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)

Massive bone marrow necrosis was seen in a 42-year-old male with acute leukemia. In December, 1988, on admission, laboratory data revealed pancytopenia and a high level of serum LDH and ALKP. Bone marrow aspiration resulted in dry-tap and showed bone marrow necrosis in the bone marrow biopsy specimen. A bone marrow scintigraphy with 111In faintly visualized the bone marrow but visualized area was expanded in the extremities compared with normal subjects. The second bone marrow biopsy showed proliferation of blasts. In the middle of March, blasts began to appear in peripheral blood. The blasts were cytochemically negative for POX, Es, PAS, AcP, TdT and had surface markers CD3-, CD19-, CD33-, CD13-, LCA-, HLA-DR-. Even by investigation on rearrangement of the immunoglobulin heavy chain region, an origin of the blasts could not be determined. In April, the number of blasts in peripheral blood increased and hepatosplenomegaly developed rapidly. Therefore, he was put on the chemotherapy with vincristine and prednisolone, but he died of cerebral hemorrhage. The autopsy revealed widespread bone marrow necrosis. It has rarely been reported that massive bone marrow necrosis is found prior to the occurrence of acute unclassified leukemia.
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PMID:[Acute unclassified leukemia with bone marrow necrosis]. 202 Jan 20

A 37-year-old male was admitted to hospital because of a right hypochondrial pain and icterus. His physical examination showed hepatosplenomegaly, and the laboratory findings demonstrated abnormal hepatic and pancreatic functions. A CT examination revealed a large mass of a low density in the pancreas head and tail. Further, an immunological study revealed that the patients serum Ca 19-9 level was elevated, but that the CEA and AFP levels were normal. Both the pathological and cytological examinations, however, did not indicate a malignancy of the pancreas. The patient subsequently developed uremia, a hemorrhagic tendency, and then died. An autopsy confirmed a pancreatic tumor which occupied the head of pancreas. Histologically, the tumor contained round cells with scanty cytoplasms and showed a sarcomatous pattern. An immunohistochemical study showed that the LCA, MB-1, and the LN-1 for B cell markers were positive, while the MT-1 for T cells was negative. The case illustrates a malignant lymphoma of the pancreas which demonstrated a serum Ca 19-9 elevation.
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PMID:[An autopsied case of malignant lymphoma of the pancreas with an elevation of the serum Ca 19-9 level]. 269 18

A 45-year-old woman was admitted with complaints of non-productive cough, chest pain, fatigue and weight loss in the last 4 months. On physical examination moderate hepatosplenomegaly and crackles most notably on the basal region of the right lung were evident. Serial chest X-rays and computed tomographies revealed a migratory nodular infiltration pattern, changing in location and size in both the lungs. The histopathological diagnosis of the open lung biopsy was lymphomatoid granulomatosis (LG) with a marked angioinvasive lymphocytic perivascular and peribronchial infiltration pattern. In the immunohistochemical analyses LCA, CD-79, CD-20 were positive, while CD-30 was negative. No response could have been achieved under combination chemotherapy and the patient died from progressive disease. LG is a rare disease and a difficult diagnosis in the routine clinical practice. This report emphasises that, LG should be considered especially when there are migratory nodules of varying sizes in lungs.
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PMID:Migratory nodules in the lung: lymphomatoid granulomatosis. 1269 Nov 64

