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)

Differentiating between primary and ectopic or pseudohyperparathyroidism may be difficllt, but certain aspects of the patient's clinical history as well as laboratory tests may be helpful. We present an unusual case report of a patient who had massive splenomegaly secondary to a localized histiocytic lymphoma. On the basis of a lower serum parathormone level for a given serum calcium increase combined with normal serum phosphorous and chloride values, the diagnosis of psuedohyperparathyroidism was made and was confirmed when the patient's serum calcium level and temperature returned to normal following splenectomy.
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PMID:Isolated histiocytic lymphoma of the spleen causing fever and hypercalcemia. 33 15

Malignant histiocytosis is an uncommon, progressive disease with a poor prognosis which is characterized by malaise, fever, lymphadenopathy, and splenomegaly. Lymphadenopathy is commonly present at presentation as well as during the course of the disease. The lymphographic findings in this case report were very similar to those encountered in malignant lymphoma, a not unexpected situation since the clinical and pathological resemblances to histiocytic lymphoma are striking.
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PMID:Lymphography in malignant histiocytosis: case report. 78 10

Ten patients with diffuse large cell (histiocytic) lymphoma of the spleen had a characteristic clinical presentation and pathologic findings. Patients presented with left upper quadrant pain, fever, weight loss, and an elevated sedimentation rate. Imaging studies revealed an enlarged spleen with a discrete mass in all cases. Moderate to massive splenomegaly (average weight, 1025 g) was found at laparotomy; a single large mass or multiple confluent nodules with extensive central necrosis replaced 85% to 90% of the parenchyma. The tumor transgressed the splenic capsule in nine of ten cases, and either invaded or was adherent to the diaphragm, stomach, pancreas, or abdominal wall. Lymph nodes in the splenic hilum or retroperitoneum were frequently involved. Seven patients were in Ann Arbor Stage II, and three were in Stage I. Eight of the ten lymphomas were subclassified as centroblastic (large noncleaved cell) and two were immunoblastic. The B-cell lineage of six tumors was established by the presence of monoclonal immunoglobulin. Despite combination chemotherapy, with or without radiation, three of the seven patients whose follow-up was adequate died in less than 2 years; four are alive at 7, 12, 12, and 81 months, respectively, the last two with recurrent lymphoma. Large cell lymphoma of the spleen is a likely diagnosis in patients who present with left upper quadrant pain, fever, and radiographic evidence of a splenic mass.
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PMID:Diffuse large cell (histiocytic) lymphoma of the spleen. Clinical and pathologic characteristics of ten cases. 638 5

Expression of the natural killer (NK) cell antigen CD56 is uncommon among lymphomas, and those that do are almost exclusively of non-B-cell lineage and show a predilection for the nasal and nasopharyngeal region. This study analyzes 49 cases of nonnasal CD56+ lymphomas, the largest series to date, to characterize the clinicopathologic spectrum of these rare neoplasms. All patients were Chinese. Four categories could be delineated. (1) Nasal-type NK/T cell lymphoma (n = 34) patients were adults 21 to 76 years of age (median, 50 years), including 25 men and 9 women. They presented with extranodal disease, usually in multiple sites. The commonest sites of involvement were skin, upper aerodigestive tract, testis, soft tissue, gastrointestinal tract, and spleen. Only 7 cases (21%) apparently had stage I disease. The neoplastic cells were often pleomorphic, with irregular nuclei and granular chromatin, and angiocentric growth was common. The characteristic immunophenotype was CD2+ CD3/Leu4- CD3epsilon+ CD56+, and 32 cases (94%) harbored Epstein-Barr virus (EBV). Follow-up information was available in 29 cases: 24 died at a median of 3.5 months; 3 were alive with relapse at 5 months to 2.5 years; and 2 were alive and well at 3 and 5 years, respectively. (2) Aggressive NK cell leukemia/lymphoma (n = 5) patients presented with hepatomegaly and blood/marrow involvement, sometimes accompanied by splenomegaly or lymphadenopathy. The neoplastic cells often had round nuclei and azurophilic granules in the pale cytoplasm. All cases exhibited an immunophenotype of CD2+ CD3/Leu4- CD56+ CD16- CD57- and all were EBV+. All of these patients died within 6 weeks. (3) In blastoid NK cell lymphoma (n = 2), the lymphoma cells resembled those of lymphoblastic or myeloid leukemia. One case studied for CD2 was negative and both cases were EBV-. One patient was alive with disease at 10 months and one was a recent case. (4) Other specific lymphoma types with CD56 expression (n = 8) included one case each of hepatosplenic gammadelta T-cell lymphoma and S100 protein+ T-cell lymphoproliferative disease and two cases each of T-chronic lymphocytic/prolymphocytic leukemia, lymphoblastic lymphoma, and true histiocytic lymphoma. All of these cases were EBV-. Six patients died at a median of 6.5 months. Nonnasal CD56+ lymphomas are heterogeneous, but all pursue a highly aggressive clinical course. The nasal-type NK/T-cell lymphoma and aggressive NK cell leukemia/lymphoma show distinctive clinicopathologic features and a very strong association with EBV. Blastoid NK cell lymphoma appears to be a different entity and shows no association with EBV.
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PMID:Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. 919 74