Autoimmune hemolytic anemia classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assosciate Editor(s)-In-Chief: Prashanth Saddala M.B.B.S; Shyam Patel [2], Irfan Dotani [3]

Overview

Autoimmune hemolytic anemia is classified into 3 broad categories. These include warm-antibody type, cold-antibody type, and mixed-antibody type. Each category is characterized by a different autoantibody (IgG or IgM) and different optimal binding temperatures (37 degrees Celsius or 4-18 degrees Celsius). Each condition is associated with different triggers, including infections, medications, and malignancies. The warm-antibody type is the most common, and the mixed-antibody type is rare and not well characterized.

Classification


Category	Features	Subtypes

Warm-antibody type	Accounts for ~75% of cases of autoimmune hemolytic anemia Due to IgG autoantibody^[1] Binding occurs optimally at 37 degrees Celsius Extravascular hemolysis (reticuloendothelial system)	Idiopathic Systemic lupus erythematosus Evans’ syndrome^[2] Chronic lymphocytic leukemia Non-Hodgkin lymphoma Drugs (methyldopa)
Cold-antibody type	Accounts for ~25% of cases of autoimmune hemolytic anemia Due to IgM autoantibody Binding occurs optimally at 4-18 degrees Celsius Intravascular hemolysis Recognition by the reticuloendothelial system Destruction by macrophages	Cold agglutinin disease^[3] Paroxysmal cold hemoglobinuria^[4] Mycoplasma infection^[5] Malignancy
Mixed warm-antibody and cold-antibody type	Due to IgM subtype Mixed features	Systemic lupus erythematosus Various clinical entities Generally rare

References

↑ Palla AR, Khimani F, Craig MD (2013). “Warm Autoimmune Hemolytic Anemia with a Direct Antiglobulin Test Positive for C3 and Negative for IgG: A Case Study and Analytical Literature Review of Incidence and Severity”. Clin Med Insights Case Rep. 6: 57–60. doi:10.4137/CCRep.S11469. PMC 3623608. PMID 23645992.
↑ Bechir A, Haifa R, Nesrine BS, Emna B, Senda M, Asma A; et al. (2016). “Multiple myeloma associated with an Evan’s syndrome”. Pan Afr Med J. 25: 127. doi:10.11604/pamj.2016.25.127.10750. PMC 5325491. PMID 28292089.
↑ Randen U, Trøen G, Tierens A, Steen C, Warsame A, Beiske K; et al. (2014). “Primary cold agglutinin-associated lymphoproliferative disease: a B-cell lymphoma of the bone marrow distinct from lymphoplasmacytic lymphoma”. Haematologica. 99 (3): 497–504. doi:10.3324/haematol.2013.091702. PMC 3943313. PMID 24143001.
↑ Moncrieff RE (1975). “Paroxysmal cold hemoglobinuria-1975”. West J Med. 123 (6): 477. PMC 1130418. PMID 18747599.
↑ Packman CH (2015). “The Clinical Pictures of Autoimmune Hemolytic Anemia”. Transfus Med Hemother. 42 (5): 317–24. doi:10.1159/000440656. PMC 4678314. PMID 26696800.

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[pmid23645992-1] Palla AR, Khimani F, Craig MD (2013). “Warm Autoimmune Hemolytic Anemia with a Direct Antiglobulin Test Positive for C3 and Negative for IgG: A Case Study and Analytical Literature Review of Incidence and Severity”. Clin Med Insights Case Rep. 6: 57–60. doi:10.4137/CCRep.S11469. PMC 3623608. PMID 23645992.

[pmid28292089-2] Bechir A, Haifa R, Nesrine BS, Emna B, Senda M, Asma A; et al. (2016). “Multiple myeloma associated with an Evan’s syndrome”. Pan Afr Med J. 25: 127. doi:10.11604/pamj.2016.25.127.10750. PMC 5325491. PMID 28292089.

[pmid24143001-3] Randen U, Trøen G, Tierens A, Steen C, Warsame A, Beiske K; et al. (2014). “Primary cold agglutinin-associated lymphoproliferative disease: a B-cell lymphoma of the bone marrow distinct from lymphoplasmacytic lymphoma”. Haematologica. 99 (3): 497–504. doi:10.3324/haematol.2013.091702. PMC 3943313. PMID 24143001.

[pmid18747599-4] Moncrieff RE (1975). “Paroxysmal cold hemoglobinuria-1975”. West J Med. 123 (6): 477. PMC 1130418. PMID 18747599.

[pmid26696800-5] Packman CH (2015). “The Clinical Pictures of Autoimmune Hemolytic Anemia”. Transfus Med Hemother. 42 (5): 317–24. doi:10.1159/000440656. PMC 4678314. PMID 26696800.

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