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Complete remission of pure white cell aplasia associated with thymoma, autoimmune thyroiditis and type 1 diabetes

Authors


Photis Beris MD, Hematology Division, Department of Medicine, University Hospital, 24, rue Micheli-du-Crest, 1211 Geneva 14, Switzerland
Tel: +41 22 372 3928
Fax: +41 22 372 72 88
e-mail: photis.beris@hcuge.ch

Abstract

Abstract: Pure white cell aplasia (PWCA) is a rare disorder of unknown origin, often associated with thymoma, characterized by selective neutropenia or pure agranulocytosis, and absence of granulocyte precursors in the bone marrow, but with normal erythroblasts and megakaryocytes. We report a case of PWCA associated with thymoma. Unusual findings in this case report included simultaneous presence of autoimmune thyroiditis, type 1 diabetes, anti-striated muscle antibodies, and the presence in the peripheral blood of CD8+ T cells that expressed a homogeneous naive phenotype. Neutrophil count became normal on immunosuppressive therapy after thymectomy.

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