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)

Isolated extrathoracic sarcoidosis is a rare entity. We present a 25-year old woman with recurrent episodes of fever of unknown origin in the 10 years previously, with a new episode of fever with hepatosplenomegaly, abdominal adenopathies and cranial osteolysis without pulmonary or mediastinal affectation. With the suspicion of linfoproliferative disease we have resected the spleen with a weight of 1500 g. and multiple non caseous granulomata also in the liver and adenopathies. The clinical presentation is compatible with an atypical sarcoidosis without thoracic affectation and with two rare associated clinical manifestation namely giant splenomegaly and sarcoidosis of the skull.
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PMID:[Extrathoracic sarcoidosis with giant splenomegaly and cranial osteolysis]. 906 37

A case of multiple myeloma associated with HIV disease and hepatosplenomegaly presented to us as pyrexia of unknown origin, is reported. Because of paucity of such cases in the literature, the case is dealt in detail and the literature reviewed briefly.
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PMID:Multiple myeloma complicating HIV-infection. A case report. 935 21

Visceral leishmaniasis (Indian kala-azar) caused by infection due to the protozoan Leishmania donovani is endemic in the Indian subcontinent and adjoining regions. Prolonged fever, hepatosplenomegaly, anaemia and pancytopenia, in the appropriate setting, are important clinical markers towards the diagnosis. Diagnosis is established by blood film or bone marrow examination for Leishman Donovan (LD) bodies and/or culture. Treatment with sodium stibogluconate, pentamidine isethionate or even amphotericin B is usually successful. We report the first case of culture-proven visceral leishmaniasis in Singapore, in a 30-year-old Bangladeshi worker who presented with pyrexia of unknown origin (PUO). He had the classical constellation of symptoms and signs as mentioned above. Diagnosis was confirmed by culture on the Novy, NcNeal and Nicolle (NNN) medium. He was successfully treated with 20 days of pentamidine isethionate daily infusions at a dose of 2 mg/kg/day.
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PMID:A case report of visceral leishmaniasis in Singapore. 949 84

We report a patient aged 41 years with fever of unknown origin. Notable aspects of his travel history were a trip to the Philippines and a sailing trip around Sicily. The patient presented with fever up to 40 degrees C since 4 weeks, weakness, headache, hepatosplenomegaly and night sweat. No specific cause could be found. Based on clinical findings tuberculosis was suspected and empirical tuberculostatic treatment was started. However, during the following 6 weeks the patient's condition deteriorated. A bone marrow biopsy performed to exclude a haematological malignancy revealed Leishmania sp. in macrophages. This histological diagnosis was confirmed retrospectively by re-examination of a previously performed liver biopsy and by an increased anti-leishmania serum antibody titer of 1:1280. The patient was treated with sodium stibogluconate (pentostam, 850 mg) for 30 days and recovered slowly.
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PMID:A case of visceral leishmaniasis in Austria. 962 27

A diagnosis of familial hemophagocytic lymphohistiocytosis (FHL) was established in an 18-month-old boy who presented with prolonged fever of unknown origin, severe pancytopenia, hepatosplenomegaly and hypofibrinogenemia. Serum levels of ferritin and soluble interleukin-2 receptor (SIL2R) were highly elevated, and the number of natural killer (NK) cells was markedly decreased. An allogeneic stem cell donor was neither found in the family nor in unrelated donor registries; however, an umbilical cord blood (UCB) donor request revealed a 5/6 HLA-matched UCB. After conditioning with busulphan 16 mg/kg body weight (BW), cyclophosphamide 120 mg/kg BW and etoposide (VP-16) 900 mg/m2 the patient received 19.6 x 10(7)UCB nucleated cells/kg BW. White blood count (WBC) reached 1.0 x 10(9)/l on day +45. Chimerism studies showed full and permanent hematopoietic and lymphopoietic engraftment on day +16. However despite full engraftment the patient still experienced two severe relapses of his disease after stem cell transplantation with the highest ferritin level in the range of 10 3967 microg/l (n = 7-142). NK cell function appeared only 6 months after UCB transplantation followed by a decrease of FHL markers and resolution of disease activity. This clinical outcome indicates that unless competent immunologic engraftment after transplantation is established, FHL is capable of relapsing even if complete three-lineage engraftment is achieved.
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PMID:Unrelated 5/6-locus matched umbilical cord blood transplantation in a 23-month-old child with hemophagocytic lymphohistiocytosis. 972 76

