Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 39-year-old woman presented with polyneuropathy, hepatomegaly, splenomegaly, endocrinopathy, monoclonal protein and skin changes, several of the many clinical features of the recently described POEMS syndrome. In addition, she had a Castleman's disease (angiofollicular lymph node hyperplasia). In this case ascites was a main presenting feature. Thus, the POEMS syndrome must be added to the list of rare causes of ascites. Electron microscopy of the liver showed perisinusoidal fibrosis.
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PMID:[Unusual cause of ascites: the POEMS syndrome]. 222 42

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

A 48-year-old woman had a variation of the syndrome of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (the so-called POEMS syndrome). The patient's neurological findings were entirely normal, but she had splenomegaly, hyperprolactinemia with galactorrhea and oligomenorrhea, a thyroid nodule with evidence of mild thyroiditis on aspiration biopsy specimen, and IgG-kappa monoclonal gammopathy, and hyperpigmentation and thickening of the skin. A short course of plasmapheresis (twelve 4-L exchanges in one month) did not alter any of the clinical abnormalities, but did result in a 70% decrease in the monoclonal IgG level (from 2.2 to 0.7 g/dl).
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PMID:POEMS syndrome: studies in a patient with an IgG-kappa M protein but no polyneuropathy. 680 33

In a 52-year-old man with general malaise, muscle stiffness and weakness, POEMS-syndrome was diagnosed based on polyneuropathy, splenomegaly, lymphadenopathy, subclinical hypothyroidism and the presence of a monoclonal paraprotein with osteosclerotic lesions and an indurated skin (POEMS is an acronym for Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes). This is a rare systemic disease from the clinical spectrum of plasma cell dyscrasias with polyneuropathy. The clinical picture is broader and more pleomorphic than the acronym suggests. The possibility of a POEMS syndrome should be considered in the differential diagnosis of polyneuropathy in association with monoclonal gammopathy. Quite often it is associated with osteosclerotic myeloma or mixed osteoscleroticlytic lesions. The patient described was treated with high dose corticosteroids which were gradually decreased over the next three months, upon which a marked improvement could be seen. The general malaise subsided, as did the splenomegaly, and the skin became supple again.
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PMID:[A man with plasma cell dyscrasia and polyneuropathy due to POEMS syndrome]. 1187 37

A 73-year-old woman was referred to our hospital because of gait disturbance. She had peripheral polyneuropathy which was mainly of the demyelinating type, splenomegaly, a skin change and M protein (IgA-lambda). A bone scinti and a CT scan showed a mass lesion in the thoracic vertebra, and a biopsy revealed plasmacytoma. She was diagnosed as Crow-Fukase syndrome, and treated with local radiation therapy. After the treatment, M protein became undetectable, and gait disturbance was improved.
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PMID:[Successful treatment of Crow-Fukase syndrome with radiation therapy]. 1204 12

We report a case of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes) with unusual clinical features. A 62-year-old woman presented a severe polyneuropathy with dysphonia and vegetative symptoms, including bradycardia and sphincterial disorders. The clinical examination showed facial hyperpigmentation, cachexia, anasarca and splenomegaly. She also presented restrictive cardiomyopathy and endocrine disturbances. Nerve conduction studies revealed a severe demyelinating sensorimotor neuropathy. Cerebrospinal fluid analysis showed an elevated protein level. We detected a biclonal gammapathy (Ig G and Ig A with lambda light chain) and lytic pelvic bone lesions. Later, she developed a severe ventilatory failure due to a bilateral phrenic nerve paralysis leading to a mechanical ventilation. Steroids followed by localized radiotherapy partially improved the respiratory status and stabilized the neuropathy. Phrenic nerve paralysis, restrictive cardiomyopathy, vegetative symptoms and cranial nerve palsy are exceptional in POEMS syndrome. Moreover, this case emphasizes the importance of radiological investigations since the discover of plasmocytoma may improve the prognosis of POEMS syndrome.
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PMID:[Bilateral phrenic nerve paralysis, dysautonomia and restrictive cardiomyopathy in a case of POEMS syndrome]. 1248 6

