Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1762617 (weakness)
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A rare form of plasma cell dyscrasia characterized by associated polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes has been termed the POEMS syndrome. The pathophysiology is unknown; plasma cell dyscrasia is essential; secondary manifestations are unexplained. We report a 67-year-old man with a 7-month history of progressive weakness and numbness of the legs. Clinical examination revealed sensorimotor polyneuropathy, predominantly affecting the lower extremities, hepatomegaly, and skin haemangiomas. Additional investigations disclosed IgG-lambda monoclonal serum protein, endocrine abnormalities, elevated cerebrospinal fluid protein level and an osteoblastic lesion of the lumbar vertebra. Biopsy of the osteosclerotic vertebra showed a marked lymphoplasmocytic infiltrate. MRI of the liver disclosed two haemangiomas; this association has not been reported previously.
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PMID:Polyneuropathy with osteosclerotic myeloma--POEMS syndrome. A case report. 154 70

A 40-year-old man was admitted to our hospital because of paresthesia and weakness of the limbs. At the age of 38, he was diagnosed as having an idiopathic thrombocytopenic purpura (ITP) which have been refractory to oral administration of prednisolone and splenectomy. Platelet-associated IgG was elevated markedly at that time. It was, however, only mildly elevated on this admission. He showed polyneuritis, generalized pigmentation, hirsutism, and marked edema on the legs. The bone X-ray disclosed a lytic lesion in the left iliac bone, which was confirmed as a plasmacytoma by bone biopsy. Axonal degeneration with marked loss of myelinated figure was seen on sural nerve biopsy. Serum immunoelectrophoresis revealed his monoclonal IgG was lambda type. Then, he was diagnosed as having a Crow-Fukase syndrome associated with ITP. Plasma exchange, pulse therapy, and irradiation to plasmacytoma resulted in a slight improvement of the polyneuritis and the skin symptoms, and a disappearance of edema. However, ITP has not responded to these therapies. Although the same autoimmune mechanism is suggested in these conditions, we could not clarify how this monoclonal IgG produce both polyneuritis and ITP.
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PMID:[A case of Crow-Fukase syndrome associated with idiopathic thrombocytopenic purpura]. 188 77

We present a case of an unusual multi-systematic disorder whose cardinal signs were severe progressive sensorimotor polyneuropathy, hepatomegaly, endocrinopathy, plasma cell dyscrasia with osteosclerotic bone lesions and M-protein production as well as skin lesions (termed POEMS Syndrome) whose pathophysiology is still unknown, as well as its relationship with myeloma multiplex. The patient, 67 years old, had a history of progressive weakness and numbness of the lower legs, clinically revealed as sensomotor polyneuropathy, hepatomegaly, IgG lambda monoclonal protein in the serum, endocrine abnormalities and skin lesions. The final and definite diagnosis was established by open, surgical biopsy of the second lumbal vertebra.
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PMID:[Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes: the POEMS syndrome]. 820 22

A rare case of Crow-Fukase syndrome was reported, which developed 7 years following myelopathy of unknown origin. In September 1983, a 31-year-old man came to our department for progressive gait disturbance and numbness in both lower extremities. Examination on admission showed hyperreflexia with clonus and moderated muscle weakness of legs, and paresthesia below Th9 level, but myelography and CT indicated no abnormality. Thereafter, he was doing as well as walkable with a cane, but in 1989 he developed hypertrichosis, skin pigmentation, leg edema and gynecomastia with aggravation of numbness of lower extremity and was rehospitalized in October. Detailed examination on the present admission indicated compression of the conus medullaris due to an osteosclerotic lesion of vertebral body L1, which did not correspond to the possible site of pyramidal sign. Thus, the origin of the myelopathy remained unknown. After hospitalization, polyneuropathy newly developed, making him confined to his bed. Thus, the diagnosis was established as Crow-Fukase syndrome. Subtotal extirpation of L1 vertebral body and steroid therapy (PSL 10 mg/day) caused improvements of polyneuropathy and numbness of lower extremity, making him walkable again and return to his social work 3 months later. Pathohistological examination of the osteosclerotic lesion proved partial plasmacytoma. In MRI findings of the osteosclerotic lesion, the osteosclerotic and plasmacytomatous regions were outlined as low and high signals, respectively for both T1 and T2-weighted. MRI examination was very useful for grasping the tumorous extent within the osteosclerotic lesion as observed with Crow-Fukase syndrome and for determining the extent of indication for surgery and radiotherapy.
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PMID:[A case of Crow-Fukase syndrome which developed seven years following myelopathy of unknown origin]. 833 76

