Evan’s syndrome a strange cause of hemolysis: a case report

Hari Babu Ramineni, Chandini M, Sri Vidya M, Narendra Babu K, Vidyadhara S

Abstract


Evan’s Syndrome is an autoimmune disorder characterized by autoimmune hemolytic anemia and Idiopathic Thrombocytopenia Purpura (ITP) or immune neutropenia in absence of any cause. We report this rare autoimmune disorder in a 37 year old female where she had presented with complaints of bleeding per vagina since 7 days with clots, non painful, bilateral joint pain since 2 days, swelling of foot & puffiness of face since 1 day, shortness of breath and pallor. Based on her past history and laboratory investigations confirmed the diagnosis of Evan’ Syndrome. Therapy was initiated with corticosteroids and IV immunoglobulin.


Keywords


Idiopathic thrombocytopenia purpura, Thrombocytopenia, Autoimmune hemolytic anemia

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References


Michel M, Chanet V, Dechartres A, Morin AS, Piette JC, Cirasino L. The spectrum of Evan’s Syndrome in adults; new insight into the disease based on the analysis of 68 cases. Blood. 2009;114(15):3167-72.

Dhingra KK, Jain D, Mandal S, Khurana N, Singh T, Gupta N. Evan’s syndrome: a study of six cases with review of literature. Hematology. 2008;13(6):356-60.

Savaşan S, Warrier I, Ravindranath Y. The Spectrum of Evan’s Syndrome. Arch Dis Child. 1997;77(3):245-8.

Teachey DT1, Manno CS, Axsom KM, Andrews T, Choi JK, Greenbaum BH,et al Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). Blood. 2005;105(6):2443-8.

Costallat GL, Appenzeller S, Costallat LT. Evans syndrome and Systemic Lupus Erythematosus: Clinical presentation and outcome of Evans syndrome and Systemic Lupus Erythematosus: Clinical presentation and outcome: Joint Bone Spine;79(4):362-4.

Janić D, Krivokapić-Dokmanović L, Jovanović N, Lazić J, Rodić P, Janković S.. Glucocorticoid-resistant Evans' syndrome successfully controlled with low-dose cyclosporine. Int J Clin Pharmacol Ther. 2011;49(10):622-5.

Mathew P, Chen G, Wang W. Evans syndrome: results of a national survey. J Pediatr Hematol Oncol. 1997;19(5):433-7.