This report describes 14 cases of large cell lymphoma with initial presentation as marrow involvement in the absence of peripheral lymphadenopathy. The disease affected mainly the middle-aged and elderly, with male predominance. Most patients presented with swinging fever and peripheral blood cytopenia. Reactive hemophagocytic syndrome was a common finding, causing significant morbidity and mortality. Peripheral lymphadenopathy was absent (by case selection), but involvement of the liver, spleen and paraaortic lymph nodes could be demonstrated in some cases. However, in some patients, the involvement was apparently restricted to the bone marrow. Involvement of the bone marrow was often subtle, and could be missed on causal examination. Immunohistochemical studies on eight cases showed that four exhibited a B-cell phenotype, three a T-cell phenotype and one a non-T non-B phenotype. The prognosis was poor, with survival being measured in days to weeks in most patients.
Pure red cell aplasia (PRCA) is a rare hematological disease characterized by selective marrow erythroid aplasia. We report the clinical features and treatment results of 16 Chinese patients with PRCA. Nine (56%) cases were not associated with any underlying disorders and were considered idiopathic, while seven patients (44%) had associated diseases, three involving the thymus, two with T large granular lymphocyte leukemia (T-LGLL), and one each with Stevens-Johnson syndrome and acute hepatitis A. Conventional-dose corticosteroid therapy resulted in complete remission in three of 13 patients. Cyclosporin A was used in six patients. There were three complete and one partial remissions. High-dose methylprednisolone was ineffective in four patients who failed conventional-dose corticosteroids but achieved complete remission in one patient with thymoma who did not respond to thymectomy. Antithymocyte globulin was used in four patients, resulting in partial remission in only one patient with concomitant T-LGLL. Intravenous gamma globulin and danazol were ineffective in three patients. Thymectomy was performed in two patients, with one patient remitting. This is the largest series of PRCA reported in an oriental population. Our results indicate that treatment of PRCA may still be problematic and better therapeutic strategy will have to be defined.
We performed a retrospective analysis of bone marrow examination (BME) in the management of Chinese adult patients less than 60 years of age with isolated thrombocytopenia at presentation. Eighty-three patients with a median age of 39 years presenting with isolated thrombocytopenia (median platelet count: 38 x 10(9)/l) had routinely undergone BME as part of the laboratory investigations during the period from January 1996 to December 1999. All 83 patients had bone marrow findings of active marrow suggesting causes due to peripheral destruction. All of these patients responded to steroid or intravenous immunoglobulin (IVIg) therapy at presentation if their platelet counts were significantly low or if they had mucosal bleeding. Eighty-one of the 83 patients, after a median of 20 months follow-up, were finally diagnosed as having idiopathic thrombocytopenic purpura (ITP). The remaining two patients were finally confirmed as cases of systemic lupus erythematosus (SLE). Our results suggest that BME is not helpful in the diagnosis of isolated thrombocytopenia or suspected ITP in adult patients at presentation, provided that a thorough clinical history and physical examination are undertaken and that the blood count and peripheral blood smear show no abnormalities apart from the thrombocytopenia.
This report describes the bone marrow findings in four patients whose marrow was involved by anaplastic large-cell Ki-1 lymphoma, an uncommon event in this type of lymphoma. In the marrow aspirate smears, the involvement was subtle, and was in the form of isolated large cells with irregular nuclear configuration, coarse chromatin, prominent nucleoli, and basophilic cytoplasm which might be vacuolated. One case showed paradoxically massive involvement in the trephine biopsy taken from the same site as the marrow aspirate. Reactive histiocytic proliferation with hemophagocytosis was also present. Since marrow aspirate or biopsy may be the first pathologic specimen examined in patients having anaplastic large-cell Ki-1 lymphoma, it is important to be able to recognize the small population of neoplastic cells, which should lead to prompt treatment or further investigations as deemed necessary.
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