Cases reported "Multiple Myeloma"

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1/586. lymphomatoid granulomatosis following autologous stem cell transplantation.

    lymphomatoid granulomatosis (LYG) is a rare angio-destructive lymphoproliferative disorder (LPD) of uncertain etiology, with prominent pulmonary involvement. Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-associated B cell LPD with large numbers of background reactive T lymphocytes (T cell-rich B cell lymphoma). Although the disease frequently, but not exclusively, occurs in various immunodeficiency states, it has not been reported in association with the transient immunosuppression following autologous bone marrow/peripheral stem cell transplantation (ABM/PSCT). We describe a patient who developed lymphomatoid granulomatosis of the lung approximately 2 weeks after high-dose chemotherapy and autologous peripheral stem cell transplantation for multiple myeloma. Although molecular studies showed no evidence of EBV genome in the biopsy material, the serologic profile with high IgM titers was suggestive of primary EBV infection. Complete radiologic remission occurred following reconstitution of the patient's immune response after a 2-week course of ganciclovir treatment. Despite the apparently low frequency of LPD (both LYG and EBV-associated post-transplant lymphoma) in the ABMT setting, we believe that it should be considered in the differential diagnosis of patients whose clinical course following ABMT is complicated by fevers, in the absence of an identifiable infectious process.
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keywords = bone
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2/586. IgE multiple myeloma.

    IgE multiple myeloma is a rare disease characterized by a high frequency of Bence-Jones proteinuria and plasma cell leukaemia when compared to other isotypes of monoclonal proteins. Only 35 cases have been reported. We describe a 70-year-old woman with a stage III IgE kappa multiple myeloma presenting with a sacral plasmacytoma. Immunological and biochemical studies showed IgE kappa producing tumoral plasma cells. serum total IgE was high without clinical symptoms suggesting an hyperIgE syndrome or mast cell activation. The patient underwent surgical removal of the sacral tumor and monthly melphalan-prednisone treatment together with intravenous pamidronate infusions. magnetic resonance imaging (MRI) of the dorsolumbar spine revealed an epidural process leading to T6-T9 radiotherapy. Bone densitometry showed a decreased bone mineral content supporting the management of myeloma-related osteoporosis with bisphosphonate infusions. A good partial response with plateau-phase and increase of bone mineral content was achieved after 1 year of treatment and still persists after a 28 months follow-up.
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keywords = bone
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3/586. Mandibular mass as the presenting manifestation of IgM myeloma in a 22-year-old man.

    We report here the youngest known IgM myeloma patient to have presented with a mandibular mass. A 22-year-old Chinese man sought medical attention due to a mass over his right mandible that had been growing progressively for 6 months. A solitary osteolytic lesion in the right mandible was identified radiologically. Incisional biopsy revealed the presence of plasma cells of monoclonal origin, as evidenced by the exclusively positive staining of the kappa light chain. The diagnosis of multiple myeloma with mandibular involvement was confirmed by bone marrow examination. Further tests, including immunoglobulin electrophoresis and assay of the serum levels of kappa and lambda light chains, demonstrated that his myeloma was of the IgM, kappa subtype. The patient achieved a nonsustained partial response to six courses of melphalan and prednisolone therapy and palliative radiotherapy.
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4/586. Simultaneous occurrence of Hodgkin's disease, nodal Langerhans' cell histiocytosis and multiple myeloma IgA(kappa).

    A 35-year-old man suffered simultaneously from nodular sclerosis Hodgkin's disease (HD), Langerhans' cell histiocytosis and multiple myeloma (MM). There was no prior history of irradiation or chemotherapy, and clinically the lymphoma was confined to cervical lymph nodes. Immunohistochemically, neoplastic lymphoma cells reacted with CD15 and CD30 markers. The patient's bone marrow exhibited a diffuse infiltration by rather atypical plasma cells showing kappa immunoglobulin light-chain restriction. At 14 months after the diagnosis, after autologous bone marrow transplantation, the clinical evolution is favourable with complete remission of the diseases. This is the first time that the coexistence of these three haematological disorders has been discussed, and only the fourth documented case of simultaneous HD and MM. Speculations about the significance of this finding are discussed.
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keywords = bone
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5/586. Donor leukocyte infusions inducing remissions repeatedly in a patient with recurrent multiple myeloma after allogeneic bone marrow transplantation.

    We describe a patient with recurrent relapses after allogeneic BMT for multiple myeloma who repeatedly went into CR after donor leukocyte infusions (DLI). The first bone marrow relapse, 24 months after allogeneic BMT, was treated successfully with the infusion of 1.2 x 10(8) donor T cells. The second extramedullary relapse, 18 months later with a pleural mass and midthoracic spine process, responded again to DLI, however, only after three courses were given, each with escalating doses of T cells. The pleural mass was treated successfully with radiation therapy after the second DLI but reappeared 3 months later and responded again to the final DLI course with 5 x 10(8) T cells/kg. Nevertheless, graft-versus-host disease (GVHD) did not occur. Retrospective analysis of minimal residual disease in bone marrow aspirates during CR periods using a sensitive quantitative tumor-specific PCR showed that BM tumor cell infiltration persisted. The possible clinical implications of this case report, like maintenance DLI and the aim for molecular remissions, are discussed.
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ranking = 6
keywords = bone
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6/586. Interferon gamma as immunomodulator in a patient with multiple myeloma.

