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 42-year-old Italian man affected with a multisystemic disease is presented. The main features were: polyneuropathy, organomegaly (liver, spleen and lymph nodes enlargement), endocrinopathy (loss of libido, low plasmatic levels of testosterone), monoclonal protein (k-light chains only in 100-fold-concentrated urine sample, without other signs of plasmocytic proliferation), skin changes. Clinical skin alterations were striking: diffuse thickening, hyperpigmentation, hyperhidrosis , hypertrichosis, while histo- and immunopathological examination of skin biopsies showed proliferation of dermal collagen fibers and deposition of melanin in the epidermis. These findings were suggestive for the diagnosis of Crow-Fukase (POEMS) syndrome, associated with peculiar angiofollicular lymph node hyperplasia (Castleman's disease). The patient was followed up for 15 months with steroids, systemic chemotherapy, plasma-exchange and immunomodulating drugs. A massive anasarca complicated the picture leading him to death. Actually the pathomechanisms of this rare disease have not been fully elucidated. The relations between this syndrome and some malignant lymphoproliferative diseases (i.e. osteosclerotic myeloma) are controversial; at least a part of its features could be either reactive or tissue-specific-antibody mediated. A genetic influence should be suggested from the Japanese reports.
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PMID:[Crow-Fukase syndrome (POEMS syndrome). The first Italian presentation of a case and review of the literature]. 219 31

Three disorders that bear the eponym of Castleman's disease (CD) are discussed. The localized CD of hyaline-vascular (HV) type features an architecturally abnormal, hypervascular lymphoid tissue with burned-out germinal centers, and presents as an asymptomatic, slowly growing mass. It may represent a lymphoid hyperplasia associated with excessive angiogenesis. The localized CD of plasma cell (PC) type, instead, features an architecturally recognizable lymph node with solid sheets of PCs and presents with systemic manifestations of inflammation and B cell hyperreactivity. It appears as a localized chronic reaction to unknown antigens. "Multicentric" CD indicates a clinicopathologic entity characterized by the histology of CD of "mixed" type, a predominantly lymphadenopathic presentation consistently involving peripheral nodes, manifestations of multisystem involvement, and an idiopathic nature. It is best considered as a systemic B cell lymphoproliferation, which probably arises in a setting of immunoregulatory deficit, and may result in the outgrowth of clonal B cell populations. An attempt is presented to place the latter two forms of CD in the larger perspective of idiopathic PC disorders, by taking into account both the nature of the diseases (hyperplastic, dysplastic, neoplastic) and the lymphocyte traffic system that is involved (bone marrow-bound, mucosa-associated or peripheral-node-bound). In such a scheme, localized CD, PC type, and multicentric CD appear as a hyperplastic and a dysplastic disorder, respectively, of peripheral-node-bound B cells, related to, and often associated with, primary nodal plasmacytoma and osteosclerotic myeloma (so-called POEMS, Takatsuki's or Crow-Fukase's syndrome).
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PMID:Castleman's disease and related disorders. 246 87

The rare POEMS syndrome which combines hematology, bone, endocrine, neurology and skin manifestations, associated to a non-frequent osteosclerotic type of multiple myeloma is reviewed. The clinical, analytical, radiologic, immunologic, electrophysiologic and histopathologic features are detailed, as well as the different pathogenetical theories of its several manifestations, in an attempt to unify them. The different treatment schedules following the onset and corresponding prognostic are detailed.
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PMID:[The POEMS syndrome]. 249 Oct 91

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 has polyneuropathy of the upper and lower extremities, splenomegaly, impotence, IgA-lambda monoclonal protein (M-protein), and marked thickening of his skin. In addition, he has polycythemia vera. Although myeloproliferative disorders have been reported to occur in association with multiple myeloma, they have not been described with POEMS syndrome. The possible etiology of this association is discussed. This patient was successfully treated with melphalan and prednisone at the time of his initial diagnosis, but relapsed 10 years later. The relapse was treated with pulse doses of prednisone alone with complete resolution of his polyneuropathy and skin changes. This was accompanied by a fall in his IgA levels and improvement of his polycythemia.
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PMID:POEMS syndrome associated with polycythemia vera. 253 36

We report the case of a 46-year-old woman presenting with a disorder characterized by the association of multicentric Castleman's disease, organomegaly, a solitary IgA lambda myeloma with lytic bone lesions but no serum monoclonal peak, peripheral neuropathy and skin changes. This unusual association resulted in an incomplete form of that peculiar multisystem syndrome described under a variety of names: Crow-Fukase syndrome, Shimpo's syndrome, Takatsuki's syndrome, PEP syndrome or POEMS syndrome. The possible relationship between localized and multicentric Castleman's disease with myeloma or extramedullary plasmacytoma on one hand and on the other hand with polyneuropathy and the other symptoms of the POEMS syndrome is discussed.
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PMID:Multicentric angiofollicular lymph node hyperplasia associated with a solitary osteolytic costal IgG lambda myeloma. POEMS syndrome in a South American (Paraguayan) patient. 260 20

