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 62-year-old man was admitted to our hospital with exertional dyspnea. On admission, neither hepatosplenomegaly nor lymphadenopathy were noted. Laboratory data revealed anemia (Hb, 4.8 g/dl), leukopenia (2,800 microliters) and a normal platelet count (21 X 10(4)/microliters). The immature blast cells in the peripheral blood were 15%, which increased to 32% during his clinical course. On cytochemical studies, the blast cells had no staining with peroxidase, alpha-naphthyl-butyrate esterase and PAS, although acid phosphatase was positive. More than 58% of the blasts were identified as being of megakaryocytic lineage by platelet peroxidase and by tests with monoclonal GP IIb/IIIa antibody. Bone marrow biopsy disclosed marked fibrosis. However, the patient constantly had normal counts of platelets ranging from 21 X 10(4) to 63 X 10(4)/microliters. This case provides evidence that the megakaryocytic leukemias can be categorized into two types, which are characterized by either undifferentiated or differentiated megakaryocytic leukemia cells.
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PMID:[Megakaryocytic leukemia with thrombocytosis]. 281 Jul 95

Twenty-one patients with the diagnosis of mucopolysaccharidosis or mucolipidosis and a history of respiratory complaints or thorough respiratory evaluation were studied retrospectively. Anatomic factors affecting respiratory status included: (i) upper airway narrowing by hypertrophied tongue, tonsils, adenoids, and mucous membranes; (ii) lower airway narrowing by glycosaminoglycan deposition within the tracheobronchial mucosa; (iii) decreased thoracic dimensions due to scoliosis and thoracic hyperkyphosis; and (iv) decreased abdominal dimensions due to lumbar hyperlordosis, gibbus formation and hepatosplenomegaly. Cardiac and neurologic involvement, while present, did not play primary roles in the development of respiratory disease. The functional consequences of these findings included increased risk of developing: (i) respiratory tract infections; (ii) airway compromise during or after anesthesia or sedation; (iii) dyspnea on exertion; (iv) obstructive lung disease; (v) obstructive sleep apnea; and (vi) cor pulmonale. A management approach is presented which can reduce the morbidity and mortality experienced by these patients.
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PMID:Respiratory complications of mucopolysaccharide storage disorders. 313 89

A 80-year-old man was admitted to our hospital complaining of appetite loss, dry cough, and dyspnea on exertion. Chest X-ray film showed massive right pleural effusion. On physical examination, the right thorax was dull to percussion, and breath sounds of the right lung field were diminished. Examination of the cardiovascular system was unremarkable. Hepatosplenomegaly and ascites were not evident. The character of the pleural fluid was transudative. Cytological findings were negative and culture of the pleural fluid was normal. First, the pleural fluid was diminished by thoracic drainage. But, after the cessation of drainage, the pleural fluid soon reaccumulated. Abdominal ultrasonography showed typical liver cirrhosis pattern without ascites. An intraperitoneal injection of 99mTc-sulfur colloid was followed by positive uptake of radioactivity in the right pleural cavity from 1 hour after the injection, demonstrating the existence of a direct perito-pleural communication. Thus, the diagnosis of liver cirrhosis presenting with right pleural fluid, without ascites was made. This case indicates that the absence of ascites cannot exclude cirrhosis as the etiologic cause of pleural effusion.
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PMID:[A case of liver cirrhosis presenting with right pleural fluid without ascites]. 780 61

We report a 59-year-old lady who presented with exertional dyspnea and was diagnosed to have sarcoidosis. She responded to steroids, but one year later developed abdominal symptoms and was found to have hepatosplenomegaly. Liver biopsy showed non caseating granulomas. As she had developed steroid-induced diabetes she was started on chloroquine and responded well with regression of the liver and spleen during one year of treatment.
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PMID:Hepatic sarcoidosis responding to chloroquine as steroid-sparing drug. 1053 25

Chronic eosinophilic leukemia is a rare entity, characterized by eosinophilia of >1.5 x 10(9)/L, persisting for >6 months after exclusion of other reactive and neoplastic causes of eosinophilia, and occurring in association with a clonal cytogenetic abnormality. Various chromosomal abnormalities have been associated with chronic eosinophilic leukemia. Partial deletion of the long arm of chromosome 16 is a cytogenetic abnormality first reported 20 years ago in patients with acute myeloid leukemia associated with bone marrow eosinophilia (AML-M4Eo). We report a case of a 45-year-old African-American male with hepatitis C and sustained peripheral blood eosinophilia who presented with gross hematuria, dyspnea on exertion, chills, decreased appetite and weight loss of 40 pounds associated with hepatosplenomegaly and lymphadenopathy. Bone marrow biopsy showed clonal cytogenetic abnormality consisting of deletion of the long arm of chromosome 16 (16q22). Philadelphia chromosome t (9;22) and polymerase chain reaction (PCR) analysis for C-kit and platelet-derived growth factor receptor-alpha (PDGFRA) mutations were negative. The patient was treated with imatinib at 400 mg/d with improvement of symptoms, reduction of lymphadenopathy and normalization of the eosinophil count. To our knowledge, this is the first case report of isolated del (16) (q22), a cytogenetic abnormality associated with AML-M4Eo, occurring in chronic eosinophilic leukemia. Whether this cytogenetic abnormality might represent a prodromal phase of AML-M4Eo is not known. In addition, the role of hepatitis C in inducing clonal proliferation of eosinophils is unclear.
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PMID:Case of chronic eosinophilic leukemia with deletion of chromosome 16 and hepatitis C. 1691 38

