Summary
In 1960 Rabinowitz and Dameshek emphasized the close relationship between idiopathic thrombocytopenic purpura (ITP) and systemic lupus erythematosus (SLE) and suggested that ITP is often a prodrome of SLE. On the basis of tis observation, the present study was designed to investigate the prevalence of SLE in patients with the initial diagnosis of ITP, autoimmune hemolytic anemia (AIHA), Fisher-Evans' syndrome (F-E) and idiopathic aplastic bone marrow (IABM) and to investigate the salient clinical manifestations and prognosis of these patients. We studied 62 patients, 35 with ITP, 16 with AIHA, 9 with F-E and 2 with IABM. Seventeen developed four or more ARA criteria for SLE within 6 of 14 years after the initial hematologic manfestations (IHM). Mucocutaneous symptoms predominated in all groups. None developed renal failure and only 2 had central nervous system involvement. When these patients were matched by age, sex and duration of illness with 24 SLE patients without hematologic abnormalities, the everity and therapeutic indices of the former showed a significantly (p 0.001) milder course. Eight of the 9 patients that required splenectomy are at present without treatment. SLE patient with IHM have a more benign course which is not worsened by splenectomy.
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Alger, M., Alarcon-Segovia, D., Rivero, J.S. Hemolytic anemia and thrombocytopenic purpura; Two related subsets of systemic lupus erythematosus. J. Rheumatol. 1977, 4, 351–357.
Baldini, M. Idiopathic thrombocytopenic purpura. N Engl. J. Med. 1966, 274, 1360–1367.
Breckenridge, R.T., Moore, R.D., Ratnoff O. A. Study of thrombocytopenia. New histologic criteria for the differentiation of idiopathic thrombocytopenia and thrombocytopenia associated with disseminated lupus erythematosus. Blood. 1967, 30, 39–53.
Conley, C.L. Immunologic precursors of autoimmune hematologic disorders. Autoimmune hemolytic anemia and thrombocytopenic purpura. Johns Hopkins Med. J. 1981, 149, 101–109.
Doan, C.A., Bouroncle, B.A., Wiseman, B.K. Idiopathic and secondary thrombocytopenic purpura: clinical study and evaluation of 381 cases over a period of 28 years. Ann. Intern. Med. 1960, 53, 861–876.
Donadio, J.V., Burgess, J.H., Holley, K.E. Membranous lupus nephropathy. A clinicopathologic study. Medicine. 1977, 56, 527–536.
Favre, H. Autoimmune hematologic diseases associated with infraclinical systemic lupus erythematosus in four patients. A human equivalent of the NZB syndrome. Am. J. Med. 1979, 66, 91–95.
Fries, J.F., Holman, H.R. Systemic lupus erythematosus: A clinical analysis. W.B. Saunders Company, Philadelphia. 1975, 7,15,16,79,83.
Harvey, A., McG., Shulman L.E., Tumulty, P.A., Conley, Cl., Schoernrich, E.H. Systemic lupus erythematosus: review of the literature and clinical analysis of 138 cases. Medicine. 1954, 33, 291.
Lavalle, M.C., Nuñez, A., Muñoz, E. Anti-DNA antibodies by the Farr and the Crithidia luciliae assays and the clinical status of systemic lupus erythematosus. Rev. Invest. Clin. 1981, 33, 349–354.
McIntosh, S., Johnson, Ch., Hartigan, P., Baumgarten, A., Dwyer, J.M. Immunoregulatory abnormalities in children with thrombocytopenic purpura. J. Pediatr. 1981, 99, 525–530.
Powers, R., Akisuki, M., Boehm-Tructt, M., Daly, V., Holman, H.R. Substantial purification of the Sm antigen and association of high titer antibody to Sm with a clinical subset of systemic lupus erythematosus by antibodies to the Sm antigen. Arthritis Rheum. 1979, 22, 1334–1337.
Rabinowitz, Y., Dameshek, W. Systemic lupus erythematosus after “Idiopatic” thrombocytopenic purpura: A Review. Ann. Intern. Med. 1960, 52, 1–28.
Reinitz, E., Grayzel, A., Barland, P. Specificity of Sm antibody. Arthritis Rheum. 1980, 23, 869.
Reyes, P.A., Lavalle, M.C. Clinical subsets of systemic lupus erythematosus. Arthritis Rheum. 1980, 23, 1064–1065.
Sergent, J.S., Lockshin M.D., Klempner M.S., Lipsky B.A. Central nervous system disease in systemic lupus erythematosus. Therapy and prognosis. Am. J. Med. 1975, 58, 644–655.
Winn, D.M., Wolfe, F.J., Lindberg, D.A., et al. Identification of a clinical subset of systemic lupus erythematosus by antibodies to the Sm antigen. Arthritis Rheum. 1979, 22, 1334–1337.
Pirofsky, B., Bardana, E.J. Jr. Autoimmune hemolytic anemia II. Therapeutic aspects. Sem Hematol. 1974, u, 376–385.
Pirofsky, B. Immune hemolytic disease: The autoimmune hemolytic anemias. Clin. Hematol. 1975, 4, 167–180.
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Lavalle, C., Hurtado, R., Quezada, J.J. et al. Hemocytopenia as initial manifestation of systemic lupus erythematosus. Prognostic significance. Clin Rheumatol 2, 227–232 (1983). https://doi.org/10.1007/BF02041395
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DOI: https://doi.org/10.1007/BF02041395