Complete remission of pure white cell aplasia associated with thymoma, autoimmune thyroiditis and type 1 diabetes.
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.
Fumeaux
Zina
Z
2nd Clinic of Internal Medicine, University Hospital, Geneva, Switzerland.
Beris
Photis
P
Borisch
Bettina
B
Sarasin
François P
FP
Roosnek
Eddy
E
Dayer
Jean-Michel
JM
Chizzolini
Carlo
C
D000368:Aged; D000380:Agranulocytosis / Q000150:complications* / Q000276:immunology / Q000628:therapy; D001323:Autoantibodies / Q000097:blood; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology / Q000473:pathology; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000628:therapy; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy; D007970:Leukopenia / Q000150:complications / Q000276:immunology / Q000628:therapy; D018482:Muscle, Skeletal / Q000276:immunology; D012074:Remission Induction; D013934:Thymectomy; D013945:Thymoma / Q000150:complications* / Q000628:therapy; D013967:Thyroiditis, Autoimmune / Q000150:complications* / Q000628:therapy
D001323:Autoantibodies