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)

1 61-year-old man had osteosclerotic myeloma that was localized in the eleventh thoracic vertebral body and associated with sensorimotor polyneuropathy, skin hyperipigmentation, edema, hypertrichosis, gynecomastia, and white nails. Cases of osteosclerotic myeloma with and without polyneuropathy in the literature were reviewed with special reference to accompanying dermatologic and endocrinologic signs and synmptoms. We assume that the polyneuropathy, cutaneous hyperpigmentation, edema, hypertrichosis, gynecomastia, and white nails are causally related to each other and are a remote effect of osteosclerotic myeloma. Quantitative histologic analysis of two sural nerves biopsied within 2 years of each other during the course of the disease indicated that both large and small myelinated fibers degenerated progressively, with relative preservation of unmyelinated fibers.
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PMID:Polyneuropathy, skin hyperpigmentation, edema, and hypertrichosis in localized osteosclerotic myeloma. 55 75

The combination of osteosclerosis, polyneuropathy, monoclonal immunoglobulin, hypertrichosis, serositis and a number of other symptoms is described. It seems probable that this is a special type of myeloma. Similar cases have been described in Japan and out findings are compared with the Japanese picture. The age of the patients is unusually low. The M-component in the plasma is small. There is very little Bence Jones protein in the urine and osteolytic lesions in the skull do not seem to have occurred. The polyneuropathy may improve during treatment with cytostatic drugs.
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PMID:Osteosclerotic "plasmocytoma" with polyneuropathy, hypertrichosis and diabetes. 64 43

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

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

A case of chronic disabling sensory-motor polyradiculitis occurring in a female patient and followed up for 18 months is reported. Clinical findings were papilledema, hypertrichosis, abnormal pigmentation of the skin, generalized edema, and spontaneous cutaneous necrosis. The diagnosis of a plasmocytic dyscrasia to IgA Lambda was made at a late stage of the disease. There were no signs of a solitary plasmocytoma or of diffuse myeloma. At autopsy, there were mild lesions in the peripheral nervous system, but no plasmocytic infiltration or amylosis was seen on optical or electron microscopy. This case is similar to other clinical cases reported mainly by Japanese authors.
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PMID:[Chronic polyradiculitis with cutaneous and endocrine signs suggesting a plasmocytic dyscrasia to IgA (author's transl)]. 741 49

A 48 years old male is reported. He presented with lower limb progressive and severe polyneuropathy, hypertrichosis, endocrinological alterations (hypothyroidism and hypogonadism) and organomegaly (hepatosplenomegaly and lymphadenopathies). This syndrome was associated with an osteosclerotic myeloma. The patient died two months after admission.
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PMID:[POEMS syndrome. A case report]. 772 96

The patient is a 60-year-old man who developed numbness of the extremities, paralysis, hyperpigmentation of the skin, hypertrichosis, anasarca and chest pain at the age of 58 years. The diagnosis of Crow-Fukase syndrome was made and myeloma was not found. Prednisolone therapy was effective but chest pain reappeared every morning when prednisolone was tapered to 30 mg alternate day. Coronary arteriogram showed no stenosis but administration of acetylcholine into the coronary artery produced ST elevation in electrocardiogram, chest pain and coronary artery stenosis which were relieved by administration of nitrates into the coronary artery.
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PMID:Crow-Fukase syndrome: a case associated with vasospastic angina. 785 70

POEMS syndrome has been defined as an association of plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. Although certain authors do not distinguish this syndrome from osteosclerosing myeloma, syndromes comparable to POEMS have been observed without bone lesions. Others have described the clinical features involved under the terms of Crow-Fukase's syndrome, PEP syndrome (pigmentation, oedema, plasma cell dyscrasia, or Takatsuki's syndrome. Seen in men twice as often as in women, usually between the ages of 40-50 years, all five clinical features are not always present or may be accompanied by other signs. The first sign of the peripheral polyneuropathy is usually sensorial impairment followed by distal then proximal motor deficit. The deficit is usually severe and 50% of the patients become unable to walk. Cranial nerves are rarely involved. Liver, spleen and lymph node enlargement are observed. The most frequent signs of an endocrinopathy are gynaecomastia, atrophy of the testicules, impotence and amenorrhoea. Testosterone levels are low and oestrogen levels are increased in men together with luteinizing hormone, follicle stimulating hormone and prolactin. Hypothyroidism is frequent and diabetes mellitus is observed in 50% of the patients. Protein M is a monoclonal immunoglobulin (IgG or IgA), almost always with a light lambda chain. Skin changes include hyperpigmentation, hypertrichosis, hyperhidrosis, thickening of the skin suggestive of sclerodermia and papillary angiomas. Other signs, especially peripheral oedema often occur early in the disease course and may precede the peripheral neuropathy. POEMS syndrome is often associated with a myeloma (up to 50% of the cases in certain series). Although immunoglobulin deposit on myelin sheaths, anti-endocrine antibodies and receptors of lambda chains have been proposed as playing a role, no mechanism of pathogenesis has been determined. The natural history of the disease leads to a severe polyneuropathy. The patients become totally bedridden and death results from complications of decubitus rather from the direct effect of the underlying dyscrasia. When bone lesions are minor, radiotherapy or surgery can improve the neuropathy and resection of a solitary plasmocytoma can lead to total remission. Chemotherapy or corticosteroids may improve the polyneuropathy in certain cases. Plasma exchange has not been successful.
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PMID:[POEMS syndrome]. 807 60

POEMS syndrome is a rare condition with cutaneous manifestations commonly including angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphadenopathy, erythematous thickening of the skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring alopecia and sparse follicular pustules at the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monoclonal gammopathy, subclinical thyreopathy, and an osteolytic bone lesion of the skull. Histologically, a plasmacytoma with lambda cell restriction was found. The overlying skin showed marked fibrosis, with loss of hair follicles, and a plasma cell infiltrate of polyclonal origin. The cervical lymph nodes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There was no evidence of further plamacytomas. After excision of the plasmacytoma and postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further folliculitis or hair loss on the rest of the scalp. This case illustrates the reactive character of POEMS syndrome as a paraneoplastic syndrome in myeloma patients.
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PMID:POEMS syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma. 1032 21