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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical characteristics of hepatic tuberculosis in 52 cases diagnosed pathologically were analyzed. Fever, abdominal pain and hepatomegaly were the major clinical manifestations; they were present in 96.2%, 42.3% and 88.5% of the patients respectively. The fever had no consistent pattern and the abdominal pain was usually localized to the right hypochondrium and not related to overwork. Hypergrammaglobulinaemia, elevated alkaline phosphatase level and increased ESR were noted in most of the patients (76.9%, 75.0%, 76.5% respectively). 62.5% of the 52 patients was diagnosed by percutaneous liver biopsy. Since there is no consistent clinical pattern in patients with hepatic tuberculosis, the diagnosis should be considered in patients with unexplained fever associated especially with hepatomegaly or hepatosplenomegaly, elevated alkaline phosphatase level, hypergrammaglobulinaemia and increased ESR, Liver biopsy is the most valuable method to confirm the diagnosis of hepatic tuberculosis.
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PMID:[Clinical characteristics of hepatic tuberculosis]. 760 Aug 75

A 57 year-old-female was incidentally found to have leukocytosis in September 1988. Physical examination revealed anemia and marked hepatosplenomegaly. Her WBC count was 33,400/microliters with 95% mature neutrophils showing toxic granules. Her neutrophil alkaline phosphatase score was 482, and serum VB12 14,600 pg/ml. Serum immunoglobulin concentrations were 582 mg/dl for IgG, 3,628 mg/dl for IgA and 48 mg/dl for IgM. IgA was determined as monoclonal origin of lambda type. Bone marrow aspiration revealed a hypercellular marrow with active granulocytopoiesis and increased plasma cells. Cytogenetic study revealed normal karyotype. The bcr rearrangement was negative for bone marrow cells. An electronmicroscopy demonstrated fibrillar inclusions in granulocytes. We diagnosed this case as a chronic neutrophilic leukemia (CNL) associated with multiple myeloma. She was treated with a course of low dose busulfan without beneficial response. She was admitted for development of huge subcutaneous hematoma of left waist in October 1990. Laboratory findings were: Hb 7.0 g/dl, WBC 55, 300/microliters, Platelets 3.3 x 10(4)/microliters, and IgA 6,607 mg/dl. She required frequent transfusions. She died of pneumonia in July 1991. The peculiar fibrillar inclusions with CNL has not been reported so far. The origin and significance of such structure remains uncertain.
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PMID:[Association of chronic neutrophilic leukemia and myeloma with fibrillar inclusions in granulocytes]. 771 83

The correlations between select clinical and laboratory data recorded at the time of diagnosis in 125 chronic myelogenous leukemia patients (60 men and 65 women) and survival time these patient was analysed. All patients were treated with similar methods. Characteristics for which there was evidence of associations with shorter survival outcome were older age, anemia, high percentage of peripheral and marrow blasts and thrombocytopenia or thrombocytosis. There was no evidence of statistically significant prognostic value such parameters as: sex, presence of symptoms, hepatosplenomegaly, high leukocytosis (WBC), high proportion of circulating promyelocytes, neutrophils and eosinophils and serum LDH or leukocyte alkaline phosphatase activity. Peripheral basophilia appeared to have advantage prognostic relevance to survival time. The median survival time observed patients was 38-65 months.
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PMID:[Prognostic significance of some clinical and laboratory parameters in patients with chronic myelogenous leukemia]. 778 14