The clinical features of inflammatory pseudotumour of lymph nodes, a distinct non-malignant histopathological entity firstly described in 1988, are summarized based upon a detailed analysis of 4 personal cases and 47 cases reported in the literature. The mean age of the patients is 38 years (range 8 to 82 years) and there is no gender predilection. One third present with asymptomatic lymphadenopathy and 47% present with fever, nearly all meeting the criteria of fever of unknown origin. Abdominal complaints are occasionally present. Intermittence of symptoms is common. Hepatosplenomegaly is unusual. All lymph node areas may be involved but abnormalities are mostly confined to one or two anatomic regions. No extranodal involvement has been reported although inflammatory pseudotumour may occur in several organs with similar morphological features and identical signs of inflammations. Routine blood tests are normal except for signs of inflammation. The lesions are Gallium-avid. Diagnosis is based upon typical histopathological features. The prognosis is favorable and surgical resection frequently leads to cure. Spontaneous resolution of symptoms has been reported and nonsteroidal anti-inflammatory drugs may suppress the clinical manifestations.
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PMID:Fever of unknown origin due to inflammatory pseudotumour of lymph nodes. 1002 46

An 8-years-old boy was admitted with fever of unknown origin, cervical lymphadenopathy and hepatosplenomegaly and weight loss. His mother's HIV infection was diagnosed two weeks before his hospitalization, so he was diagnosed as perinatally acquired AIDS. Serology and serial cultures were negative for viral infections, toxoplasmosis, chagas, tuberculosis and atypical mycobacterium. The patient met clinical and laboratory criteria for hemophagocytic syndrome (HS) that was confirmed on bone marrow aspirate and biopsy. A cervical lymph node biopsy was performed which was diagnosed as Hodgkin's disease (HD) diffuse fibrosis lymphocyte depletion subtype. EBERs in situ hybridization and LMP-1 immunohistochemistry on the lymph node biopsy established the EBV association. On the basis of a sequence of appearance of the clinical, laboratory and histological signs, HIV, EBV or HD may have triggered HS as the last fatal event in this pediatric patient.
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PMID:EBV-associated Hodgkin's disease in an HIV-infected child presenting with a hemophagocytic syndrome. 1169 13

We report an unusual case of aggressive natural killer (NK)-like cytotoxic T-cell lymphoma in a previously healthy immunocompetent West African male. He presented with a fever of unknown origin, subsequently developed erythematous skin nodules, generalized lymphadenopathy, and hepatosplenomegaly, and then died of multiple organ failure. A skin nodule and lymph node biopsy showed an infiltrate of pleomorphic atypical medium and large lymphoid cells with extensive necrosis and prominent apoptosis. Peripheral blood and ascites also harbored these cells, with cytology revealing irregular nuclear folding and basophilic cytoplasm, and some with azurophilic cytoplasmic granules. Flow cytometry and immunohistochemistry demonstrated the expression of CD2, CD7, CD8, CD30, CD56, and cytoplasmic but not surface CD3. In situ hybridization demonstrated Epstein-Barr virus transcripts. A monoclonal T-cell receptor gamma chain gene rearrangement was detected by polymerase chain reaction. This is the first reported case of an NK-like T-cell lymphoma with these unusual features, making precise classification difficult. Some features suggest an NK1.1 or NKT lymphocyte origin. Because the earliest clinical manifestation was splenomegaly and abnormal liver function, the normal cellular counterpart may be a distinct subset of NK1.1 cells normally present in hepatosplenic sinusoids. This tumor disseminated early and pursued a fulminant clinical course, thus emphasizing the importance of early recognition and diagnosis.
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PMID:Aggressive Epstein-Barr virus-associated, CD8+, CD30+, CD56+, surface CD3-, natural killer (NK)-like cytotoxic T-cell lymphoma. 1175 78

The clinical case of one patient with fever of unknown origin, due to granulomatous hepatitis of tuberculous etiology was presented. The patient was a a 50-year-old woman, with 50 days illness characterized by chills, 39 degrees C fever and heavy diaphoresis. She had a record of seven malaria cases. She looked thin and pale at the initial physical examination. During the evolution, she developed pancytopenia, massive hepatosplenomegaly, jaundice, and anasarca. The patient underwent screening tests for infection, neoplasias, collagenosis, and granulomatous diseases. The laboratory tests showed transaminase-alkaline phosphatase dissociation, which led to the final diagnosis of tuberculosis, through the histological examination of the liver parenchyma. The specific treatment against tuberculosis caused remission of fever, ascites, and hepatomegaly and normalization of liver tests, with satisfactory clinical evolution.
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PMID:[Granulomatous tuberculous hepatitis as cause of fever of unknown origin]. 1252 48

A 2-month-old male infant with consanguineous parents had fever of unknown origin, pancytopenia, and hepatosplenomegaly. Laboratory tests, peripheral blood smear and bone marrow revealed haemophagocytic lymphohistiocytosis.
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PMID:[Diagnostic image (137) An infant with fever and pancytopenia. Hemophagocytic lymphohistiocytosis]. 1275 77


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