Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The presenting symptoms of malignancies like anemia and splenomegaly in thalassemic patients can be overlooked and considered as complications of thalassemia. Our paper deals with a case of large B cell lymphoma with bone marrow involvement in an old lady with thalassemia intermedia. The patient showed complete response after the second cycle of chemotherapy that consists of cyclophosphamide, vincristine, and prednisone (CVP protocol). However, the patient relapsed with bone marrow involvement shortly after (2 weeks) completion of the sixth cycle of chemotherapy, and she was started on monoclonal antibodies Rituximab. On reviewing the literature, only four cases of thalassemia and lymphoma have been reported worldwide, thus making our case the fifth report of this rare combination of diseases.
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PMID:Beta-thalassemia intermedia and non-Hodgkin's lymphoma. 1240 86

This presentation represents consensus recommendations for the treatment of patients with Waldenstrom's macroglobulinemia (WM), which were prepared in conjunction with the second International Workshop held in Athens, Greece during September 2002. The faculty adopted the following statements for the management of patients with Waldenstrom's macroglobulinemia: (1) Alkylating agents, nucleoside analogues, and rituximab are reasonable choices for first line therapy of WM. (2) Both cladribine and fludarabine are reasonable choices for the therapy of WM. (3) Combinations of alkylating agents, nucleoside analogues, or rituximab should at this time be encouraged in the context of a clinical trial. (4) In WM, rituximab can cause a sudden rise in serum IgM and viscosity levels in certain patients, which may lead to complications, therefore close monitoring of these parameters and symptoms of hyperviscosity is recommended for WM patients undergoing rituximab therapy. (5) For relapsed disease, it is reasonable to use an alternate first line agent or re-use of the same agent; however, since autologous stem cell transplantation may have a role in treating patients with relapsed disease it is recommended that for patients in whom autologous transplantation is seriously being considered, exposure to alkylator or nucleoside analogue drugs should be limited. (6) Combination chemotherapy for patients who can tolerate myelotoxic therapy, thalidomide alone or with dexamethasone, can reasonably be considered to have relapsed. (7) Autologous stem cell transplantation may be considered for patients with refractory or relapsing disease. (8) Allogeneic transplantation should only be undertaken in the context of a clinical trial. (9) Plasmapheresis should be considered as interim therapy until definitive therapy can be initiated. (10) Rituximab should be considered for patients with IgM-related neuropathies. (11) Corticosteroids may be useful in the treatment of symptomatic mixed cryoglobulinemia. (12) Splenectomy is rarely indicated but has been used to manage painful splenomegaly and hypersplenism.
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PMID:Treatment recommendations in Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. 1272 Jan 20

Bcl-2 protein is upregulated in a wide variety of lymphoid malignancies, including chronic lymphocytic leukemia (CLL). The protein is thought to be responsible for maintaining the viability of malignant lymphoid cells and may contribute to chemotherapy and radiotherapy resistance. Previous studies have shown that reduction of bcl-2 expression by antisense therapy sensitizes cells to chemotherapy-induced apoptosis. In vitro, the Bcl-2 antisense drug oblimersen sodium (Genasense, previously known as G3139) enhances the apoptotic response in CLL cells to fludarabine (Fludara), corticosteroids, alemtuzumab (Campath), and rituximab (Rituxan). A phase I trial in patients with refractory/relapsed CLL showed that patients with CLL were more sensitive to oblimersen than patients with solid tumors. The maximum tolerated oblimersen dose was 3 mg/kg/d, and the most common dose-limiting reaction was hypotension, frequently in association with high spiking fever. In this study, oblimersen displayed limited single-agent activity, including tumor lysis syndrome, transient decreases in circulating CLL cells, and reduction of splenomegaly and size of lymph nodes. Major responses were observed in 9% of patients. Subsequently, a phase III trial evaluating fludarabine and cyclophosphamide with or without oblimersen (3 mg/kg/d for 7 days) was initiated in patients with relapsed or refractory CLL. This trial recently completed accrual of 241 patients.
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PMID:Potential therapeutic applications of oblimersen in CLL. 1565 Nov 75

Prolymphocytic leukemia is a rare chronic lymphoproliferative disorder that includes two subtypes, B cell and T cell, each with its own distinct clinical, laboratory and pathological features. T-cell prolymphocytic leukemia has an aggressive course with short median survival and poor response to chemotherapy. With the use of the purine analogue pentostatin more than half of patients will have a major response and a minority will have a complete remission, usually lasting months. With the introduction of alemtuzumab, most patients who progressed despite treatment with pentostatin had a major response with a complete remission rate higher than that obtained with pentostatin when used as a first line. Unfortunately, progression still follows shortly. We recommend alemtuzumab as initial therapy and offer stem cell transplant (SCT) to selected young, healthy patients who respond. Although B-cell prolymphocytic leukemia is also a progressive disease, some patients can achieve a prolonged progression-free-survival with fludarabine. Patients presenting with massive splenomegaly may be effectively palliated with splenic irradiation or splenectomy. Rituximab is a promising agent and further investigations are warranted to better define its role in treatment of this disorder.
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PMID:Prolymphocytic leukemia. 1586 31

