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)

An autopsy case of a 19-year-old boy who had shown typical gargoyle features, strictly consistent with mucopolysaccharidosis type II (Hunter's syndrome) was reported. Histologically, cytoplasmic vacuolar change was found in hepatocytes, sinusoidal epithelium of spleen, follicular cells of thyroid, Sertoli cells of testis, chromophobe cell of pituitary and generalized fibroblast-like cells including meninges, cardiac valve and periosteum. The vacuoles consisting of membrane-bound structures with flocculus protein-like material and occasional electron dense bodies on electron microscopy, were considered to be the site of mucopolysaccharide deposition by histochemical analysis. Deposition of lipid material consistent with so-called membranous cytoplasmic body was observed in the neurons of central, peripheral and autonomic nervous system. Hepatosplenomegaly could be explained by cytoplasmic deposition, but the cause of cardiomegaly remained further to be studied. Biochemically hepatic mucopolysaccharide was identified as heparan sulfate, while in the kidney dermatan sulfate and heparan sulfate were detected. The correlation between morphology and biochemistry, and between deposition and degeneration was discussed.
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PMID:Morphological and biochemical studies of a case of mucopolysaccharidosis II (Hunter's syndrome). 13 78

An infant case of erythrophagocytic lymphohistiocytosis is reported. The disease began at the age of two months with hepatosplenomegaly and intractable fever, and later developed pancytopenia. Splenectomy had to be performed at the age of six months and led to the final diagnosis. After the age of nine months the patient developed progressive neurological signs (loss of visual function convulsions, opisthotonus and CSF pleocytosis). The changes in the CT-scan of the brain were explained by the neuropathologic findings after the death of the patient at the age of 13 months. The markedly atrophic brain showed diffuse and mainly perivascular infiltration of brain and meninges with lympho- and histiocytes and extensive microscopic calcification.
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PMID:Neurological and neuropathological findings in familial erythrophagocytic lymphohistiocytosis. 661 91

Trypanosomiasis, caused by Trypanosoma congolense, was diagnosed for the first time in Israel in two boxer dogs imported from Kenya. The dogs developed clinical signs two days after arrival and succumbed to the disease within four days. The major clinical and clinicopathological findings included anaemia, haemorrhages, lymphadenomegaly, hepatosplenomegaly and neurological signs. Histopathology showed lymphocytic-plasmacytic infiltration in the skin, brain, meninges, kidney and liver.
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PMID:Trypanosoma congolense infection in two dogs. 772 95

Disseminated histoplasmosis has been recognized as a serious opportunistic infection in patients with acquired immunodeficiency syndrome (AIDS). However, cases reported in the literature have been predominantly in adult patients. Here we report an infant with AIDS who presented with fever, cough, rhinorrhea, hepatosplenomegaly, pancytopenia and coagulopathy, and died of respiratory failure. Autopsy revealed disseminated histoplasmosis involving multiple organs including lungs, intestines, liver, spleen, bone marrow, lymph nodes, kidneys, and meninges. The diagnosis was established based on histomorphology and confirmed by blood culture.
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PMID:Disseminated histoplasmosis in an infant with acquired immunodeficiency syndrome. 989 38

Multicentric lymphomas, usually of B-cell origin, are rather common neoplasms in pigs, which usually affect lymph nodes and different organ systems. An uncommon manifestation of malignant lymphoma represents neoplastic peripheral nerve involvement, which has been reported in human medicine. So far, only single cases of lymphoma with manifestation in peripheral nerves (known as neurolymphomatosis) without leukemic conversion have been mentioned in different animal species others than swine. The present case report describes the occurrence of a multicentric T-cell lymphoma in a fattening pig with marked leukocytosis. The pig was presented with symptoms of lethargy, weight loss, and fever. Bone marrow aspirate contained all stages of granulocytic and erythrocytic precursors, and a remarkable large number of eosinophiles and undifferentiated blasts. At necropsy, the carcass showed generalized lymphadenopathy and severe hepatosplenomegaly. Histological findings included neoplastic round cell infiltrations in lymph nodes, bone marrow, tonsils, lung, spleen, liver, kidneys, urinary bladder, small and large intestine, and meninges. Additionally, neoplastic cells were present in the sciatic and medial plantar nerve of the left hind leg. The diagnosis T-cell lymphoma was confirmed immunohistochemically. To the author's knowledge, this work represents the first report of peripheral nerve involvement in a pig with multicentric T-cell lymphoma accompanied by CNS involvement and leukemia.
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PMID:Multicentric T-cell lymphoma accompa- nied by infiltration of the peripheral and central nervous system in a fattening pig. 2659 79

Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disorder that is clinically characterized by fever, hepatosplenomegaly, cytopenia and sometimes vague or dramatic central nervous system (CNS) dysfunction. FHL affecting the CNS imitates several neurologic disorders and may be misdiagnosed, in particular when family history is unknown. We report an autopsy case of FHL that was firstly considered as progressive encephalitis. FHL was suspected after sibling had been affected by hemophagocytosis and the same CNS symptoms. Histopathologically, lymphocytes and macrophages infiltrated into the meninges, perivascular space, and parenchyma of the brain. Those lymphocytes were positive for CD3, CD8, GranzymeB, and negative for CD4, perforin. FHL must be included in the differential diagnostic considerations in children with progressive encephalitis.
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PMID:[Familial Hemophagocytic Lymphohistiocytosis, That was Considered as Progressive Encephalitis before Sibling was Diagnosed]. 2659 29