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

The presence of a monoclonal protein in the serum of a patient with peripheral neuropathy raises the suspicion of systemic amyloidosis, POEMS syndrome, macroglobulinemia, multiple myeloma, or lymphoma. If these conditions are excluded, the patient is classified as having a monoclonal gammopathy of undetermined significance (MGUS) with an associated neuropathy. Approximately one half of patients with peripheral neuropathy and IgM monoclonal gammopathy have IgM antibodies that bind to a myelin-associated glycoprotein (MAG). In addition to MAG, other antigens such as glycolipids or gangliosides represent other target antigens. Monoclonal gammopathies and motor neuron diseases have also been reported. Sensorimotor peripheral neuropathy occurs in about 15% of patients with primary amyloidosis but is uncommon in multiple myeloma.
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PMID:Monoclonal proteins in neuropathy. 132 50

Two cases of myeloma with roentgenographic evidence of bone sclerosis confirmed by iliac histomorphometric measurements are reported. In one patient, increased resorption, major depression of osteoblast activity, initial intense myelofibrosis, and myeloid deposits were found. The other patient had both increased resorption and increased osteoblast activity with clinical manifestations suggestive of POEMS syndrome. These two cases are compared with 116 cases previously published in the occidental medical literature and with five histomorphometric studies demonstrating increased bone trabecula volume (BTV). Conventional histologic studies suggest several mechanisms as possible explanations for the occurrence of bone sclerosis, including increased modeling unit activity, isolated osteoblast activation, metamorphic neoosteogenesis in myelofibrosis foci, and, in exceptional cases, inhibition of resorption due to increased production of calcitonin. The diversity of bone modeling patterns evidenced by the seven histomorphometric studies reviewed in this article is striking. Bone modeling patterns provide only a snapshot of bone modeling units and may vary over time in a given patient. Reported cases are too few to allow conclusions but emphasize the need for performing further histomorphometric investigations.
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PMID:[Condensing myeloma. Apropos of 2 cases with bone histomorphometric study]. 141 Dec 13

POEMS syndrome is a multisystem disorder associated with polyneuropathy, organomegaly, endocrinopathy, a monoclonal protein (M-protein), and skin changes. The authors describe a patient with POEMS syndrome who had osteosclerotic myeloma confirmed by open bone biopsy. Magnetic resonance imaging (MRI) showed discrete lesions of low signal intensity in both T1 and T2-weighted images. This patient is now being successfully treated with melphalan and prednisone with much improvement in skin thickening and sensory change in the lower extremities.
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PMID:POEMS syndrome--a case report. 141 69

A 58-year-old man developed polyneuropathy, organomegaly, gynecomastia, skin pigmentation, and multiple myeloma (IgG-lambda type). Although transient clinical improvement was obtained with prednisolone and cyclophosphamide, his general condition deteriorated progressively, and he died 3 years after onset. Lymph nodes showed angiofollicular hyperplasia, and the sural nerve revealed segmental demyelination. At autopsy, the glomeruli were enlarged and showed mild mesangial cell proliferation with segmental mesangiolysis and proliferation of endothelial cells forming abnormal vessels. The massive volume of ascites contained a high level of interleukin-6 (IL-6). IL-6, a multifunctional cytokine, may be the pathogenic factor which induces the various clinical symptoms and pathological features of Crow-Fukase (POEMS) syndrome.
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PMID:An autopsy case of Crow-Fukase (POEMS) syndrome with a high level of IL-6 in the ascites. Special reference to glomerular lesions. 147 60

A patient with peripheral polyneuropathy, hepatosplenomegaly, osteosclerotic myeloma in the ilium, hyperpigmentation and hypertrichosis is described. A diagnosis of POEMS syndrome was made. This is the first report of this syndrome from India.
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PMID:POEMS syndrome. 181 11

Peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes are often the presenting features of the POEMS syndrome. Approximately 50% of these are associated with osteosclerotic myeloma, a rare variant of multiple myeloma and some with Castleman's disease, an unusual lympho-proliferative disorder. The underlying pathogenetic mechanisms have not been elucidated but amelioration of symptoms with the disappearance of the M-component suggests possible etiopathogenic mechanisms. We present a 40-year-old woman with manifestations of all three disorders.
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PMID:POEMS syndrome, osteosclerotic myeloma and Castleman's disease: a case report. 195 37

POEMS syndrome, a rare multisystem disease, is a variant of osteosclerotic myeloma and is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins, and skin changes. Presented herein is a case of POEMS syndrome with flushing. The flushing was intermittent, involving the face and upper third of the trunk, and was associated with hypotension and bronchospasm. Final diagnosis was made by biopsy examination of an axillary lymph node, which showed angiofollicular hyperplasia that stained strongly and selectively for lambda light chains. The patient had most of the typical features of POEMS syndrome but was unique in that her most striking finding was carcinoid-like flushing. The flushing improved with steroid therapy, as did some of the other clinical features of her disease. This case suggests that idiopathic flushing can be added to the skin changes observed in POEMS syndrome.
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PMID:POEMS syndrome with idiopathic flushing mimicking carcinoid syndrome. 202 23

A case of POEMS Syndrome of six years of evolution is reported. This syndrome is characterized by Raynaud phenomenon, polyneuropathy, edema, anasarca, papilledema, osteosclerosis and lymphadenopathy with the histopathology of Castleman's disease, hypothyroidism, hypogonadism, cutaneous sclerosis, hyperpigmentation, axillary alopecia and the presence of urinary lambda light chains. A bone marrow biopsy did not show plasmocytic infiltration and there was no evidence of extramedullary plasmocytoma. Methylprednisone was given at the dose of 1 mg/kg/day and subjective and objective improvement was observed. The edema and anasarca disappeared as well as the lymphadenopathies; muscle strength improved and the patient was able to walk without aid. Papilledema persisted. The pathogenesis of this syndrome remains unknown; some of the symptoms have been attributed to paraprotein deposits in peripheral nerves, high capillary permeability due to vascular alterations, accelerated conversion of androgen to estrogen, or to the production by plasma cells of a toxic substance. Mortality is related to complications of the polyneuropathy. Some patients in whom POEMS syndrome was associated, or not, with myeloma were treated with chemotherapy and/or radiotherapy with different responses; in others, corticosteroids were of short lived benefit. Our patients remains well after 42 months treatment with 20 mg methylprednisone every other day.
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PMID:[POEMS syndrome: report of a case and review of the literature]. 210 48

A patient with chronic, bilateral optic disc edema developed multiple systemic manifestations of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes). A serum immunoelectrophoresis showed an abnormal serum IgG lambda protein, and urine immunoelectrophoresis revealed a monoclonal lambda protein plus an IgG lambda fragment. Bone survey and MRI scan revealed a sclerotic lesion of the first lumbar vertebra, and lymph node biopsy showed changes consistent with Castleman's disease. Thus, his optic disc edema was the presenting feature of the POEMS syndrome and osteosclerotic myeloma.
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PMID:When do optic disc edema and peripheral neuropathy constitute poetry? 217 27

We report a 36-year-old patient with progressive tetraparesis and diffuse osteosclerotic lesions without secretion of a monoclonal component, in whom a diagnosis of non-secreting diffuse osteosclerotic myeloma with POEMS syndrome was made with open rib biopsy. We discuss the clinical, biological, radiological and electrophysiological features of this patient. We analyze the possible pathogenesis of his neurological involvement, with a detailed review of the relevant literature. Finally, we underline the therapeutic success with a combination of cyclophosphamide and prednisone, comparing it with the previous references to this therapeutic modality, and emphasize the validity of our therapeutic approach.
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PMID:[Polyneuropathy associated with osteosclerotic myeloma. Excellent response to chemotherapy]. 219 67


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