Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adult Burkitt's lymphoma is an uncommon disease. Few cases of spinal involvement in adults with sporadic Burkitt's lymphoma are reported in the literature. We present a case of a middle-aged man who was found to have an epidural mass in the thoracic spine when investigated for back pain and lower extremity weakness. He underwent a laminectomy with resection of the epidural mass. Histologic examination revealed a primary Burkitt's lymphoma of the spinal cord. He was treated with aggressive chemotherapy and is now experiencing remission of his disease. We also present a review of the literature for the etiology and clinical features of other spinal lymphomas and Burkitt's lymphoma involving the spine in adults.
Clin Lymphoma Myeloma 2006 Jan
PMID:Spinal Burkitt's lymphoma in adults. 1650 12

Total 14 cases of myeloma in young age group (<40 years) have been reported out of 178 cases of myeloma in a time period of 7 years (1993-1999). Males predominated overfe males. Like adult myeloma, patients presented mostly with the backache, pain in pelvis, lower spine and weakness in about 60% of cases followed by swelling of bone in 40% of cases. One case presented with bleeding gum, malena and hepatosplenomegaly and was diagnosed as plasma cell leukemia. Radiological examination revealed lytic lesion in almost all the cases with fracture femur and rib in 28.57% of cases. Anaemia and raised ESR was noted in all the cases. Myeloma typing revealed IgG myeloma in 10 cases, light chain myeloma in 3 cases and IgA myeloma in one case. None of the patient was traceable after 2 years. Thus our study concludes that myeloma in the young age in India occurs in increased frequency and clinically presents just like adult and elderly myeloma, but serologically are predominantly of IgG type. There is also an increased frequency of solitary plasmacytoma as compared to adult myeloma.
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PMID:Myeloma in young age. 1676 40

At age of 57 years, a man experienced an episode of rhabdomyolysis. On that occasion muscle biopsy was not performed, however monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. Further he developed a moderate proximal muscle weakness with CK level persistently elevated (1000-1200U/l). When he came to our observation, at age 67, a muscle biopsy revealed an amyloid myopathy and multiple myeloma was at the same time disclosed. Terminal complement complex C5b9 (membrane attack complex) deposits were found in the vessel walls and muscle fibers surface depicted by amyloid. Our case suggests to keep in mind the possibility that amyloid myopathy may begin as an isolate episode of rhabdomyolysis. The detection of complement complex C5b9 suggests that complement cascade is implicated in the muscular damage of amyloid myopathy.
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PMID:Amyloid myopathy presenting with rhabdomyolysis: evidence of complement activation. 1691 49

Crow-Fukase syndrome (CFS) is a rare multi-system disorder, characterized by polyneuropathy, organomegaly, endocrinopathy, M-proteins, skin changes and anasarca, with or without myeloma. The pathophysiology, diagnosis, and treatment of CFS are controversial. CFS may be associated with the overproduction of vascular endothelial growth factor (VEGF). However, there have been no reports of monitoring the serum VEGF level after recurrence, to the best of our knowledge. We report a 54-year-old man with CFS presenting with a 3-year history of ascites, anasarca, weakness of the lower extremities, and plasmacytoma in the scapula. At the initial examination, the VEGF level was 1,590 pg/ml (the VEGF level of a healthy control, 78.4 +/- 75.2 pg/ml). After initial treatment with chemotherapy and irradiation of the affected shoulder, the VEGF level decreased to 154 pg/ml and the symptoms disappeared. Twenty one months later, gate disturbance and anasarca recurred, and the VEGF level was over 2,000 pg/dl. After total scaplectomy, the VEGF level decreased to 730 pg/dl and the symptoms disappeared. The serum level of VEGF well correlated to the clinical course of the patient. In conclusion, measurement of the VEGF level is useful for diagnosing CFS and for monitoring its clinical course.
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PMID:Recurrent Crow-Fukase syndrome associated with increased serum levels of vascular endothelial growth factor: a case report and review of the literature. 1707 5

