Cases reported "Splenomegaly"

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1/98. Massive splenomegaly and Epstein-Barr virus-associated infectious mononucleosis in a patient with gaucher disease.

    PURPOSE: gaucher disease should be considered in the differential diagnosis of a patient with Epstein-Barr virus (EBV) infection who has unexplained or disproportionate splenomegaly. patients AND methods: A previously asymptomatic adolescent with EBV-associated infectious mononucleosis and massive splenomegaly is described. He was found to have gaucher disease on bone marrow biopsy, which was performed to exclude a hematologic malignancy. The diagnosis was confirmed by assay of beta-glucosidase enzyme activity. RESULTS: Regression of splenomegaly and improving hematologic indices. CONCLUSION: patients with infectious mononucleosis and disproportionate organomegaly should be investigated to exclude a hematologic malignancy or an underlying storage disorder such as gaucher disease.
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ranking = 1
keywords = infectious mononucleosis, mononucleosis
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2/98. vitamin a toxicity secondary to excessive intake of yellow-green vegetables, liver and laver.

    We report a case of sudden onset of vitamin a poisoning. A 20-year-old Japanese woman had been eating pumpkin and only a very limited amount of other foods on a daily basis for 2 years. She was overly concerned about weight reduction. Aurantiasis cutis and abnormal liver function tests were noted by her family doctor in 1995 when she was 18 years old. At that time, she stopped eating pumpkin. However, she secretly continued an excessive intake of other beta-carotene-rich vegetables, liver and laver for about 2 years. Two and one-half years after being seen by her family physician, she experienced sudden onset of low-grade fever, limb edema, cheilitis, dry skin, and headache. These symptoms worsened daily. A liver needle biopsy was performed, and it showed a normal portal tract along with fat-laden Ito cells in the space of Disse. A final diagnosis of vitamin a poisoning and hepatic injury secondary to an eating disorder was made. Her symptoms and serum beta-carotene levels returned to normal with successful adjustment of her diet.
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ranking = 0.00024453989388496
keywords = fever
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3/98. infectious mononucleosis.

    infectious mononucleosis is a unique disease in its hematologic aspects; it is different from the frequently occurring acute microbial diseases in that it affects primarily the reticuloendothelial system; and it is interesting serologically because of the heterophil antibody reaction, as well as the multiplicity of antibodies which may be produced. The diagnosis should be suspected clinically before hematology is reported - by remembering the prototypes. In fact, a patient between 16 and 25 years old complains of sore throat and fever is more likely to have infectious mononucleosis than another disease; and if - in addition - he is jaundiced, a diagnosis of infectious mononucleosis is almost certain. Finally, a negative result of treatment with corticosteroid has the diagnostic significance mentioned above. Positive effect of treatment has no diagnostic significance.
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ranking = 0.5350541071817
keywords = infectious mononucleosis, mononucleosis, fever
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4/98. Congenital acute megakaryoblastic leukemia (M7) with chromosomal t(1;22)(p13;q13) translocation in a set of identical twins.

    Chromosomal translocations at t(1;22)(p13;q13) have been reported to occur in a number of infants with acute megakaryoblastic leukemia. A set of female twins with acute megakaryoblastic leukemia are reported with this unique translocation of 1p13 to 22q13. The twins presented at 2 months of age with fever and poor feeding and subsequently developed progressive hepatosplenomegaly. One twin died before treatment could be started; the other became septicemic 5 days after initiation of chemotherapy and eventually died.
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ranking = 0.00024453989388496
keywords = fever
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5/98. Hepatosplenic B-cell lymphoma associated with hemophagocytic syndrome: a case report.

    While T-cell non-Hodgkin's lymphoma (NHL) associated with hemophagocytic syndrome (HPS) has been frequently observed, B-cell NHL associated with HPS has been rarely reported. We report a case of hepatosplenic B-cell lymphoma associated with HPS in a 41-year-old woman who presented with fever of unknown origin. An abdominal CT scan revealed splenomegaly with focal splenic infarction. splenectomy and a liver wedge biopsy showed sinusoidal-pattern infiltration of medium to large tumor cells with positive reaction to a B-lymphocyte marker. Findings on bone marrow examination showed proliferation of histiocytes with avid hemophagocytosis.
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ranking = 0.00024453989388496
keywords = fever
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6/98. Splenic littoral cell angioma in an infant.

