Published: 2016-12-24

Laboratory work-up and its translational significance in cold agglutinin syndrome

Manish Raturi, Shamee Shastry


Autoimmune hemolytic anemia (AIHA) is characterized by the presence of agglutinins directed against autologous erythrocytes causing their reduced survival. Approximately 20% of AIHA are associated with cold-reactive antibodies. About half of patients are termed idiopathic without any underlying causes. Secondary cases are associated with underlying diseases or with certain drugs. We report herein a case of idiopathic cold autoimmune hemolytic anemia in a 65 year old lady without any underlying cause who presented with grouping discrepancy and responded well to treatment. Blood transfusion was completely avoided by keeping her warm and other conservative management including hematinic. At the time of discharge from the hospital her haematocrit reached 24%. Patient now is being followed as an outpatient and she continues to work daily albeit acro-cyanosis occurs sometimes on exposure to cold water. To our knowledge a careful communication between the transfusion services and clinicians can avoid unnecessary blood transfusions in such cases especially in clinically responding patients.


Cold autoimmune haemolytic anaemia, Acro-cyanosis, Hematinic

Full Text:



Khosroshahi BN, Mohammad J, Hossein V, Ahmadi A, Keivan S, Mahdi K. Cold Autoimmune Hemolytic Anemia due to High-grade non-Hodgkin’s B cell Lymphoma with Weak Response to Rituximab and Chemotherapy Regimens. Int J Hematol Oncol Stem Cell Res. 2015;9(3):157-60.

Gehrs BC, Friedberg RC. Autoimmune hemolytic anemia. American Journal of Hematology. 2002: 258-71.

Petz L, Garratty G. Immune Hemolytic Anemias: Second Edition. 2nd ed. Immune Hemolytic Anemias. Philadelphia. 2004:1-591.

McNicholl FP. Clinical syndromes associated with cold agglutinins. Transfus Sci. 2000;22(1-2):125-33.

Roelcke D. Cold agglutination. Transfus Med Rev. 1989;3(2):140-66.

Swiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood. 2013:1114-21.

Marcus DM. A review of the immunogenic and immuno modulatory properties of glycosphingolipids. Mol Immunol. 1984;21(11):1083-91.

Gertz MA. Cold hemolytic syndrome. Hematol/ Educ Progr Am Soc Hematol. 2006;19-23.

Silberstein LE, Berkman EM, Schreiber AD. Cold hemagglutinin disease associated with IgG cold-reactive antibody. Ann Intern Med. 1987;106(2):238-42.

Donham JA, Denning V. Cold agglutinin syndrome: Nursing management. Hear Lung J Acute Crit Care. 1985;14(1):59-67.