A middle-aged man presented with progressive dyspnea and a pleural effusion. He had slowly progressive bilateral symmetric extremity weakness, mediastinal lymphadenopathy, and splenomegaly. The diagnosis of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-Protein and Skin changes was suspected clinically and confirmed with a bone marrow biopsy. He responded symptomatically to corticosteroids and is awaiting a stem cell transplant.
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PMID:POEMS syndrome. 1538 41

A 55-years-old male, who presented with insidious onset gradually progressive sensorimotor polyneuropathy, POEMS-syndrome was diagnosed based on polyneuropathy, splenomegaly, hypothyroidism, the presence of IgG-monoclonal serum protein with osteosclerotic lesions and hyperpigmention of skin. Biopsy of the osteosclerotic lesion from the right superior pubic rami was consistent with plasmocytoma. Electrophysiological studies revealed demyelinating sensorimotor neuropathy and biopsy from sural nerve showed demyelinating neuropathy with secondary axonopathy. The patient showed improvement with radiotherapy. This is a rare systemic disease from the clinical spectrum of plasma cell dyscrasias with polyneuropathy. The importance of POEMS syndrome in the differential diagnosis of polyneuropathies has been emphasized.
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PMID:Polyneuropathy with osteosclerotic myeloma--POEMS syndrome. 1588 63

A 39-year-old male presented with pedal edema, pleural effusion, splenomegaly, and generalized lymphadenopathy. Serum protein electrophoresis demonstrated the presence of a monoclonal protein. Histological examination of the spleen following splenectomy showed multifocal vascular proliferation and angiovascular lesions consistent with multicentric Castleman disease. He was treated with steroids and rituximab, but without improvement. The patient was found to have portal venous thrombosis and lower extremity arterial thrombosis. He then received combination chemotherapy with cyclophosphamide and mitoxantrone but developed a severe inflammatory polyneuropathy that left him disabled and wheelchair-bound. A diagnosis of multicentric Castleman disease with POEMS syndrome was made, and he then received high-dose chemotherapy with melphalan followed by autologous peripheral blood stem-cell transplantation. Following transplantation, his nerve conduction studies improved and his serum protein electrophoresis normalized. He is currently ambulatory and does not need wheelchair assistance. Hematopoietic stem-cell transplantation may be a treatment option for patients with multicentric Castleman disease and POEMS syndrome.
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PMID:Successful hematopoietic stem-cell transplantation in multicentric Castleman disease complicated by POEMS syndrome. 1598 Dec 32

A 36-year-old man was admitted because of numbness and muscle weakness in the lower extremities. He had gait disturbance, malaise, and body weight loss. Based on the existence of monoclonal gammopathy, the proliferation of abnormal plasma cells in the bone marrow, the presence of sclerotic bone lesion, polycythemia, mild splenomegaly, and an elevated level of serum vascular endothelial growth factor (VEGF) (14,900 pg/mL; normal, 62-707), he was diagnosed as having peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. Following 2 courses of conventional chemotherapy with doxorubicin and dexamethasone, peripheral blood stem cells were mobilized by high-dose etoposide (500 mg/m(2) x 3 days) and granulocyte colony-stimulating factor. After purging by CD34+ selection using the CliniMACS device, the selected cells (12.4 x 10(6)/kg) were cryopreserved. He was then treated with tandem high-dose chemotherapy (HDC) (melphalan 100 mg/m(2) x 2 days) with autologous stem cell rescue. After the first course of HDC, the serum level of VEGF normalized and the minimal residual disease in the bone marrow was reduced below the detection limit of CDR3 analysis by polymerase chain reaction. The patient has been in remission for more than 20 months. He has gradually recovered from the neurological symptoms and now has no impairments of daily living. Our experience suggests that autologous purged stem cell transplantation should be considered as the treatment of choice for POEMS syndrome.
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PMID:Successful treatment of a patient with POEMS syndrome by tandem high-dose chemotherapy with autologous CD34+ purged stem cell rescue. 1692 43


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