Hemocytopenia as initial manifestation of systemic lupus erythematosus. Prognostic significance.

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.