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 3-year-old, male, domestic shorthaired cat was presented with a 3-day history of anorexia and depression. The cat was moderately dehydrated, had pale, slightly icteric, mucous membranes, oral ulcerations, and mild hepatosplenomegaly. A feline leukemia virus (FeLV) antigen test was positive. CBC results obtained at initial presentation included severe normocytic, normochromic, nonregenerative anemia, severe thrombocytopenia, and marked leukocytosis (>100,000/microL) with 77% eosinophils. After 15 days of treatment with prednisone and doxycycline, the cat had persistent severe nonregenerative anemia (HCT 3.4%), thrombocytopenia (28,000/microL), and extreme eosinophilia (total eosinophils, 123.1 x 10(3)/microL; segmented 103.0 x 10(3)/microL; immature 20.1 X 10(3)/microL). Cytologic examination of aspirates from bone marrow, liver, lymph nodes, and spleen revealed a predominance of mature and immature eosinophils, many with dysplastic changes. The M:E ratio was 96.4. On histopathologic examination, multiple organs were infiltrated by eosinophilic granulocytes. Neoplastic cells in blood and bone marrow stained positive for alkaline phosphatase and were negative for myeloperoxidase, chloroacetate esterase, and alpha-naphthyl acetate esterase. On flow cytometric analysis of peripheral blood, the neoplastic cells were positive for CD11b and CD14. These findings were consistent with chronic eosinophilic leukemia. To our knowledge, this is the first report of chronic eosinophilic leukemia in a cat associated with naturally acquired FeLV infection, in which flow cytometry was used to characterize the neoplastic cells.
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PMID:Chronic eosinophilic leukemia in a cat: cytochemical and immunophenotypical features. 1712 54

Leukaemoid reaction is a rare, growth factor-driven, paraneoplastic manifestation of hepatocellular carcinoma. It may masquerade as the neutrophilic chronic myeloid leukaemia or as chronic neutrophilic leukaemia. A 52-year-old male presented with hepatosplenomegaly and severe leucocytosis. He had progressive leucocytosis, neutrophil alkaline phosphatase score elevated, liver function tests altered. FNAC from the mass in the liver revealed features of moderately differentiated hepatocellular carcinoma. The patient deteriorated within two weeks and died thereafter.
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PMID:Hepatocellular carcinoma presenting as neutrophilic leukaemoid reaction--a rare entity. 1823 11

We diagnosed an 86-year-old woman with chronic neutrophilic leukemia (CNL) because she showed sustained leukocytosis dominated by mature neutrophils, hepatosplenomegaly, high neutrophilic alkaline phosphatase score, absence of the Ph chromosome, low serum level of granulocyte colony-stimulating factor (G-CSF) and granulocyte-macrophage colony-stimulating factor (GM-CSF), and no evidence of leukemoid reaction. We found that the extent of stimulation of her neutrophil functions by G-CSF and GM-CSF was greatly reduced compared to healthy donars neutrophils. We showed that CNL neutrophils have intact expression of granulocyte colony-stimulating factor receptor (G-CSFR) and granulocyte-macrophage colony-stimulating factor receptor (GM-CSFR). This suggests that failure of enhancement by G-CSF and GM-CSF in CNL neutrophil functions might be due to disturbances in the intracellular domains of G-CSFR and GM-CSFR, regardless of external cytokine stimulation. However, the patient's neutrophils did not show any mutations in the G-CSFR and GM-CSFR intracellular critical regions. We also showed that stat3 and mitogen-activated protein kinase activation by G-CSF or GM-CSF in the patient's neutrophils were significantly lower than those in healthy donor neutrophils. These results suggest that deficiency of CNL neutrophil function might be due to insufficiency of some inflammatory cytokine-specific signaling. Hence, we are the first to show that CNL neutrophils have partially insufficiency in some cytokine-specific signaling. Further studies are needed to elucidate the signal transduction pathways relating to functional defects in CNL neutrophils.
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PMID:Neutrophil function and cytokine-specific signaling in chronic neutrophilic leukemia. 1824 Dec 14

A 78 year-old lady presented with abdominal swelling and fatigue. She was anaemic with mild hypoalbuminaemia, and had a normal alkaline phosphatase. Computed tomography showed hepatosplenomegaly and mild ascites. Anti mitochondrial antibodies were strongly positive, as were anti nuclear antibodies, and the gamma glutamyl-transferase was shown to be elevated. A diagnosis of primary biliary cirrhosis was made. A brief discussion of treatment of primary biliary cirrhosis follows. The case is notable for the fact that primary biliary cirrhosis can manifest clinically without an elevation in alkaline phosphatase - normally the hallmark of the disease.
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PMID:Primary Biliary Cirrhosis with a normal Alkaline Phosphatase: a case report. 1862 74

We report four sporadic and three familial patients with Camurati-Engelmann disease. One patient had follow-up examinations over 8 years. Pain in the extremities and muscle weakness were common clinical symptoms. Most patients also had cranial nerve impairment, hepatosplenomegaly, a waddling gait, and elevated serum alkaline phosphatase levels. Long bones were affected in all. We discuss the differential diagnosis for this interesting bone entity.
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PMID:Camurati-Engelmann disease: imaging, clinical features and differential diagnosis. 1921 2