In Japan, Crow-Fukase syndrome is a target of active research because of many cases reported. Therefore, the following three topics regarding this syndrome were discussed. 1) Etiology of Crow-Fukase syndrome. Even in reported cases of Crow-Fukase syndrome with monoclonal protein or polyclonal immunoglobulins and no apparent multiple myeloma, there were plasmacytomas in the lymph node or other tissues, and symptoms became improved after removal of these tumors. Therefore, plasma cells seem to be involved. According to recent studies, impairment of the pituitary function may play some role. 2) New pathological findings in the lymph node histology. In the lymph node of patients with this syndrome, we found in its germinal center a peculiar abnormality called angiosclerosis, in which branching tortuous capillaries with thickened wall including many cells were prominent, which may be different from those of Castleman's disease. 3) Nerve conduction studies using a new collision technique. Crow-Fukase syndrome is associated with marked slowing of even the maximal motor and sensory conduction velocities. Therefore, in order to understand its pathophysiology better, submaximal conduction velocities should be measured using the new collision technique because this technique is different from Hopf's method that is affected by the refractory period of nerve fibers.
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PMID:[Crow-Fukase syndrome]. 269 95

Specific antibody binding activity directed against pituitary tissue was demonstrated in a typical case of POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome with paraproteinemia of IgG lambda type. IgG binding against various other tissues could not be detected by immunoelectroblotting and immunocytochemistry. We propose that POEMS syndrome is an antibody-mediated immune disorder, in which the hypophysis is the primary target of the pathologic myeloma antibodies. The immunopathogenetic lesion of the hypophysis could induce secondary disturbances of other organs, thus explaining the multisystemic disorder.
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PMID:Specific antibody activity against neuroendocrine tissue in a case of POEMS syndrome with IgG gammopathy. 283 85

We describe the clinical, radiographic, and pathologic manifestations of a group of disorders characterized by osteosclerosis in association with plasmacytic infiltration of bone marrow. These conditions include multiple myeloma, plasma cell granuloma, sternocostoclavicular hyperostosis, the POEMS syndrome (plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes), and chronic symmetric plasma cell osteomyelitis of childhood. Although clinically unrelated in many respects, features shared by these diseases support the existence of a specific factor linking the plasma cell to local osteogenesis.
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PMID:Plasma cell sclerosis of bone: a spectrum of disease. 293 79

A patient with osteosclerotic myeloma and POEMS syndrome, unresponsive to pulse prednisone and melphalan therapy, was admitted to the hospital for a trial of plasma exchange therapy. The presentation included IgG lambda monoclonal gammopathy, peripheral neuropathy, hepatosplenomegaly, hyperpigmentation and thickening of the skin, edema, and tense ascites. Laboratory tests confirmed hypothyroidism, hypogonadism, and adrenal insufficiency. Six exchange procedures failed to affect the clinical course, and the patient died. Greater-than-one-plasma-volume exchanges (patient's measured plasma volume, 2,703 cc) were performed. When IgG and cholesterol removal were compared to the predicted removal, based on the volume of plasma removed, significantly less reduction in concentration than predicted was measured. IgG concentrations increased postapheresis and, at 2 weeks, three-fourths of the removed IgG had reaccumulated. A reduced efficiency of removal of both IgG and cholesterol can be explained by postulating increased vascular permeability with free exchange of soluble substances from one compartment to another. If an abnormal product is produced by the disease and is responsible for the clinical syndrome, a more intensive schedule of plasma exchange therapy may be needed to achieve a sustained depletion of the responsible soluble substance. Alternatively, neither increased vascular permeability or the clinical manifestations are responsive to removal of a soluble substance or are caused by a soluble substance produced by the malignancy.
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PMID:Therapeutic trial of plasma exchange in osteosclerotic myeloma associated with the POEMS syndrome. 299 55

Three patients with POEMS syndrome were studied: two of them had myeloma, whereas the third had M protein without myeloma. Peripheral nerve biopsies showed no deposits of anti-Ig sera at direct immunopathological examination. Ultrastructural study revealed myelino-axonal degeneration in all three cases. This was associated in the first case with a dramatic loss of myelinated fibers and there were lesions consistent with acute degeneration in the second case. In addition, all three had some fibers showing uncompacted myelin lamellae. This peculiar modification has previously been reported only in cases of dysglobulinemia, lymphoma and inflammatory demyelinating polyneuropathy, but its mode of formation remains unclear.
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PMID:Uncompacted lamellae in three patients with POEMS syndrome. 304 Apr 18


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