Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Interleukin-6, IL-6, is a pleiotropic cytokine which plays a central role in defense mechanisms, including the immune response, acute phase reaction and hematopoiesis. Abnormal expression of the IL-6 gene has been suggested to be involved in the pathogenesis of a variety of diseases, especially rheumatoid arthritis, Castleman's disease, mesangial proliferative glomerulonephritis, multiple myeloma and Kaposi's sarcoma. In the case of multiple myeloma and Kaposi's sarcoma, the existence of an IL-6-IL-6 receptor autocrine loop has been implicated in the oncogenesis process. On the other hand, IL-6 has a potent anti-tumor activity against certain types of tumors. This anti-tumor effect is mediated by in vivo induction of tumor specific cytotoxic T cells and in part by a growth inhibitory activity of IL-6.
 ...
PMID:The evidence for interleukin-6 as an autocrine growth factor in malignancy. 164 91

Peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes are often the presenting features of the POEMS syndrome. Approximately 50% of these are associated with osteosclerotic myeloma, a rare variant of multiple myeloma and some with Castleman's disease, an unusual lympho-proliferative disorder. The underlying pathogenetic mechanisms have not been elucidated but amelioration of symptoms with the disappearance of the M-component suggests possible etiopathogenic mechanisms. We present a 40-year-old woman with manifestations of all three disorders.
 ...
PMID:POEMS syndrome, osteosclerotic myeloma and Castleman's disease: a case report. 195 37

A case of POEMS Syndrome of six years of evolution is reported. This syndrome is characterized by Raynaud phenomenon, polyneuropathy, edema, anasarca, papilledema, osteosclerosis and lymphadenopathy with the histopathology of Castleman's disease, hypothyroidism, hypogonadism, cutaneous sclerosis, hyperpigmentation, axillary alopecia and the presence of urinary lambda light chains. A bone marrow biopsy did not show plasmocytic infiltration and there was no evidence of extramedullary plasmocytoma. Methylprednisone was given at the dose of 1 mg/kg/day and subjective and objective improvement was observed. The edema and anasarca disappeared as well as the lymphadenopathies; muscle strength improved and the patient was able to walk without aid. Papilledema persisted. The pathogenesis of this syndrome remains unknown; some of the symptoms have been attributed to paraprotein deposits in peripheral nerves, high capillary permeability due to vascular alterations, accelerated conversion of androgen to estrogen, or to the production by plasma cells of a toxic substance. Mortality is related to complications of the polyneuropathy. Some patients in whom POEMS syndrome was associated, or not, with myeloma were treated with chemotherapy and/or radiotherapy with different responses; in others, corticosteroids were of short lived benefit. Our patients remains well after 42 months treatment with 20 mg methylprednisone every other day.
 ...
PMID:[POEMS syndrome: report of a case and review of the literature]. 210 48

A patient with chronic, bilateral optic disc edema developed multiple systemic manifestations of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes). A serum immunoelectrophoresis showed an abnormal serum IgG lambda protein, and urine immunoelectrophoresis revealed a monoclonal lambda protein plus an IgG lambda fragment. Bone survey and MRI scan revealed a sclerotic lesion of the first lumbar vertebra, and lymph node biopsy showed changes consistent with Castleman's disease. Thus, his optic disc edema was the presenting feature of the POEMS syndrome and osteosclerotic myeloma.
 ...
PMID:When do optic disc edema and peripheral neuropathy constitute poetry? 217 27

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.
 ...
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).
 ...
PMID:Castleman's disease and related disorders. 246 87

Interleukin 6, an essential factor for B cells to differentiate to immunoglobulin-producing cells has been demonstrated to be involved in abnormal polyclonal plasmacytosis in cardiac myxoma. Castleman's disease, rheumatoid arthritis and AIDS. Furthermore, IL-6 was found to be a potent growth factor for both plasmacytoma and myeloma. The involvement of IL-6 in plasma cell abnormalities was further demonstrated by the generation of a lethal plasma cell growth in IL-6 transgenic mice. Continuous IL-6 gene expression could be considered to play an essential role in both polyclonal plasmacytosis and the generation of plasma cell neoplasias.
 ...
PMID:Interleukin 6 and plasma cell neoplasias. 249 Dec 60

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.
 ...
PMID:[Crow-Fukase syndrome]. 269 95

A rare case of a 43-year-old man with polyneuropathy, monoclonal gammopathy, myeloma and Castleman's disease of plasma cell type was diagnosed. Immunohistological examination of the lymph node with Castleman's disease showed polyclonal binding while the myeloma was monoclonal containing IgA with lambda-light chains. At the immunoelectrophoresis IgA/IgA paraprotein was demonstrated. The possible pathogenetical relationship between polyneuropathy and plasmacytoma as well as Castleman's disease will be discussed.
 ...
PMID:[Angiofollicular lymphatic hyperplasia with plasmacytoma and polyneuropathy: a case report with immunohistochemical study]. 321 Jun 55

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.
 ...
PMID:[Crow-Fukase syndrome (POEMS syndrome) and osseous mastocytosis secondary to Castleman's angiofollicular lymphoid hyperplasia]. 382 4


1 2 3 4 5 6 Next >>