Congenital erythropoietic porphyria (CEP) is a rare disorder of heme biosynthesis. Skeletal abnormalities have been described in patients with this disease. We report a 25-year-old woman with osteodystrophy from CEP. On examination, mild hepatosplenomegaly, multiple hyperpigmented scars, hypertrichosis, erythrodontia and red coloration of urine were found. Biochemical studies showed increased serum levels of alkaline phosphatase, fasting and total 24-h urinary calcium excretion. Serum 250H vitamin-D concentration was low due to avoidance of sun exposure. Skeletal radiographs disclosed marked vertical and horizontal trabecular pattern and biconcavity of most of the dorsal and lumbar vertebral bodies. Several round sclerotic lesions (1-3 cm in diameter) were seen in the skull, pelvis and one lumbar vertebrae. The sclerotic lesions were augmented in size and number compared to X-rays obtained 8 years before. Bone mineral density (evaluated by DEXA) was markedly reduced at the spine and moderately diminished at the proximal femur and total skeleton. Treatment for 11 months with pamidronate (and the addition of hydrochlorotiazide for the last 6 months) reduced to normal values the serum levels of alkaline phosphatase and fasting urinary calcium. The 24-h urinary excretion of calcium and hydroxyproline were also decreased. The BMD increased in all the skeletal areas with presumably hyperactive bone marrow: spine, head, ribs and pelvis (and total skeleton), but did not change at the extremities and diminished at the femoral neck. Patients with CEP may present osteodystrophy characterized by sclerotic lesions and osteopenia, most likely due to accelerated bone turnover in areas of active bone marrow.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Congenital erythropoietic porphyria: skeletal manifestations and effect of pamidronate treatment. 802 43

Five weeks after a four-day visit to Malta, a 39-year old white male German national developed septic temperatures of up to 40 degrees C, progressive jaundice and a pronounced hepatosplenomegaly. The initial examination showed a very sick, somnolent patient with jaundice, cyanosis, tachypnea and a markedly enlarged liver on both physical examination and sonography. The laboratory evaluation revealed a moderate leukocytosis, markedly accelerated ESR, poor liver function with strongly elevated gamma-GT and alkaline phosphatase levels. Primary antibiotic therapy consisted of doxycycline. Ultrasound examination of the liver four days after admission revealed multiple hypodense abscesses. On the sixth day after admission, gram-negative rods were first isolated from blood cultures; antibiotic therapy was switched to ofloxacin (2 x 400 mg/day) and amoxycillin (3 x 2 g/day) after sensitivity testing. As a result of treatment with this combination of antibiotics, the patient was free of fever 10 days after hospitalization; on the same day yersinia enterocolitica was isolated from the first blood cultures taken on admission. The diagnosis of non-enteric forms of yersinia infection can prove very difficult, especially if the serology is not clear cut and there are no immunological complications. A presentation including intermittent fever, moderate leukocytosis, strongly accelerated ESR and multiple hypodense abscesses in the liver should lead one to consider a non-enteric type of yersinia infection. Hepatic abscesses usually occur in patients who have an iron overload.
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PMID:[Yersinia enterocolitica infection with extraintestinal manifestations: case report and overview]. 819 10

A 30-year-old man with chronic neutrophilic leukemia (CNL) in association with monoclonal gammopathy is presented. Physical examination on admission revealed moderate hepatosplenomegaly. Initial blood count showed neutrophilic leukocytosis (42.2 x 10(9)/1 with 90% mature neutrophils). Leukocyte alkaline phosphatase (LAP) score was elevated. Bone marrow aspiration showed myeloid hyperplasia without dysplastic features. Karyotypic and molecular analyses of bone marrow cells showed the absence of Philadelphia (Ph1) chromosome and bcr gene rearrangement. Because there was no underlying infection or neoplasm, he was diagnosed as having CNL associated with IgG kappa-type monoclonal gammopathy (IgG, 1,269 mg/dl). In addition to its association with monoclonal gammopathy of undetermined significance (MGUS), the present case was also characterized by spontaneous remission of CNL during the 12-year follow-up, accompanied by a gradual increase in serum IgG levels up to 3,000 mg/dl. As far as we know, there have been 19 cases of CNL associated with monoclonal gammopathy in the literature. The median survival of these cases was 5 years. Although there have been only 6 cases of CNL associated with MGUS, survival of these cases was particularly favorable. Taken together with the observation that leukocytosis and hepatosplenomegaly in the present case subsided without specific treatment, we speculate that myeloid proliferation in the present case may have been a leukemoid reaction to underlying monoclonal gammopathy.
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PMID:Chronic neutrophilic leukemia associated with monoclonal gammopathy of undetermined significance. 863 44