A 56-year-old male was admitted with symptoms of belching, abdominal pain and weight loss of 2 weeks duration. Examination revealed hepatosplenomegaly which was confirmed by computed tomography (CT). CT images also revealed filling defects in the portal vein and intrahepatic branches consistent with thrombosis and hepatosplenic infarcts. Alkaline phosphatase was elevated at 688 units, all other investigations, including full blood count, coagulation screen and tumour markers, were normal. Magnetic resonance cholangiopancreatography did not reveal any mass in the porta hepatis. Upper gastrointestinal endoscopy and colonoscopy were normal. Liver biopsy was normal and did not reveal any evidence of lymphoma. The raised alkaline phosphatase settled to reference range over a period of 3 weeks. Thrombophilia screen was negative. Contrast CT of the abdomen performed after 4 weeks displayed revascularisation of the previously thrombosed portal vein and intrahepatic branches. The patient has remained asymptomatic since and we note spontaneous recanalisation of the previously occluded portal vein.
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PMID:Spontaneous resolution of portal vein thrombosis. 2149 Aug 72

Background. The present study sought and compared the utility and safety of endoscopic retrograde cholangiopancreatography (ERCP) in the elderly and younger people in a great sample of Iranian population. Methods. Our study involved 780 patients undergoing diagnostic and therapeutic ERCP at the Taleghani hospital in Tehran between 2010 and 2011; among them, 558 patients were less than 70 years old and others were 70 years old or older. The patients were prospectively identified and data including clinical and biochemical features, ERCP procedures, ERCP diagnosis, and ERCP complications were gathered on them prospectively. Results. Clinical manifestations were comparable in young and older groups except for hepatosplenomegaly and constipation that were more prevalent in the elderly. Laboratory findings were similar in both groups other than mean levels of alkaline phosphatase, hemoglobin and albumin levels, which were higher in the elderly group. Selective biliary cannulation was technically more successful in the younger than in others (89.0% versus 81.8%). Common bile duct stone was the most frequent diagnosis in both young and older groups (36.6% and 45.9%, resp.), whereas ERCP was reported to be normal in 13.4% of the younger and 5.0% of the elderly patients. Post-ERCP complications were observed in 4.8% of patients aged less than 70 years in comparison with 2.3% of patients aged over 70 years. The most frequent complication was pancreatitis that was significantly more developed in young than older patients (3.6% versus 1.5%; OR: 8.216, P = 0.015). Conclusion. Diagnostic ERCP is safe and well tolerated in the elderly and even associated with significantly less risk than the younger.
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PMID:Utility and safety of ERCP in the elderly: a comparative study in iran. 2282 48

Gall bladder distension with acute viral acalculous cholecystitis is a rare event in pediatric cases with a high incidence of perforation, gallbladder necrosis and mortality. We report a two and a half year old female child presenting with fever, vomiting, pain abdomen, mild hepatosplenomegaly and tenderness in right hypochondrium. Laboratory investigations revealed hyperbilirubinemia and elevated alkaline phosphatase, but there was no evidence of bacterial or parasitic infection. Serology for viral hepatitis suggested acute Hepatitis A infection. Ultrasonographically, distended inflamed gallbladder without calculous was observed. Finally acute acalculous cholecystitis due to Hepatitis A virus was diagnosed and the child responded to the conservative management.
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PMID:Acalculous gallbladder distension in a young child due to HAV infection: Diagnostic dilemma. 2310 56

A 39-year-old male reported fevers, weight loss, watery loose stools, and decreased visual acuity in his right eye over the prior five years. He was pancytopenic, had an elevated American council on exercise level, total bilirubin, and alkaline phosphatase. Computed tomography revealed massive hepatosplenomegaly and emphysematous lung changes. Liver biopsy showed non caseating granulomas. The patient was diagnosed with extrapulmonary sarcoidosis and was treated with prednisone. The patient symptomatically improved but 5 mo later presented with abdominal pain caused by perforation of the cecum. He underwent a cecectomy and pathology revealed pneumatosis cystoides intestinalis. This represents the first reported association between pneumatosis cystoides intestinalis and sarcoidosis. The etiology of pneumatosis cystoides intestinalis in this case was likely multifactorial and involved both effects of the corticosteroids as well as the advanced nature of the gastrointestinal sarcoidosis. Furthermore this case has the unique features of emphysematous lung changes and pancytopenia which are uncommon with sarcoidosis.
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PMID:Gastrointestinal sarcoidosis associated with pneumatosis cystoides intestinalis. 2346 42

Disseminated cryptococcosis is rare in immunocompetent hosts and hepatic manifestations as the presenting feature is further rare. We report a case of disseminated cryptococcosis with hepatic involvement as an initial manifestation in a previously healthy, immunocompetent adult. A young married female presented with progressive jaundice, anorexia, weightloss, cough with expectoration, and hepatosplenomegaly. Biochemical profile showed liver function derangement with increased transaminases, alkaline phosphatase, bilirubin with deranged coagulation assay, and decreased albumin. The patient was treated initially for disseminated tuberculosis with associated sepsis, but she succumbed on the third day. Diagnosis of cryptococcosis was made on the basis of sputum culture, serology, and liver histopathology.
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PMID:Disseminated cryptococcosis with hepatic dysfuction as the initial manifestation in an immunocompetent adult. 2364 46


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