We report a case of POEMS syndrome with various endocrine dysfunctions. A 49-year-old man was admitted to our hospital for pretibial edema and general fatigue. He had weakness of the lower extremities, hepatomegaly, monoclonal protein (IgG-lambda type), impotence, pigmentation and hypertrichosis. Endocrinological examination revealed impaired glucose tolerance, primary hypothyroidism and hypogonadotropic hypogonadism. After three months of treatment with corticosteroids, he showed an improvement in gonadotropin secretion, but no considerable change in the secretion of the other hormones. To our knowledge, this is the first case that showed an improvement in gonadotropin secretion as a result of corticosteroid therapy in POEMS syndrome.
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PMID:Improvement in gonadotropin secretion after corticosteroid therapy in a case of POEMS syndrome. 979 Feb 78

Multiple myeloma is characterized by the presence of bone pain, weakness, and fatigue. Ninety-eight percent of patients have an M-protein in the serum or urine at the time of diagnosis. Skeletal roentgenograms are abnormal in nearly 80%. Renal insufficiency (creatinine > or = 2 mg/dL) is present in one-fourth. The major causes of renal insufficiency are "myeloma kidney" and hypercalcemia. The diagnosis of multiple myeloma depends upon the presence of more than 10% plasma cells or a plasmacytoma plus an M-protein in the serum or urine or lytic bone lesions. Multiple myeloma must be differentiated from monoclonal gammopathy of undetermined significance and smoldering multiple myeloma. The plasma cell labeling index and the presence of circulating plasma cells in the peripheral blood are helpful in the differential diagnosis. Plasma cell leukemia, osteosclerotic myeloma (POEMS syndrome), and plasmacytomas are discussed. The heavy-chain diseases consist of alpha, gamma, and mu heavy-chain disease. The fibrils of primary amyloidosis consist of kappa or lambda monoclonal light chains. Weakness, fatigue, and weight loss are the most frequent symptoms. Macroglossia occurs in 10%. An M-protein is found in the serum or urine in 90%. The presence of nephrotic syndrome, renal insufficiency, congestive heart failure, orthostatic hypotension, or sensorimotor peripheral neuropathy, and an M-protein in the serum or urine suggest the possibility of primary amyloidosis. The diagnosis depends upon the demonstration of amyloid in tissues. The subcutaneous fat aspirate is positive in 80% while the bone marrow is positive in 55%. If these tissues are negative, one should obtain tissue from an involved organ.
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PMID:Clinical aspects of multiple myeloma and related disorders including amyloidosis. 1019 81

The POEMS syndrome is a rare multisystemic disorder with polyneuropathy, organomegaly, endocrinopathy of various forms, production of monoclonal (M) component, and skin changes. We describe a 46-year-old man who developed ascites one year after the onset of peripheral neuropathy with accompanying muscle atrophies and increasing weakness. Extensive evaluation revealed that the patient had no underlying liver disease, malignancy, infection, or cardiac or renal disease. The ascites initially responded to high-dose corticosteroid therapy. The patient had many clinical features of the described POEMS syndrome including sclerotic bone lesions, a persistent lambda-paraprotein and refractory ascites. In this case ascites was a main presenting feature. Thus, the POEMS syndrome must be added to the list of rare causes of refractory ascites.
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PMID:[46-year-old patient with polyneuropathy, hepatosplenomegaly, endocrinopathy, M-gradient, skin manifestations, sclerotic bone changes and therapy refractory ascites]. 1037 64

