T-gamma-lymphoproliferative disorder arising in a background of autoimmune disease and terminating in plasma cell dyscrasia with primary amyloidosis.
T-gamma-lymphoproliferative disorder, a syndrome of T-cell lymphocytosis with neutropenia has been described in patients with various autoimmune disorders, especially rheumatoid arthritis. We report a case of T-gamma-lymphoproliferative disorder occurring in a 42-year-old white woman with a long history of dermatitis herpetiformis and subsequent development of Coomb's positive autoimmune hemolytic anemia and polymyositis. The peripheral blood lymphocytes showed the T-suppressor cell phenotype (CD2-, CD3-, CD8-, and CD4-). DNA analysis of the peripheral blood lymphocytes revealed a T-cell receptor beta-chain gene rearrangement and an immunoglobulin heavy-chain gene rearrangement. The patient's course was marked by numerous bouts of infection. The unique factor in this patient was the development of a plasma cell dyscrasia and amyloidosis prior to death.
Amparo
E
E
Department of Clinical Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, Calif.
Kaplan
L
L
Rosenbloom
B
B
Lee
S
S
D000328:Adult; D000686:Amyloidosis / Q000150:complications / Q000473:pathology*; D001327:Autoimmune Diseases / Q000150:complications / Q000473:pathology*; D003874:Dermatitis Herpetiformis / Q000150:complications / Q000473:pathology*; D005260:Female; D006333:Heart Failure / Q000150:complications / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000473:pathology; D007142:Immunoglobulin gamma-Chains / Q000276:immunology; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000473:pathology*; D009220:Myositis / Q000150:complications / Q000473:pathology; D010265:Paraproteinemias / Q000150:complications / Q000473:pathology*; D013601:T-Lymphocytes / Q000276:immunology
D007142:Immunoglobulin gamma-Chains