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

• Published in: European journal of haematology Vol. 70; no. 3; pp. 186 - 189
• Main Authors: Fumeaux, Zina, Beris, Photis, Borisch, Bettina, Sarasin, François P., Roosnek, Eddy, Dayer, Jean‐Michel, Chizzolini, Carlo
• Format: Journal Article
• Language: English
• Published: Oxford, UK Munksgaard International Publishers 01.03.2003; Blackwell
• Subjects: Aged; Agranulocytosis - complications; Agranulocytosis - immunology; Agranulocytosis - therapy; Autoantibodies - blood; autoimmunity; Biological and medical sciences; CD8-Positive T-Lymphocytes - immunology; CD8-Positive T-Lymphocytes - pathology; cyclosporine; Diabetes Mellitus, Type 1 - complications; Diabetes Mellitus, Type 1 - therapy; Female; glucocorticoids; Hematologic and hematopoietic diseases; Humans; Immunosuppression; Leukopenia - complications; Leukopenia - immunology; Leukopenia - therapy; Medical sciences; Muscle, Skeletal - immunology; Other diseases. Hematologic involvement in other diseases; pure white cell aplasia; Remission Induction; Thymectomy; thymoma; Thymoma - complications; Thymoma - therapy; Thyroiditis, Autoimmune - complications; Thyroiditis, Autoimmune - therapy; Endocrinopathy; Human; Thyroiditis; cyclosporine; Immunopathology; Thymoma; Thyroid diseases; Autoimmune disease; Hemopathy; Bone marrow aplasia; Glucocorticoid; Case study; Concomitant disease; Chemotherapy; Treatment; autoimmunity; Insulin dependent diabetes; pure white cell aplasia; Evolution; Female; Remission; Ciclosporin; glucocorticoids; Elderly
• ISSN: 0902-4441; 1600-0609
• DOI: 10.1034/j.1600-0609.2003.00023.x

: 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.