Granulocytic sarcomas are extramedullary tumors (EMD) of malignant myeloid precursor cells. EMD or granulocytic sarcoma of ovary is rare disease. A 15-year-old girl had complaints of abdominal pain and weight loss for 3 months. On physical examination, there were hepatosplenomegaly and a painless mass under the umbilicus. Breast development was grade II. There was no clitoris hypertrophy. Her labia majora were separate and vagina hypoplastic. Hemoglobin level was 9.3 g/dl, white blood cells count 2.8 x 10(6)/1, platelet count 31.6 x 10(9)/1. There were dysplastic features in the blood and bone marrow cells. There were 10 and 22% blasts in the peripheral blood smear and bone marrow, respectively. The levels of serum follicle stimulating and luteinizing hormones were high. An inguinal mass (diameter 9.5 x 7.6) cm was detected on computed tomography. The histopathological diagnosis of this was obtained from laporascopy was composed of ovotestis and there was marked blastic infiltration in this ovotestis which had myeloid markers on flow cytometry. In the immunohistochemical analyses of ovotestis and bone marrow, blasts were positive for LCA, CD-13, CD-33 and CD 68. The cytogenetic analysis of the bone marrow shaved 46 XY karyotype. No response was achieved with combination chemotherapy and the patient died from progressive leukemia. Here we report a rare patient with myelodysplastic syndrome, EMD and hermaphroditism. To our knowledge this is the first case of MDS, EMD and hermaphroditism.
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PMID:Granulocytic sarcoma of the ovotestis: an association of myelodysplastic syndrome and hermaphroditism. 1536 14

Subcutaneous panniculitic T-cell lymphoma (SPTL) is a rare variant of cutaneous T-cell lymphoma where lymphoma cells infiltrate preferentially into subcutaneous tissue. Five cases of SPTL were seen during the period from 2001-2004 at the Department of Dermatology, Hospital Kuala Lumpur. All five presented with multiple subcutaneous nodules on the face, trunk and limbs of one week to six months duration with associated fever and loss of weight. Physical examination showed multiple tender, erythematous indurated plaques and subcutaneous nodules on their face, trunk and limbs. One patient also presented with unhealing ulcerated nodules. Two patients had hepatosplenomegaly and one hepatomegaly. Two patients had pancytopaenia while the other three had leucopaenia. One patient had deranged liver function. Out of the five patients, three had bone marrow examination with haemophaegocytosis in two and one hypocellular marrow. Skin biopsy of all patients showed infiltration with atypical lymphoid cells in the upper dermis and subcutaneous fat. These neoplastic cells showed positivity for CD3 and CD30 in three patients with CD8, TIA-1 and LCA (Leucocyte common antigen) being positive in one patient. One patient treated with prednisolone and subcutaneous Roferon 3Mu three times a week since 2001 was in remission. Two patients who were planned for chemotherapy had deteriorated rapidly and succumbed to septicaemia from pancytopaenia. Subcutaneous panniculitic T-cell lymphoma has been reported to show two distinct clinical presentations. The first is characterized by an indolent course with good prognosis and the second with rapid clinical deterioration, haemophaegocytosis and death. Both presentations were seen in our five patients seem to demonstrate these two subtypes of SPTL.
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PMID:Subcutaneous panniculitic T-cell lymphoma-review of five cases. 1762 60

Acute myeloid leukemia (AML) is a morphologically diverse group of hematopoietic malignancies characterized by proliferation of immature cells that arise in the myeloid progenitor cells of the bone marrow. It shows cutaneous lesions relatively rarely. The most common cutaneous manifestation is the appearance of one or several tumors. An association of AML with skin involvement and trisomy 8 has rarely been reported. We present the case of a 74-year-old woman that presented with fatigue, nausea, dyspnea, and night sweats. On physical examination we found no hepatosplenomegaly, peripheral lymphadenopathy, or skin abnormalities. Hematological examination revealed Hb: 8.4 g/dl, PLT: 35,000/ml, WBC 105,000/ml, and blasts 51%. Bone marrow aspiration showed blasts 88%. Cytogenetic findings in the marrow showed trisomy 8. The patient received 3 courses of systemic chemotherapy with aracytin and idarubicin and then, while she was in remission, multiple red nodules developed on the upper and lower limbs. A skin nodule from the right arm was excised and histology showed a diffuse infiltration of the dermis consisting of large cells with round to oval nuclei and little basophilic cytoplasm. Immunohistochemistry was performed and the neoplastic cells showed strong positivity for MPO but were negative for LCA. Accordingly, a diagnosis of AML involving the skin was made. The patient received another course of systemic chemotherapy with aracytin and idarubicin and is in good condition.
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PMID:Skin relapse of acute myeloid leukemia associated with trisomy 8. 1799 63