Patients who undergo autologous peripheral blood stem cell (PBSC) transplantation experience multiple symptoms that adversely affect quality of life. We assessed symptoms during the acute phase of autologous PBSC transplantation to determine the severity of individual symptoms and to determine overall symptom profiles in 100 patients with multiple myeloma or non-Hodgkin's lymphoma. Study subjects completed the blood and marrow transplantation module of the M. D. Anderson Symptom Inventory before hospitalization, during conditioning, on day of transplantation, at nadir (the time of lowest white blood cell count) and on day 30 post-transplantation. Additional symptom, quality-of-life and medical status measures were collected. Symptom means were mild at baseline, intensified during conditioning, peaked at nadir and decreased by day 30. At nadir, the most severe symptoms for the entire patient sample were lack of appetite, fatigue, weakness, feeling sick, disturbed sleep, nausea and diarrhea. Cancer diagnosis was a significant predictor of changes in symptoms over time. The patterns of fatigue, pain, sleep disturbance and lack of appetite were significantly different for patients with multiple myeloma as compared with patients with non-Hodgkin's lymphoma.
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PMID:Symptom burden in patients undergoing autologous stem-cell transplantation. 1743 88

Duchenne Muscular Dystrophy (DMD) is an incurable inherited disease of childhood, characterized by progressive muscle degeneration and weakness. Our previous findings supported the idea that dystrophin and associated proteins, absent or greatly reduced in DMD, are degraded in dystrophin-deficient muscle by the proteasomal-dependent pathway. Indeed, treatment with the proteasome inhibitor MG-132 of skeletal muscles from mdx mice--a spontaneous mouse model of DMD--as well as from DMD patients, effectively rescued the expression and correct cellular localization of dystrophin and associated proteins. These promising results led us to further explore the use of proteasome inhibitors as a therapy for DMD. Therefore, we directed our attention towards two new dipeptide boronic acid inhibitors blocking the proteasomal-dependent degradation pathway: Velcade (bortezomib or PS-341) and MLN273 (PS-273). The exciting aspect of this development is that these drugs have already progressed to preclinical and clinical trials, in different fields than muscular dystrophy. Indeed, Velcade has been already FDA-approved for treatment of multiple myeloma and its side effects had been already explored and managed. Promisingly, MLN273 is currently in the preclinical trial phase. Here, we test the effectiveness of Velcade and MLN273 by local injection into the gastrocnemius muscle of mdx mice. We show the rescue of expression and membrane localization of alpha-dystroglycan, beta-dystroglycan, alpha-sarcoglycan, and dystrophin after Velcade and MLN273 localized treatment, versus untreated (PBS only) mdx mice. Intriguingly, we also show that localized treatment with Velcade and MLN273 reduces the activation of Nuclear Factor-kappaB (NFkB). Because the NFkB pathway has been shown to be involved in inflammation responses in myopathies and DMD, our current results may have important clinical implications. Clearly, more investigations are needed, but our results emphasize the effectiveness of the pharmacological approach as a potential treatment for Duchenne muscular dystrophy.
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PMID:Localized treatment with a novel FDA-approved proteasome inhibitor blocks the degradation of dystrophin and dystrophin-associated proteins in mdx mice. 1749 27

We report the case of a patient with multiple myeloma (MM) and extensive lytic bone disease who presented with a short history of back pain and leg weakness. Magnetic resonance imaging (MRI) of the lumbar spine revealed a sacral insufficiency fracture complicated by an epidural haematoma. Bleeding tendency in this case can be accounted for by platelet function defects, often described in plasma cell disorders in the absence of obvious coagulation abnormalities. Surgical intervention was contraindicated as a result of poor overall patient performance status (poor surgical candidate due to extensive myelomatous bone disease, previous vertebral compression fractures requiring orthopaedic stabilisation, and requiring opiate analgesia for bone pain) and management was conservative. Patients presenting with back pain and documented bone disease in the setting of myeloma should be managed with a high index of clinical suspicion and considered for urgent MR imaging to avoid missing this serious and potentially reversible complication. We report the undescribed causative association between sacral insufficiency fracture and lumbo-sacral epidural haematoma. We illustrate the MRI signal and contrast enhancement pattern of an acute presentation of epidural haematoma.
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PMID:Sacral insufficiency fracture complicated by epidural haematoma and cauda equina syndrome in a patient with multiple myeloma. 1757 3