POEMS syndrome is a multisystem disorder with signs such as peripheral neuropathy, organomegaly, endocrinopathy, monoclonal protein, skin lesions, papilledema, and increased cerebrospinal fluid proteins, which can also evolve with renal and cardiac affection. It is considered a result of a plasma cell cyscrasia with the production of a monoclonal protein. A 46-year old man was seen as an outpatient referring progressive weakness of legs and arms, fever, impotence, inguinal and cervical lymphadenopathies, peripheral edema, hepatomegaly and skin hyperpigmentation. In laboratory test, platelet count was between 528 x 10(9)/L and 599 x 10(9)/L, creatinine clearance 27.2 ml/min, proteinuria 0.8 g/dl, IgA 455 mg/dl, T3 30 ng/100 ml, T4 2.6 vg/dl, T4F 0.5 ng/dl, TSH 12.4 vU/ml; testosterone 1.56 ng/ml. The electromyography showed a mixed sensitive-motor pattern. On the pelvis radiography, an osteosclerotic lesion on the left sacroiliac joint was identified. Bone biopsy of the site of the sclerotic lesion revealed plasma cell dyscrasia. The patient was treated with diuretics, digitalis and prednisone. Diagnosis of this disorder is difficult because of the multipathology it is necessary to establish differential diagnosis.
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PMID:[POEMS syndrome. Report of a case]. 1042 26

The contemporary presence of organomegaly, skin manifestations, polyneuropathy, endocrinopathy and monoclonal component characterises the POEMS syndrome, often associated with osteosclerotic myeloma and Castelman's disease and more frequent in the Japanese. Clinical manifestations seem to be related to the production of many interleukins, mainly IL-1, IL-6 and TNF. Several endocrinopathies have been described, the most frequent being diabetes. Only one previous case of hypoparathyroidism associated with the syndrome has been described in medical reviews. Polyneuropathy is often sensitivo-motory and skin disease accounts for Raynaud phenomenon, skin pigmentation, hypertricosis and others. We describe the case of a 74-year-old man who underwent clinical examination for weakness mainly in the legs. Clinical and instrumental data showed rhabdomyolysis due to hypoparathyroidism. The contemporary presence of a monoclonal band of light chains on proteic electrophoresis, organomegaly and distal leg neuropathy allowed us to make a diagnosis of POEMS syndrome.
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PMID:A man with worsening weakness. 1078 Jan 92

We here reported a 54-year-old female patient with Crow-Fukase syndrome associated with pulmonary plasmacytoma. She was found to have scattered tumor in 1990. Although the tumor had slowly grown for the last 10 years, she showed no clinical symptoms. Numbness and weakness of lower extremities began in June 1999, and she was referred to Kyoto University Hospital on Oct. 21 1999 for evaluation of progressive symptoms. She had skin pigmentation, edema of the lower extremities, lymphadenopathy, muscle weakness and sensory disturbance in a glove-and-stocking distribution. Serological examination showed monoclonal IgG-lambda gammopathy. Serum vascular endothelial growth factor (VEGF) was markedly elevated. Microscopic studies on biopsied sural nerve demonstrated mild decrease of myelinated fibers. Immunohistochemically, the pulmonary tumor was defined as an IgG (lambda type) plasmacytoma. After treatment with melphalan-prednisolone therapy, the neurological symptoms improved along with decrease of serum VEGF levels as well as the size of pulmonary plasmacytoma. This is the first report of a patient with Crow-Fukase syndrome associated with pulmonary plasmacytoma. This case suggests that growth of pulmonary plasmacytoma might have played an important role in the overproduction of VEGF and thus development of Crow-Fukase syndrome.
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PMID:[A patient with Crow-Fukase syndrome associated with pulmonary plasmacytoma]. 1148 54


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