    A 1-year-old girl presented with fever, asthenia, and splenomegaly with hypersplenism. Abdominal ultrasound scan and magnetic resonance imaging showed multiple nodular cystic masses in an enlarged spleen. The histological examination of the resected spleen showed a novel type of vascular tumor called littoral cell angioma. The histopathologic and immunohistochemical features of this rare lesion are described. Distinction from other splenic vascular tumors is stressed because the clinical behavior of this new entity seems to be benign.
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ranking = 0.00024453989388496
keywords = fever
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7/98. Disseminated histoplasmosis with reactive hemophagocytosis: aspiration cytology findings in two cases.

    Two cases of disseminated histoplasmosis associated with reactive hemophagocytic syndrome are described. The clinical presentation was with prolonged unexplained fever and hepatosplenomegaly. On a strong clinical possibility of tuberculosis, antitubercular treatment was initiated in both patients. Lymph node (case 1), splenic (case 2), and bone marrow aspiration, however, showed sheets of proliferating histiocytes, and intracellular and extracellular histoplasma organisms. Aspiration cytology was thus valuable in establishing the final diagnosis. The patients had a fulminant clinical course and died of hemorrhagic shock within 48 hr of hospital admission before specific therapy could be initiated. histoplasmosis can mimic tuberculosis clinically. There is a need for an increased awareness of the clinicopathological spectrum of histoplasmosis, especially its rarer manifestations as hemophagocytic syndrome. In suspected cases, aspirations from the lymph node, liver, and spleen can be performed safely and should be utilized for early diagnosis.
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ranking = 0.00024453989388496
keywords = fever
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8/98. Splenic tuberculosis presenting as hypersplenism.

    A 9-year-old girl with a 5-6-month history of abdominal distension and fever was found to have massive splenomegaly with features of hypersplenism. Apart from a strongly positive Mantoux test, all investigations for massive splenomegaly proved negative. splenectomy was carried out and histopathological examination of the spleen revealed granulomatous lesions suggestive of tuberculosis. The child improved after splenectomy and anti-tuberculous therapy and is doing well on follow-up. Splenic tuberculosis should be considered as an unusual cause of massive splenomegaly and hypersplenism.
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ranking = 0.00024453989388496
keywords = fever
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9/98. An unusual presentation of shock in a previously healthy child.

    A previously healthy, 7-year-old African-American male presented to the emergency department with a high fever and signs of compensated shock. With this case presentation, we review the clinical evaluation of a child in shock and discuss the importance of early recognition and treatment. Additionally, the varied etiologies of shock in a previously healthy individual are discussed.
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ranking = 0.00024453989388496
keywords = fever
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10/98. Hemophagocytic syndrome.

    This case report is about an elderly man who presented with a long-standing history of high-grade fever and weight loss. He initially had only hepatosplenomegaly, but then developed jaundice. He also had pancytopenia and raised liver enzymes. His septic screen was negative, but he had a positive Monospot test and immunoglobulin g for Epstein-Barr virus. The liver biopsy showed sinusoidal phagocytosis and the subsequent bone marrow aspiration and biopsy showed significant hemophagocytosis, hence Hemophagocytic syndrome was diagnosed. The fever was refractory to antibiotic and anti-tuberculosis therapy, but it responded only partially to steroids. Full response was only noticed following anti-viral treatment in the form of intravenous ganciclovir. The patient's general condition, liver enzymes, bilirubin, hematological parameters and even the weight returned back to their normal range 2 weeks after ganciclovir therapy. Cessation of this drug resulted in relapse of his symptoms and oral antivirals did not help. splenectomy, steroid pulse therapy and immunosuppressive treatment were only partially helpful. Reintroduction of ganciclovir did help for a short period. We conclude that our patient had virus-associated hemophagocytic syndrome most likely related to Epstein-Barr virus infection, which was then confirmed by the splenic biopsy, and that ganciclovir can be of great help in eradicating the virus and treating the disease, provided that it is given for a long enough period.
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ranking = 0.00048907978776992
keywords = fever
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