DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20163705

Evans syndrome with severe thrombocytopenia: a rare presentation

Rugmini Kamalamma, Siddaraju ML, Swathi Badam, Niranjan Mahankali

Abstract


Evans syndrome is a rare disease characterized by the sequential or simultaneous presence of autoimmune haemolytic anemia and immune thrombocytopenia with a chronic relapsing and remitting course. Most of them present with severe anemia and moderate thrombocytopenia while neutropenia is associated in some cases. Associated laboratory parameters include unconjugated hyperbilirubinemia, decreased total serum IgG and IgM levels, presence of non-cross reacting auto antibodies against erythrocytes, platelets and in some cases against neutrophils. Management of evans syndrome includes glucocorticosteroids as the first line therapy and IVIG as second line treatment. Resistant cases are treated with cyclosporine, mycophenolate mofetil, vincristine, danazol, rituximab (monoclonal anti-CD20 antibody), alemtuzumab (humanized monoclonal anti-CD52 antibody). Autogenic or allogenic hematopoietic stem cell transplantation (HSCT) is the only curative option available. Here we are presenting an adolescent boy with Evans syndrome who presented with severe thrombocytopenia and moderate degree of haemolytic anemia and responded well to first line oral corticosteroid therapy.


Keywords


Evans syndrome, Immunosuppressive therapy, Severe thrombocytopenia

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References


Evans RS, Takahashi K, Duane RT, Payne R, Liu C. Primary thrombocytopenic purpura and acquired haemolytic anemia: Evidence for a common etiology. AMA Arch Intern Med. 1951;87:48-65.

Porcaro F, Valenzise M, Candela G, Chiera F,Corica D , Pitrolo, E etal: Evans syndrome: A case report. Ped Med Chir Med Surg Ped. 2014;36:167-9.

Karakantza M, Moukaki A, Theodoropoulou M, Bussel JB, Maniatis A. Th 1 and Th2 cytokines in a patient with Evans’ syndrome and profound lymphopenia. British J Haematol. 2000;110:968-70.

Wang W, Herrod H, Pui CH, Presbury G, Wilimas J. Immunoregulatory abnormalities in Evans syndrome. Am J Hematol. 1983;15:381-90.

Deleze M, Oria CV, Segovia AD. Occurrence of both hemolytic anemia and thrombocytopenic purpura (evans' syndrome) in systemic lupus erythematosus: relationship to antiphospholipid antibodies. J Rheumatol. 1988;15:611-5.

Aladjidi N, Fernandes H, Leblanc T, Vareliette A, Laucat RF, Bertrand Y. Evans syndrome in children: long-term outcome in a prospective french national observational cohort. Pediatr. 2015;29(3):79.

Norton A, Roberts I. Management of evans syndrome. Br J Haematol. 2006;132(2):125-37.

Hamidah A, Thambidorai CR, Jamal R Prolonged remission after splenectomy for refractory Evans syndrome-a case report and literature review.

Dosi RV, Ambaliya AP, Patell RD, Patil RS, Shah PJ. A case report of evans syndrome. Indian J Med Sci. 2012;66:82-5.

Oyama Y, Papadopoulos EB, Miranda M, Traynor AE, Burt RK. Bone marrow transplant. Medicine Plus. 2001;28(9):903-5.