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)

A patient with the syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) had multiple myeloma and IgA-kappa monoclonal gammopathy. To our knowledge, of over 50 cases in the literature, this is the only instance of kappa light chains noted in a patient with the complete syndrome. Sural nerve biopsy revealed increased immunofluorescence for IgA and kappa chains. Furthermore, this patient had a hemorrhagic diathesis characterized by a prolonged bleeding time and a prolonged thrombin time due to a qualitative platelet dysfunction and an apparent inhibition of fibrin monomer polymerization. In none of the other cases of POEMS syndrome was a coagulopathy reported. A short course of plasmapheresis improved the bleeding time but did not alter the polyneuropathy.
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PMID:Multiple myeloma with polyneuropathy and coagulopathy. A case report of the polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin change (POEMS) syndrome. 308 99

Three cases of the Crow-Fukase syndrome without radiographic changes of multiple myeloma are reported, with special reference to the glomerular changes seen. Proteinuria was detected in one case, although decreased renal function was observed in all (GFR: 41.0, 62.0, 74.1 ml/min respectively) at the time of renal biopsy. Glomerular changes were similar in all three cases. The main characteristic changes were mesangial proliferation and thickening of the glomerular capillary walls. Pictures by light microscopy were therefore similar to that of MPGN. On electron microscopy, the thickened capillary walls showed circumferential mesangial interposition and the subendothelial zone was electron-lucent and contained small dense granules or flocculent deposits. By immunofluorescent microscopy, no immunoglobulins, complement components or light chain were detected in the glomeruli except in one case.
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PMID:Glomerular lesions associated with the Crow-Fukase syndrome. 308 38

The case is reported of a 32-year-old woman with polyneuropathy, organomegaly, edema, endocrinopathy, dark skin and solitary myeloma with monoclonal gammopathy of IgG/lambda type. More than 100 cases of this rare disorder have been observed up to now, mainly in Japan. In recent reviews the terms POEMS-syndrome or Crow-Fukase-syndrome have been used. Local radiotherapy of the myeloma led to lasting regression of symptoms. Studies with immunocytochemistry and immunoelectroblotting revealed specific antibody activity against hypophysis, suggesting that the pathologic monoclonal myeloma antibodies may damage the neuroendocrinic centers in hypothalamus and hypophysis as the primary target. Most of the multisystemic symptoms would thus be explained as secondary alterations.
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PMID:[Polyneuropathy, organomegaly, endocrinopathy and skin changes in a case of solitary myeloma]. 309 18

We describe three patients with sclerotic multiple myeloma. One patient had diffuse bone sclerosis, one had mixed sclerotic and lytic lesions, and the third had POEMS syndrome. The radiological, clinical, and etiological features of sclerotic myeloma variants are discussed.
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PMID:Osteosclerotic myeloma variants. 336 76

In a case of Crow-Fukase (POEMS) syndrome there was a chronic, progressive, and eventually lethal polyradiculoneuropathy. In addition, adenomegaly, oedema and pleural effusions, gonadic atrophy, serum monoclonal IgA, and skin pigmentation were present. Plain x-rays and CT scan of the pelvis and lower vertebrae showed multiple poorly defined lesions. At postmortem there was no myeloma and a bone mastocytosis was found. In addition, next to T11, there was an abdominal nodule, 2 cm in diameter, with histological characteristics of Castleman's angiofollicular lymphoid hyperplasia. Immunohistochemical studies showed that plasmocytes of this lesion secreted polyclonal immunoglobulins with a high prevalence of IgA. Thus, the primary interest of this case lies in the association of bone mastocytosis with a POEMS syndrome. Also, one single localisation of Castleman angiofollicular lymphoid hyperplasia was found, assumed to be in this case the cause of the POEMS syndrome. Therefore, a minute and benign hyperplasic lesion, which was only discovered at autopsy, secreted the protein responsible for the symptoms and signs, and eventually the patient's death.
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PMID:[Crow-Fukase syndrome (POEMS syndrome) and osseous mastocytosis secondary to Castleman's angiofollicular lymphoid hyperplasia]. 382 4