A 76-year-old woman was admitted to our hospital because of exertional dyspnea and leg edema during the previous month. Her systolic blood pressure on admission was 80 mmHg with 12 mmHg of pulsus paradoxous, and her pulse rate was 110 beats/min. Chest radiography revealed marked cardiomegaly and echocardiography showed massive pericardial effusion mainly behind the left ventricle and collapse of the right ventricle. The initial diagnosis was pericardial tamponade. Pericardiocentesis and pericardial drainage revealed bloody pericardial effusion. After drainage, her vital signs improved and her symptoms immediately disappeared. The cytological analysis of the pericardial effusion revealed numerous lymphoma cells. Computed tomography of the neck, chest and abdomen showed no evidence of tumor masses, lymph node enlargement, or hepatosplenomegaly. Infectious disease, collagen disease and aortic dissection were excluded. The final diagnosis was primary effusion lymphoma. The prognosis of primary effusion lymphoma is generally unfavorable because it is frequently accompanied by immunodeficiency disease. However, there was no human immunodeficiency virus infection in this patient. Fortunately, the effect of chemotherapy was excellent and the patient is doing well 1 year after the diagnosis.
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PMID:[Primary effusion lymphoma complicating cardiac tamponade: a case report]. 1746 Aug 82

A 74-year-old woman presented with dyspnea on exertion and nocturnal cough. Chest computed tomography (CT) revealed scattered bilateral ground-glass opacities without a zonal dominance. Bronchoalveolar lavage elicited increased lymphocytes, but transbronchial lung biopsies were not performed because of hypoxemia during the examination. She received steroid therapy because of her subsequent worsening respiratory condition, but her condition continued to deteriorate. The ground-glass opacities partially consolidated with the appearance of new ground-glass opacities and a nodular shadow. Hepatosplenomegaly was observed on CT while soluble interleukin-2 receptor was elevated. A biopsy of a Campbell de Morgan spot of the trunk yielded a diagnosis of intravascular large B-cell lymphoma. There was marked clearing of the pulmonary infiltrates and significant symptomatic improvement in response to systemic chemotherapy.
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PMID:Ground-glass opacities and a solitary nodule on chest in intravascular large B-cell lymphoma. 2639 59

The prognosis of pulmonary arterial hypertension (PAH) has significantly improved over the past two decades due to advances in medications, including pulmonary vasodilators. However, the side effects of these drugs remain problematic in some patients. A 51-year-old woman with chronic hepatitis C was diagnosed with PAH 7 years before presenting to our hospital. She was unable to continue her treatment with pulmonary vasodilators due to various side effects. She had a World Health Organization functional class of IV and was started on continuous infusion of prostaglandin I2 (PGI2). This therapy improved her symptoms, including dyspnea and fatigue. However, she began to complain of abdominal distension after 4 months of PGI2 therapy. Computed tomography showed significant hepatosplenomegaly. Her abdominal distension improved slightly after decreasing PGI2 treatment, but her dyspnea on exertion was exacerbated. She died 12 years after diagnosis of PAH due to uncontrollable heart failure. Here, we describe a rare case of PAH with hepatosplenomegaly after administration of PGI2. <Learning objective: Intravenous continuous prostaglandin (PG) I2 therapy is useful for the treatment of severe pulmonary arterial hypertension (PAH). However, it has numerous side effects that are difficult to control. We report a rare case of PAH with chronic hepatitis C that resulted in hepatosplenomegaly after PGI2 administration. In cases of chronic liver disease, it is important to keep in mind that administration of PGI2 may result in hepatosplenomegaly.>.
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PMID:A case of pulmonary arterial hypertension with chronic hepatitis that resulted in hepatosplenomegaly after administration of prostaglandin I2. 3237 43

Allopurinol is a xanthine oxidase inhibitor used in management of chronic gout. It acts by reducing the amount of uric acid by inhibiting purine metabolism. A middle-aged hypertensive female who was on allopurinol for 7 months presented with generalized weakness and exertional dyspnea. Investigations revealed pancytopenia: normocytic normochromic anemia (Hb-3.2g/dL, TLC-3400/mm3) and severe thrombocytopenia (Platelets-1000/mm3) with mild hepatosplenomegaly and grade 2 medico renal disease with normal cardiac status. Nutritional, hemolytic and infective causes were ruled out. She was transfused with fresh whole blood, platelets, administered empirical antibiotics and started on steroids. Initially, she responded to treatment but later developed an episode of convulsions with anuria and succumbed to leukopenic sepsis secondary to hypo/aplastic anemia probably due to allopurinol. Allopurinol is used extensively in the management of chronic gout and is well tolerated due to its safety profile. But we here report a case of allopurinol induced aplastic anemia leading to the demise of a patient. Allopurinol though safe needs careful monitoring.
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PMID:Allopurinol: Sorrow to the marrow. 3275 32