In this retrospective study, we evaluated the clinical features and the effects of various treatment modalities on the clinical course in patients diagnosed with idiopathic thrombocytopenic purpura (ITP) at our center between 1984-2000. We retrospectively examined the medical records of 321 (229 females, 92 males) ITP patients. One hundred and seventy-one (53.3%) patients were lost to follow-up. When evaluating the clinical features, all 321 patients were included; however, when the response to treatment modalities was evaluated only 150 patients followed up regularly were considered. The median age of the patients on initial diagnosis was 34 years (range: 14-78). At initial diagnosis, 235 (73.2%) patients had signs of bleeding. Of patients diagnosed with ITP initially, six later turned out to have systemic lupus erythematosus (SLE) and two myelodysplastic syndrome (MDS). The median follow-up of 150 patients followed up regularly was 30 months (range: 4-396). One hundred and thirty-seven of these subjects had an indication for treatment and 94.2% of them were administered either standard or high-dose steroids as the first-line therapy. Complete remission (CR) was defined as any platelet count >100,000/mm(3) lasting for 3 months or longer without treatment. CR was achieved in 51.9% of the patients given steroids as the initial therapy. During a median follow-up of 33 months, relapse occurred in 58.2% of these patients, and after a median follow-up of 11 months the rest of them were still in remission. Ninety-eight patients followed up regularly were administered second-line therapies. CR was obtained in 44.4% of the patients who used steroids as second-line therapy. Within a median follow-up of 15 months, 20.8% of these patients relapsed. Splenectomy was performed in 76 patients and CR was obtained in 68.4% of the regularly followed up patients. Relapse occurred within a median of 96 months in 15.4% of the patients who had CR. Kaplan-Meier curves showed that the duration of CR obtained by splenectomy was significantly higher than that obtained by steroids (p<0.001). The 10-year disease-free survivals in patients who used steroids and who underwent splenectomy were, respectively, 13% and 58%. In our adult ITP patients, steroids induced nearly similar rates of CR both as first-and second-line therapies. Splenectomy seems to be effective in patients unresponsive to steroids. The duration of CR obtained by splenectomy is significantly longer when compared with the duration of CR obtained by steroid therapy.
It is quite rare to diagnose secondary amyloidosis during the course of Castleman's disease (CD). A 51-year-old female who complained of fatigue, weight loss, and fever was diagnosed with CD -- plasma cell type -- in our hospital in 1993. One year after diagnosis, she developed nephrotic syndrome, the etiology of which was found to be secondary amyloidosis based on renal biopsy. As the patient rejected therapy, she was discharged after only symptomatic treatment. At her last follow-up in March 2001, she had no complaints; physical examination, blood chemistries, and urinalysis were normal. Abdominopelvic tomography revealed no lymphadenopathy in the abdomen, which had been previously present. We could identify 17 other cases of CD with secondary amyloidosis in the literature. Ours is the 18th such case and the 2nd case of multicentric CD leading to amyloidosis. This case also shows that CD might sometimes run a relatively benign course being cured with no therapy, whereas it might have a rapidly fatal downhill course -- even with therapy -- in others. Still, effective treatment strategies need to be developed.
Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma and four cases of PCNSL have previously been described in association with mycophenolate mofetil. We report the fifth case of PCNSL in a patient with lupus nephropathy while on mycophenolate mofetil treatment.
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