Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Here we report an unusual case of T-cell lymphoma presenting as ascites. A 49-yr-old woman was admitted to the hospital for abdominal discomfort associated with increasing abdominal girth over the course of 3 mo. She also complained of nausea, vomiting, and diarrhea. On physical examination, a tense and distended abdomen and edema of the lower extremities were noted. Neither hepatosplenomegaly nor lymphadenopathy was found. A CT scan of the abdomen and pelvis showed a large abdominal/pelvic mass surrounding the small bowel and omentum and small nodes in the para-aortic and mesenteric regions. The cytospin prepared from the peritoneal fluid was hypercellular and composed of a population of monotonous, noncohesive cells with a high nuclear/cytoplasmic ratio and a single prominent central nucleolus. The cells were positive for leukocyte common antigen and Leu-22 (CD43) but negative for keratin, L26, UCHL-1, kappa, lambda, CD3, Ki-1 (CD30), S-100, and carcinoembryonic antigen. Morphologic and immunologic findings were suggestive of T-cell immunoblastic lymphoma. Peripheral T-cell lymphomas rarely present as ascites; this case demonstrates the value of effusion cytology in making this diagnosis.
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PMID:Peripheral T-cell lymphoma presenting as ascites: a case report and review of the literature. 1035 13

We report an unusual case of T-cell lymphoma presenting as ascites. A 72-year-old HIV-negative woman was admitted to our hospital for abdominal discomfort associated with increasing abdominal girth over the course of 1 month. Physical examination showed a tense and distended abdomen and edema of the lower extremities. There was no hepatosplenomegaly or lymphadenopathy. A computed tomographic scan of the abdomen and chest showed massive ascites and pleural effusions, but there was no evidence of tumor masses or lymph node enlargement. The cytospin prepared from the peritoneal fluid was hypercellular and composed of a population of monotonous, large cells containing fine chromatin. No herpesvirus-8 (HHV-8) DNA was detected by polymerase chain reaction in the cells. Immunohistochemistry showed the neoplastic cells to be CD3+, CD4, CD7+. CD8-, CD34-, CD56, and TCR-alphabeta+. Repeated cytogenetic studies showed common abnormalities of del(1) (p11p22), +i(7)(ql0), and t(11:14)(q23;q11). The morphologic and immunologic findings were suggestive of peripheral T-cell lymphoma (PTCL), unspecified. This case suggests that some PTCLs with clonal chromosomal aberrations can exhibit peculiar serosal spreading in the absence of HHV-8 infection.
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PMID:CD3+CD4-CD8-TCR-alphabeta+ T-cell lymphoma with clinical features of primary effusion lymphoma: an autopsy case. 1179 1

Wolman disease (WD) is a rare, inherited, rapidly fatal condition presenting in early infancy. The disease manifests in the first month of life with failure to thrive, vomiting, diarrhea, abdominal distension, hepatosplenomegaly and bilateral adrenal calcification and is nearly always fatal before the age of 1 year. Barring a case report of isolated fetal ascites, there is no report of intractable ascites as the presentation of WD till date. We report two siblings with WD who both had intractable ascites and required therapeutic paracentesis, albumin infusion, and diuretics to control tense ascites. Although rare, WD should be considered in the differential diagnosis of infantile ascites.
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PMID:Intractable ascites as a manifestation of Wolman's disease: report of two sibs. 2300 84