Fulminant Evans Syndrome and Splenic Infarction As Initial Manifestations of Poems Syndrome with Monoclonal Kappa Light Chain Restriction: A Diagnostic and Therapeutic Challenge.

Abstract

Introduction: Autoimmune cytopenias have been very rarely reported in conjunction with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome). To the best of our knowledge, there is no reported case in the existing literature of an association of Evans syndrome with POEMS syndrome.

Case description: A 55-year-old female patient, with a medical history of well-controlled dermatomyositis, developed fulminant autoimmune haemolytic anaemia with immune thrombocytopenic purpura, thus fulfilling the diagnosis of Evans syndrome. To control the disease, acute management required supportive measures in the intensive care unit, repeated transfusions of red blood cells and platelets, high- dose intravenous corticosteroids, a bolus of cyclophosphamide, and splenectomy. Three days post-splenectomy, the patient developed, subacutely, full-blown POEMS syndrome. Therefore, the diagnosis of coexisting secondary Evans syndrome and POEMS syndrome was made. Notably, the monoclonal kappa light chain restriction and multiple splenic infarctions were distinctive features in our patient. Due to procurement issues of lenalidomide, we opted for monthly parenteral cyclophosphamide and autologous stem cell transplantation was programmed.

Conclusion: This case highlights that severe secondary Evans syndrome can coexist with POEMS syndrome, leading to challenges in diagnosis and management.

Learning points: Severe secondary Evans syndrome can coexist with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome.High doses of corticosteroids required for the acute management of Evans syndrome may artificially lower the genuine value of serum vascular endothelial growth factor in the context of associated POEMS syndrome.In atypical circumstances like in our case (associated Evans syndrome, kappa light chain restriction), the nerve biopsy is critical to exclude differential diagnoses and confirm typical characteristics of POEMS syndrome- related polyneuropathy.