Fifty-one patients with acute nonlymphocytic leukemia (16 with end-stage disease, 17 in second or third remission or in early relapse, and 18 in first remission) were given infusions of HLA-identical sibling marrow after cytoreduction with high doses of busulfan and cyclophosphamide. Actuarial two-year survival rates were 0 per cent, 29 per cent, and 44 per cent, respectively. Twelve patients are still alive and in remission after 327 to 1488 days, with 10 surviving beyond two years. Acute graft-versus-host disease and viral pneumonia were the major causes of death. Leukemic cells failed to clear in one patient with end-stage disease, and a relapse with meningeal leukemia occurred in another. Only one other relapse was seen--in a patient given a transplant during a third remission. Survival was favorably affected by younger age and transplantation during first remission. We conclude that high-dose chemotherapy with busulfan and cyclophosphamide, followed by allogeneic-marrow transplantation, can produce long-term remission of acute leukemia. Chemotherapy with high-dose busulfan and cyclophosphamide before transplantation provides an effective alternative to cyclophosphamide and total-body irradiation before transplantation for the treatment of acute nonlymphocytic leukemia.
Objective. To investigate a high prevalence of systemic sclerosis (SSc; scleroderma) in a well-defined population of 21,255 Choctaw Indians residing in 8 southeastern Oklahoma counties who were "users" of Indian Health Services.Methods. A case-control study of 12 SSc cases and 48 matched non-SSc controls (4 per case) was conducted to investigate potential occupational, residential, and infectious exposures, as well as genetic factors which might predispose to SSc. HLA class I1 alleles were determined by DNA oligotyping, and class I and 111 alleles were defined serologically.Results. The prevalence of SSc in full-blooded Choctaws was at least 8/1,704, or 469/100,000 (95% confidence interval 195% CI] 203-930) over the 4-year interval 1990-1994 and was significantly higher than that among non-full-blooded Choctaws (6/19,551, or 31/100,000) (P = 0.00001, odds ratio [OR] = 15.4, 95% CI 4.9-49.8). The overall prevalence of SSc in Oklahoma Choctaws (66/100,000) also was significantly higher than that in other Native Americans in Oklahoma (9.5/100,000) (P = OR = 6.95, 95% CI Supported by granls from the Sclerodcrma Federation/ United Sclerodcrma Foundation, and the RGK Foundation.
Clinical, serologic, and genetic findings in Sjögren's syndrome patients were correlated with quantitative determinations for antibody against Ro (SS‐A), La (SS‐B), and nRNP (Sm) using newly developed, sensitive solid‐phase assays. In 86 Sjögren's syndrome patient sera, more than 96% had anti‐Ro (SS‐A), and 87% had anti‐La (SS‐B), spanning a 4.8 log10 range of autoantibody concentration, whereas only 95% of the patients had anti‐nRNP (Sm). Low levels of anti‐Ro (SS‐A) and anti‐La (SS‐B) were found in 10% and 12.5%, respectively, of the 40 normal control sera. In Sjögren's syndrome patients, the level of anti‐Ro (SS‐A) correlated strongly with that of anti‐La (SS‐B) (r = 0.80; P 0.0001) but not with the level of anti‐nRNP (Sm). We found much higher levels of anti‐Ro (SS‐A) and anti‐La (SS‐B) in patients with purpura, leukopenia, lymphopenia, and increased polyclonal gamma globulins than in those without these conditions (between 4.3‐fold and 17‐fold higher; P < 0.001 to P < 0.05). Anti‐Ro (SS‐A) and anti‐La (SS‐B) levels correlated with the rheumatoid factor titer and with the concentrations of total globulin, IgG, and IgA, but not with the IgM concentration. The association of rheumatoid factor titer with levels of anti‐Ro (SS‐A) and anti‐La (SS‐B) occurred only in patients with primary Sjögren's syndrome. Antinuclear antibody titers correlated with levels of anti‐Ro (SS‐A) and anti‐nRNP (Sm). HLA‐DR3‐positive patients had higher levels of anti‐Ro (SS‐A) and anti‐La (SS‐B).
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