Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe five white patients with peripheral T-cell lymphoma. Four patients were older than 65 years. All cases presented with a short clinical course and advanced stage at the time of diagnosis. Clinical manifestations included asthenia, weight loss, peripheral and abdominal lymphadenopathy. One case showed tonsillar involvement and subcutaneous lymph node enlargement; hepatomegaly was present in four cases, two of them with splenomegaly. Only one case presented peripheral lymphocytosis and antibodies to human T-leukemia virus. Although three cases were classified as diffuse mixed lymphomas and two as poorly differentiated lymphocytic lymphomas, there were some common characteristics: diffuse infiltration by different proportions of small lymphoid cells and large immunoblasts, some of them multinucleated and similar to Reed-Sternberg cells; accumulation of histiocytes, plasmacytosis, eosinophilia, venular proliferation and compartmentalization were also found. Bone marrow infiltration was observed in two patients. Results of monoclonal markers showed four cases to be OKT4+ and the other OKT8+. The morphologic and immunologic characteristics of these patients were typical and similar to those reported from other geographical areas.
 ...
PMID:Peripheral T-cell lymphoma. A clinical, histologic, and immunologic study of five cases. 387 77

Multiple myeloma is usually manifested with multiple destructive bone lesion, plasmacytosis and monoclonal protein. Here in we report a case of IgA (lambda) multiple myeloma with hepatomegaly and anemia but without bone lesions. Differential diagnostic points among lymphoma, amyloidosis, cancer and heavy chain disease are discussed.
 ...
PMID:[A case report of variant form of multiple myeloma]. 697 40

Primary plasma cell leukaemia (P-PCL) is a variant of multiple myeloma (MM) first diagnosed in the leukemic phase, with >2000/mm(3) circulating plasma cells (PCs) and plasmacytosis >20% of the white cell count. We investigated the clinical characteristics, therapy, immunophenotype and prognosis factors of 18 patients. Common features at diagnosis were asthenia (seven patients), renal insufficiency (ten patients), bone pain (seven patients), splenomegaly or hepatomegaly (five patients). Hypercalcemia was present at diagnosis in seven patients and was the most potent poor prognosis factor (P<0.05). Most patients (16 out of 18) were treated with an anthracyclin containing regiment; complete remission was attained in one patient and partial remission in 11 patients while six patients had no response. The median survival time from diagnosis was 7 months (2--12, 95% confidence interval), but response to treatment had favorable predictive value (P<0.05). The PCs were usually positive for mature B-cell markers (PCA-1, CD38). They expressed integrins which may increase their binding to endothelial cells and thus participate in PCL physiopathology by favoring plasmocyte extramedullary spread.
 ...
PMID:Primary plasma cell leukaemia: a report of 18 cases. 1116 24

A 47-year-old man presented with fever of unknown origin, generalized weakness, edema, and renal failure. He had left-sided pleural effusion, generalized lymphadenopathy, multiple nontender cutaneous nodules, hepatomegaly, renal failure, and hypergammaglobulinemia. Axillary lymph node biopsy showed findings consistent with Castleman disease of the hyaline vascular type associated with interfollicular plasmacytosis. A renal biopsy performed in view of proteinuria and acute renal failure showed hypercellular glomeruli with capillary loop thickening and double contours consistent with membranoproliferative glomerulonephritis. Skin nodule biopsy showed a glomeruloid hemangioma characterized by dermal proliferation of capillary loops in a nodular manner resembling a glomerulus. He experienced clinical and biochemical remission with steroids. Discontinuation of steroid therapy was associated with recurrence of renal failure, reappearance of nodules, lymphadenopathy, and appearance of bony lytic lesions. Biopsy of bony lytic lesions showed plasmacytoma. The patient achieved complete remission on treatment with steroids and cyclophosphamide and is free of symptoms at the end of 40 months of follow-up. To our knowledge, this is the first case report of the occurrence of membranoproliferative glomerulonephritis, glomeruloid hemangioma of the skin, and plasmacytoma in a patient with multicentric Castleman disease without human immunodeficiency virus infection.
 ...
PMID:Renal involvement in multicentric Castleman disease with glomeruloid hemangioma of skin and plasmacytoma. 1686 Jan 82

Simultaneous occurrence of T-cell and B-cell neoplasms is rare, and etiologic relationships between these 2 malignancies are poorly understood. We describe the case of a 66-year-old woman who was admitted to the hospital because of fever, hemoptysis, lymphadenopathy, and skin rash. Enlarged lymph nodes in axillary, pectoral, paratracheal, and periportal regions as well as slight hepatomegaly and splenomegaly were confirmed. A peripheral blood smear revealed rouleaux formation and numerous circulating plasma cells, with plasmacytoid lymphocytes. Immunofixation-electrophoresis detected a monoclonal band defined as immunoglobulin (IgG)-lambda light chains with broad-band polyclonal IgA. The patient died from abrupt splenic rupture before diagnostic work-up was finished. Postmortem examination revealed infiltration of atypical lymphoid cells exhibiting high proliferative activity admixed with typical and atypical plasma cells in several organs. Thus, plasma cell leukemia (IgG-lambda) as a rare and aggressive variant of plasma cell myeloma in the present case was associated with aggressive peripheral T-cell lymphoma and polyclonal (IgA) plasmacytosis.
 ...
PMID:Rapid Fatal Acute Peripheral T-Cell Lymphoma Associated With IgG Plasma Cell Leukemia and IgA Hypergammaglobulinemia. 2782 44