Three patients with an unusual multisystemic syndrome characterized by polyneuropathy, organomegaly *especially hepatosplenomegaly), endocrine dysfunction, M-protein, and skin abnormalities (POEMS syndrome) are discussed. Characteristic radiographic features include single or multiple osteosclerotic lesions and peculiar variety of bony proliferation. The relationship of the disorder to multiple myeloma and plasmacytoma and the pathogenesis of the skeletal abnormalities remain obscure.
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PMID:Plasma-cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes: the POEMS syndrome. Distinctive radiographic abnormalities. 724 23

Immunofluorescent staining (immunofluorescence bone marrow aspirate) and immunoperoxidase staining (immunoperoxidase bone marrow biopsy) were compared in 26 patients with plasma cell dyscrasia and less than 10% marrow plasma cells. Their clinical diagnoses included monoclonal gammopathy of undetermined significance (13 patients), treated multiple myeloma (four patients), multiple myeloma with less than 10% marrow plasma cells (two patients), primary systemic amyloidosis (two patients), monoclonal gammopathy of undetermined significance with neuropathy (two patients), angiofollicular lymph node hyperplasia (two patients, all with the POEMS [polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes] syndrome), and primary (amyloidosis) amyloid neuropathy (one patient). The percentage of plasma cells was greater than 5% in 23% of patients and less than or equal to 5% in 77% of patients. With immunofluorescence bone marrow aspirate and immunoperoxidase bone marrow biopsy, light-chain restriction was demonstrated in 84% of all cases and accurately determined in 96% of all cases as shown by serum and urine paraprotein analysis or tissue amyloid typing. Monoclonal populations of plasma cells can be readily identified with immunofluorescence bone marrow aspirate and immunoperoxidase bone marrow biopsy in most patients with paraproteins and marrow plasmacytoses not diagnostic of multiple myeloma.
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PMID:Bone marrow aspirate immunofluorescent and bone marrow biopsy immunoperoxidase staining of plasma cells in histologically occult plasma cell proliferative marrow disorders. 752 Feb 28

We report six patients affected by POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes), a peculiar multiorgan disease frequently associated with osteosclerotic myeloma or other plasma cell disorders. Sensorimotor polyneuropathy was associated with multisystem involvement in all of the patients, with osteosclerotic myeloma in 2 cases, monoclonal gammopathy of undetermined significance in 2 cases and Castleman's disease in the final two. In all of the patients, sural nerve biopsy findings were consistent with a mixed, axonal and demyelinating neuropathy. Increased levels of Interleukin-6 were found in two cases, but the pathogenesis of the disease is far from established.
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PMID:The POEMS syndrome: report of six cases. 769 93

The association of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes forms a characteristic multisystem syndrome, Crow-Fukase syndrome, or acronym POEMS syndrome. The pathogenesis of the syndrome is still unknown. An M component found in more than three quarters of the patients appears not be the direct cause, because no evidence of deposition or interaction was observed in skin, peripheral nerve or endocrine organs with few exceptions. Solitary or multiple bone lesions, or plasmacytoma, are found in more than half of patients with Crow-Fukase syndrome. The nature of abnormal plasma cells of Crow-Fukase syndrome seems to be distinct from that of multiple myeloma. Substances elaborated by plasmacytoma cells are recently postulated as a pathogenesis of the disorder because of the finding that the Crow-Fukase syndrome may regress after resection or irradiation of solitary plasmacytoma, although a definite conclusion awaits further investigations.
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PMID:[Crow-Fukase syndrome]. 769 17

A young Italian woman with a POEMS syndrome is described. The patient had a plasma cell dyscrasia without clinical or laboratory evidence of multiple myeloma. The phenotypic analysis of bone marrow cells and peripheral blood lymphocytes revealed a normal pattern. The immunological study of CSF showed high levels of interleukin-6, whereas this cytokine was not detectable in the serum. Electrophysiological studies and sural nerve biopsy showed a mixed, demyelinating-axonal sensorimotor neuropathy with marked loss of large myelinated fibres. Long-term treatment with prednisone gave some clinical improvement.
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PMID:POEMS syndrome: clinical, pathological and immunological study of a case. 770 42


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