A 60-year-old man was admitted with muscle weakness and numbness in the extremities. Based on the existence of monoclonal gammopathy of the IgG-lamda type, a slight increase of plasma cells in the bone marrow, and an elevated level of serum vascular endothelial growth factor (VEGF), the diagnosis of POEMS syndrome was made. After peripheral blood stem cell collection by etoposide and G-CSF, the patient received high dose melphalan (200 mg/m2) therapy supported by autologous peripheral blood stem cell transplantation (autoPBSCT). After high-dose chemotherapy with autoPBSCT, the serum VEGF level normalized and the monoclonal IgG-lamda, disappeared. The patient gradually recovered from a bedridden state and at the time of writing has no impairment in his activities of daily life. After the autoPBSCT, monoclonal IgG-kappa, protein was detected transiently in serum. The new monoclonal immunoglobulin was considered to be due to normal immune reconstitution after myeloablation rather than alteration of the abnormal plasma cell clone, similarly as oligoclonal immunoglobulins occur in multiple myeloma after autoPBSCT. AutoPBSCT with high-dose chemotherapy should be considered among the treatments of choice for POEMS syndrome.
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PMID:[POEMS syndrome presenting with transient immunoglobulin isotype switching after successful treatment with autologous peripheral blood stem cell transplantation]. 1786 1

Association of a peripheral neuropathy with an IgA monoclonal gammopathy of undetermined significance (MGUS) is not commonly observed and is sometimes considered as coincidental. We present a case in which the nerve biopsy revealed the presence of crystalline inclusions in the endoneurium, a very unusual finding. A 75-year-old man complained of paresthesiae in both feet and unsteady gait for 6 months. He had no weakness, but deep tendon reflexes were absent and vibratory sensation distally diminished in both legs. An IgA lambda MGUS was evidenced in his serum at 10.2 g/L with 7% plasma cells in his bone marrow and no lytic lesion at skeletal examination. A superficial peroneal nerve biopsy was performed and showed numerous crystalline inclusions in the endoneurium. These were located in the cytoplasm of macrophagic histiocytes or free in the vicinity of nerve fibers. There was also a marked loss of myelinated nerve fibers and several "onion bulb" formations surrounding either isolated remyelinating fibers or small clusters of remyelinating fibers. Such crystalline inclusions have mainly been observed in the cytoplasm of plasma cells in cases of multiple myeloma, and correspond to non-secreted IgA or IgG immunoglobulins with a kappa or rarely lambda light chain. Such inclusions have also been reported in the cytoplasm of the epithelial cells from corneal fragments, in patients with multiple myeloma or IgG MGUS, and in the tubular cells from the kidney of patients with multiple myeloma and a nephrotic syndrome. In the literature, there is only one very briefly mentioned case of neuropathy associated with a myeloma and with crystalline inclusions present in the epineurium. Thus, in dysglobulinemic neuropathy, nerve fibers can be damaged by three kinds of interstitial deposits, easily identified by immunohistochemistry and at ultrastructural examination: the well known amyloid fibrils, granulo-fibrillar deposits and also crystalline inclusions.
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PMID:Presence of crystalline inclusions in the peripheral nerve of a patient with IgA lambda monoclonal gammopathy of undetermined significance. 1841 Feb 74

Thalidomide has been estimated as a useful drug in therapy of refractory or relapsed multiple myeloma. Recently, several studies have shown very good results in therapy combination of thalidomide, cyclophosphamide and dexamethasone, but still high doses of thalidomide associated with serious adverse events have been used. In our study, we performed low-dose thalidomide regimens; the aim of this study was to verify the effect and to assess their toxicity. For younger patients up to 65 years we used a "CTD-junior" regimen, consisting of oral thalidomide 200 mg daily, pulsed intravenous cyclophosphamide 800 mg on day 1 and pulsed oral dexamethasone 40 mg on days 1-4 and 12-15, for every three weeks. For patients over 65 years, the "CTDsenior" regimen was used, with oral thalidomide 50-100 mg daily (according to tolerability), oral cyclophosphamide 50 mg daily and pulsed dexamethasone 20 mg on days 1-4 and 15-18, for every four weeks. From the group of 97 patients with progressive form of multiple myeloma or with resistance to conventional chemotherapy, 85 patients were evaluated. According to the EBMT criteria, we observed in 8% complete remission (CR), in 50% partial response (PR) and in 22% minimal response (MR). Ten patients (12%) were stabilized and seven patients (8%) progressed. Toxicity of both regimens was mild and well manageable, when weakness, obstipation, neuropathy of lower extremities, glycoregulation worsening and mild leucopenia occurred most often. These results showed that low doses of thalidomide are still effective, when combined with other drugs. Both CTD regimens are safe also for patients with advanced and heavily pretreated multiple myeloma.
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PMID:Low-dose thalidomide regimens in therapy of relapsed or refractory multiple myeloma. 1850 47


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