエキセイヨウケツセイヒンケツ
Czech Imunitní hemolytické anémie
English immune hemolytic anemia, anemias hemolytic immune, anemia hemolytic immune, immune hemolytic anemias, Immune Hemolytic Anemia, Anaemias haemolytic immune, immunohemolytic anemia, immune complex hemolytic anemia, Anemias hemolytic immune
Hungarian Anaemiák haemolyticus immun
Portuguese Anemias hemolíticas imunes
Spanish Anemia hemolítica inmune
Dutch anemieën hemolytisch immunologisch
French Anémies hémolytiques auto-immunes
German Anaemien haemolytisch immun
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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