Hard-To-Treat Idiopathic Refractory Autoimmune Haemolytic Anaemia with Reticulocytopenia
KeywordsHaemolytic anaemia, reticulocytopenia, azathioprine
Autoimmune haemolytic anaemia (AIHA) is an uncommon condition characterized by increased destruction of erythrocytes associated with reticulocytosis in the great majority of cases. We present the case of a 68-year-old woman with jaundice and malaise. Investigation revealed AIHA with reticulocytopenia. The patient failed to respond to prednisolone or to rituximab. Azathioprine and epoetin beta were subsequently started, the prednisolone dose was increased, and the patient began to respond after 1 month. In AIHA, reticulocytopenia is a very rare presentation and a sign of great severity and poor outcome. The scarcity of therapeutic options in refractory cases poses a major challenge for physicians.
Hill QA, Stamps R, Massey E, Grainger JD, Provan D, Hill A. The diagnosis and management of primary autoimmune haemolytic anaemia. Br J Haematol 2017;176(3):395–411.
Go RS, Winters JL, Kay NE. How I treat autoimmune hemolytic anemia. Blood 2017;129(22):2971–2979.
Petz LD, Garratty G. Immune hemolytic anemias. In: Petz LD, Garratty G, editors. Immune hemolytic anemias. 2nd ed. Philadelphia: Churchill Livingstone; 2004, p. iv.
Engelfriet CP, Overbeeke MA, von dem Borne AE. Autoimmune hemolytic anemia. Semin Hematol 1992;29(1):3–12.
Barcellini W, Fattizzo B. Clinical applications of hemolytic markers in the differential diagnosis and management of hemolytic anemia. Dis Markers 2015;2015:635670.
Liesveld JL, Rowe JM, Lichtman MA. Variability of the erythropoietic response in autoimmune hemolytic anemia: analysis of 109 cases. Blood 1987;69(3):820–826.
Conley CL, Lippman SM, Ness P. Autoimmune hemolytic anemia with reticulocytopenia: a medical emergency. JAMA 1980;244(15):1688–1690.
Fattizzo B, Zaninoni A, Nesa F, Sciumbata VM, Zanella A, Cortelezzi A, et al. Lessons from very severe, refractory, and fatal primary autoimmune hemolytic anemias. Am J Hematol 2015;90(8):E149–151.
Barcellini W, Fattizzo B, Zaninoni A, Radice T, Nichele I, Di Bona E, et al. Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients. Blood 2014;124(19):2930–2936.
Worlledge S, Brain M, Cooper A, Hobbs J, Dacie J. Immunosuppressive drugs in the treatment of autoimmune haemolytic anemia. Proc R Soc Med 1969;61:1312–1315.
Arbach O, Funck R, Seibt F, Salama A. Erythropoietin may improve anemia in patients with autoimmune hemolytic anemia associated with reticulocytopenia. Transfus Med Hemother 2012;39(3):221–223.
Horning K, Schmidt C. Azathioprine-induced rapid hepatotoxicity. J Pharm Technol 2014;30(1):18–20.
Huskisson EC. Azathioprine. Clin Rheum Dis 1984;10(2):325–332.
Bennett CL, Silver SM, Djulbegovic B, Samaras AT, Blau CA, Gleason KJ, et al. Venous thromboembolism and mortality associated with recombinant erythropoietin and darbepoetin administration for the treatment of cancer-associated anemia. JAMA 2008;299(8):914–924.
Issue: Vol 8 No 1 (view)