A 67-year-old housewife, who had a history of rheumatoid arthritis since 1984, was admitted to the hospital in June 1988 because of lassitude, anorexia, visual and auricular disturbance, dizziness and gait disturbance for 14 months.
Physical examination showed marked anemia of the eyelids, facial “butterfly rashes”, erythrodermia over the trunk and extremities, generalized lymphadenopathy, hepatomegaly 4cm and splenomegaly 4cm, bilateral swelling of the wrists, elbows, knees and feet.
Blood count showed RBC 225×10
4/μl, Hb 6.8g/dl, WBC 16, 100/μl (Neutrophils 20%, Basophils 1%, Lymphocytes 79%) and platelets 12.3×10
4/μl. The blood film showed lymphocytosis and remarkable “rouleau formations”; lymphocytosis were present in the bone marrow. Lymphocyte surface marker studies comfirmed the B cell lymphoid nature.
The total serum protein was 10.1g/dl, with 4.8g of a significant increase of monoclonal globulin: IgM kappa chain. Serological tests for C-reactive protein were 12.3mg/dl, erythrocyte sedimentation-rate (E.S.R.) 187mm/h, the rheumatoid factor strongly positive, the rheumatoid arthritis hemagglutination test 10, 485, 760, the antinuclear antibody (ANA) 40, two lupus erythematosus (LE) preps negative.
A lymph node biopsy revealed non-Hodgkin's lymphoma; diffuse, mixed type (LSG classification). Lymphocyte surface marker studies confirmed the T cell lymphoid nature; OKT-3 70.8%, OKT-4 59.2%.
She was treated with mild chemotherapy (MEPA combination chemotherapy) and plasmapheresis and interferon therapy. Two months later she had a complete remission of T-cell lymphoma. She developed fulminant hepatitis and died in March 1989.
Rearrangements of the beta chain of the T-cell receptor gene and the immunoglobulin gene by Southern Blot Analysis were not detected in lymph node cells and peripheral blood lymphocytes.
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