    We describe here a patient with multiple myeloma, who, while in remission after chemotherapy, received 100 micrograms of rIFN-gamma (Imukin, Boehringer, Ingelheim) subcutaneously 3 times a week for 4 weeks as supportive therapy before autologous peripheral blood stem cell transplantation (PBSCT). The patient was monitored for serum IFN, TNF, IL-2 activities and for the ability of peripheral blood leukocytes (PBL) to produce IFN-alpha/beta, IFN-gamma, IL-2 and TNF-alpha after in vitro induction. Changes in the percent of plasma cells in the bone marrow, in the total and differential white blood cell counts, in T cell subsets and NK cells were also monitored. IFN-gamma yielded no clinical antitumor activity. The number of bone marrow plasma cells increased, however, the percentage of blood and bone marrow NK cells and the CD4/CD8 T cell subset ratio decreased. Monitoring the cytokine production ability of PBL during IFN-gamma therapy revealed an increase in IL-2, IFN-gamma and TNF-alpha titers produced upon in vitro induction after 2 weeks of treatment (6 injections of rIFN-gamma). However, after 9 injections there was a significant decrease in IFN-gamma and IL-2 production in the PBL, and at the end of therapy (12 injections) the decrease not only in IL-2 and in IFN-gamma but also in IFN-alpha production was observed. In contrast to these changes, TNF production was strongly enhanced and reached the level observed before the therapy. These data suggest that the schedule of IFN-gamma therapy in multiple myeloma should perhaps be adapted to become more effective, taking advantage from the immunomodulating activity of IFN-gamma.
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keywords = bone
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7/586. Effect of calcitriol and pamidronate in multiple myeloma.

    Addition of bisphosphonates to standard treatment of multiple myeloma (MM) decreases bone pain and skeletal events without influencing bone healing. calcitriol, besides its established effects on bone remodeling and calcium metabolism, has both immunoregulatory and cell differentiating effects in vitro and in vivo. Moreover, low serum calcitriol has been reported in MM. We tested the effects of supportive treatment with calcitriol and pamidronate on bone disease in two stage-III-B MM patients with diffuse bone involvement, normal serum calcium, and low serum calcitriol. Complete blood counts, serum calcium, creatinine, quantitative serum and urine immunoglobulins, and biochemical indices of bone turnover, serum calcidiol, calcitriol, parathyroid hormone, skeletal radiographs, and bone mineral density by dual x-ray absorbtiometry were measured every 1-6 months for 16 months in the first patient and 7 months in the second patient. Both patients showed a dramatic improvement of MM activity and in bone disease documented by serial radiographs in the first patient and by increased bone mineral density (approximately 15%) in the second. The reduced serum calcitriol in both patients and the elevated parathyroid hormone observed in the first patient before treatment returned to normal. Supportive treatment with pamidronate does not induce bone healing in MM. Therefore, the results observed with the addition of calcitriol suggest that this hormone may have contributed to the apparent arrest of the progression of MM and caused stimulation of bone healing.
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ranking = 11
keywords = bone
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8/586. Computed tomography guidance in bone marrow aspiration for diagnosis of marrow necrosis and metastasis.

    Bone marrow necrosis is most frequently diagnosed at postmortem examination. Antemortem diagnosis is still uncommon. We illustrate four cases where initial bedside attempts at needle aspiration and biopsy of primary and metastatic tumor tissue from the sternum were complicated by inadequate specimen retrieval secondary to marrow necrosis and/or tissue destruction by tumor. In these cases, CT guidance was useful in the precise localization of the bulk of the tissue mass and consequently the successful retrieval of adequate diagnostic specimens. We demonstrate CT guidance as an excellent and convenient alternative in circumstances where adequate marrow aspirations and biopsies are difficult and complicated.
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ranking = 4
keywords = bone
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9/586. Non-secretory or low-secretory myeloma with intracellular kappa chains. Report of six cases and review of the literature.

    This report concerns six cases of multiple myeloma characterized by either no demonstrable monoclonal immunoglobulin in plasma or urine or by trace amounts (less than or equal to 0.1 g/l) of monoclonal kappa chains in the urine. In all cases there was an infiltration of the bone marrow by plasma cells containing kappa chains but no heavy chains. A retrospective analysis was made of 126 consecutive cases of Bence Jones myeloma. The number of kappa and lambda cases was approximately the same. All cases secreting less than or equal to 0.1 g light chains per 1 urine were of kappa type. This contrasts with a kappa/lambda ratio of 1.4-1.9 among reported series of M-components containing both heavy and light chains. A review of reported cases of non-secretory myeloma revealed a preserved capacity for Ig synthesis in the majority of cases and among these a preponderance of kappa chain producing clones. These observations might be explained by a higher tendency for kappa chain producing cells to mutate to low secretors or to cells producing abnormal light chains which are catabolized rapidly. The clinical data from our patients do not indicate a more pessimistic prognosis in non- or low-secretory myeloma, than in other cases of multiple myeloma.
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keywords = bone
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10/586. Massive myelomatous ascites responsive to VAD chemotherapy and autologous stem cell transplantation.

    Plasmacytic ascites is an infrequent complication of multiple myeloma. To date, only few cases have been reported with a very rapidly fatal course unresponsive to therapy. We describe a patient with plasmacytic ascites and quiescent multiple myeloma of 8 years of duration. disease progression became apparent due to myelomatous ascites, unexplained fever, pancytopenia and bone marrow infiltration. This case showed a complete and long-lasting response after VAD chemotherapy followed by autologous PBSC transplantation. ascites in association with MM may respond for lengthy periods to high-dose chemotherapy and ASCT.
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keywords = bone
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