In this retrospective study, rituximab was found to be effective therapy in 10 of 11 patients with splenic marginal zone lymphoma, inducing prompt reduction in splenomegaly, improvement in blood counts in 9 patients and clearance of a pleural effusion in 1 patient. Median response duration was 21 months (range 4 to 37 months). Two patients who relapsed at 21 and 23 months responded to retreatment. Rituximab should be considered in patients who are poor candidates for splenectomy.
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PMID:Rituximab monotherapy for splenic marginal zone lymphoma. 1595 3

A 75-year-old woman was diagnosed of MCD plasma cell (PC) variant with B symptoms. Diffuse lymph-node enlargement, splenomegaly and pancytopenia were detected. Induction with Rituximab was made because pancytopenia was present. Actually patient is free of disease. This is the first complete response of MCD published, VIH negative, induced with anti CD20.
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PMID:Complete remission in a pancytopenic HIV negative, HHV-8 positive patient with multicentric Castleman's disease induced with anti-CD20. 1687 May 45

Splenectomy has traditionally been considered as a standard first line treatment for splenic marginal zone lymphoma (SMZL) conferring a survival advantage over chemotherapy. However it carries significant complications, especially in elderly patients. The purpose of this retrospective study was to report our experience on the efficacy of Rituximab as first line treatment in 16 consecutive SMZL patients, diagnosed in our department. The diagnosis was established using standard criteria. Patients' median age was 57 years (range, 48-78). Prior to treatment initiation all patients had splenomegaly, nine had anemia, five lymphocytosis, five neutropenia and six thrombocytopenia. Rituximab was administered at a dose of 375 mg/m2/week for 6 consecutive weeks. The overall response rate was 100%. After treatment, all patients had a complete resolution of splenomegaly along with restoration of their blood counts. Eleven patients (69%) achieved a CR, three (19%) unconfirmed CR and two (12%) a PR. Among the complete responders seven patients had also a molecular remission. The median time to clinical response was 3 weeks (range, 2-6). Rituximab maintenance was given to 12 patients. Eleven of them had no evidence of disease progression after a median follow-up time of 28.5 months (range, 14-36), while two out of four patients who did not receive maintenance, relapsed 7 and 24 months after the completion of induction treatment. Median follow-up time for the entire series was 29.5 months (range, 15-81). No deaths were recorded during the follow-up period. Therapy was well tolerated. The present study demonstrates that rituximab is an effective treatment for SMZL and could be considered as a substitute or alternative to splenectomy.
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PMID:Rituximab monotherapy is highly effective in splenic marginal zone lymphoma. 1827 Oct 62

Hairy cell leukemia (HCL) is an indolent B-cell lymphoproliferative disease, characterized by splenomegaly and pancitopenia related to this. The lymphocytes present characteristic citoplasmatic projections and are positive for tartrate-resistant acid phosphatase (TRAP). Immunophenotyping is necessary to identify the co expression of CD103, CD25, CD11c associated with a typical B-cell clonally pattern and to make a differential diagnosis from other indolent malignancies. Despite the indolent clinical course, treatment is required to resolve symptoms related to splenomegaly and to reduce the incidence of severe infections that are the major complications and a common cause of death. In the past the treatment was only able to resolve the symptoms. In the revised literature, purine analog have been identified as the treatment of choice for this disease. Cladribrine (2-CdA) is able to induce more than 80% of complete remission and is also effective in relapsed patients. Rituximab after 2-CdA treatment can obtain a molecular response in most cases. The introduction of purine analog, and recently of Rituximab, in association with conventional chemotherapy can modify the clinical course of the disease with low toxicities.
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PMID:Hairy Cell Leukemia. 1792 10

Rituximab is a chimeric monoclonal antibody directed against the phosphoprotein CD20. Because of its efficacy and acceptable toxicity profile, rituximab is now commonly used for the treatment of CD20-positive B-cell malignancies, including B-cell non-Hodgkin's lymphoma. However, rituximab-induced acute thrombocytopenia is an extremely rare side effect. We report a case of acute thrombocytopenia occurring immediately after rituximab infusion in a mantle cell lymphoma patient with bone marrow involvement and massive splenomegaly. Although the mechanism of thrombocytopenia is still unclear, it is possible that tumor burden, bone marrow involvement, the presence of infusion-related symptoms, and mantle cell histology are related to this rare complication of rituximab therapy. Hence, rituximab should be used with caution in patients who have these factors, and clinicians must be aware of this rare, but serious, side effect.
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PMID:Rituximab-induced acute thrombocytopenia: a case report and review of the literature. 1854 33

Rituximab is a monoclonal antibody specific for the CD20 antigen. Clinical factors associated with thrombocytopenia after administration of rituximab have only been reported as case reports. We have analysed retrospectively the change of platelet counts following the administration of rituximab in 253 patients with non-Hodgkin lymphoma (NHL). Correlations with clinical and laboratory parameters were assessed. A mean overall decrease in platelets was observed after rituximab infusion. A downward trend in platelet count of more than 30% was observed in 7.2% of the patients. The decline was observed when rituximab was given as a single agent or in combination with chemotherapy. The risk factors to develop a decline in platelets after infusion of rituximab were pre-existent thrombocytopenia, advanced lymphoma stage, bone marrow infiltration, splenomegaly, leukemic presentation, and Burkitt lymphoma histology. In conclusion, a decline in platelet count after administration of rituximab was observed in patients with NHL, mainly those with pre-existing thrombocytopenia.
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PMID:Rituximab-associated changes in platelet count in patients with non-Hodgkin lymphoma. 1902 Oct 44


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