Chronic neutrophilic leukemia (CNL) is a rare hematologic disorder usually presenting with a persistent neutrophilia in the leukemoid range (WBC > 40-50 x 10(9)/1) and consisting largely of mature neutrophils. Patients have no obvious cause for an elevated white count and typically have an elevated leukocyte alkaline phosphatase score, hepatosplenomegaly, elevated vitamin B12 and are Philadelphia chromosome-negative. CNL has occasionally been associated with paraproteinemia or outright myeloma. Dysplastic features within the neutrophils in CNL have rarely been reported. We report the clinical, pathological and cytogenetic features of a case of CNL in an elderly white female initially diagnosed with refractory anemia with excess blasts, which subsequently progressed to CNL.
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PMID:Chronic neutrophilic leukemia evolving from a myelodysplastic syndrome. 935 49

A 62-year-old man was admitted to our hospital because of severe jaundice and fever. Physical examination demonstrated hepatosplenomegaly. The laboratory data revealed elevated serum bilirubin, alkaline phosphatase, lactate dehydrogenase, aspartate aminotransferase and alanine aminotransferase, and the reduced hepaplastin test (Normotest). Computed tomography showed hepatosplenomegaly and swelling of the paraaortic lymph nodes. Although he was treated with antibiotics and steroids, he died of hepatic failure 22 days after admission. At autopsy, his liver weighed 1910 grams, and a histological examination of the liver revealed marked infiltration of CD30 (Ki-1) positive lymphoma cells. He was diagnosed as having non-Hodgkin lymphoma, large cell anaplastic type, Ki-1 lymphoma. We herein report our findings of this very rare case of Ki-1 lymphoma associated with hepatic failure.
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PMID:An autopsy case of Ki-1 lymphoma associated with hepatic failure. 944 89

A 52-year-old male presented with a prolonged fever, anemia, weight loss, hepatosplenomegaly, and elevated serum level of alkaline phosphatase. The patient developed septicemia, metabolic acidosis, and died 3 days after admission. Liver necropsy, tissue showed group C, peripheral T-cell proliferative disease. Epstein-Barr virus genomes were demonstrated in the nuclei of these abnormal T-cell lymphocytes. Granulomatous reaction was observed in the liver and bone marrow. Stains and cultures for mycobacteria and fungi gave negative results. Granulomatous reaction is believed to be induced by cytokines which were released from the T-cell lymphocytes that infiltrated in the liver and bone marrow.
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PMID:Granulomatous reaction in peripheral T-cell proliferative disease: a case report. 947 Mar 34

A previously well 70 year old woman was admitted to hospital following a three day history of vomiting and confusion. Her serum calcium was 6.58 mmol/l, phosphate 1.09 mmol/l, and alkaline phosphatase 91 iu/l. The mechanism of this hypercalcaemia was not obvious as there was no evidence of a primary malignancy, lymphadenopathy or hepatosplenomegaly. The calculation of indices of urinary excretion of calcium and phosphate suggested the presence of excessive parathyroid hormone (PTH) activity as the mechanism of hypercalcaemia. Plasma intact PTH, 25-hydroxycholecalciferol, and 1,25-dihydroxycholecalciferol were not raised suggesting the presence of PTH related peptide (rP). This led to a systematic search for a malignancy, which revealed the presence of a high grade B cell non-Hodgkin's lymphoma confined to the bone marrow. Plasma PTH-rP was subsequently shown to be raised confirming the interpretation of the initial urinary and calcium excretion indices. This case highlights the value of standard laboratory measurements such as urinary calcium and phosphate excretion in cases of hypercalcaemia of obscure aetiology, which can complement measurements of PTH and other calcitropic hormones.
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PMID:Value of assessing parathyroid hormone-like activity in a case of extreme hypercalcaemia. 965 76


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