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)

Neurological and skin involvements usually dominate the clinical presentation of intravascular lymphomatosis (IL), while fever is the most frequent general sign. However, an onset only characterized by fever of unknown origin (FUO) has been rarely reported. We would like to describe a further case of IL, which presented a long-lasting FUO before the diagnosis. At admission, physical examination detected hepatosplenomegaly without lymph nodes enlargement or dermatological or neurological abnormalities. Significant laboratory data included severe anemia, leukopenia, thrombocytopenia, and increased serum LDH. Moreover, a chest CT evidenced bilateral multiple pulmonary infiltrates and pleural effusion. After the development of proteinuria, a diagnosis of large B-cell intravascular lymphoma was made with a renal biopsy 10 months after the onset of the clinical manifestations. So far, more than 100 cases of IL have been reported and the diagnosis often turned out to be difficult, as clinical signs did not point to a lymphoproliferative disorder. This report confirms that FUO is not only frequently associated with IL but that it even marks the real onset of the disease. We are then tempted to conclude that undiagnosed fever is not so rare in IL and if we call it FUO, it is only because diagnosis is necessarily elusive and hence time-consuming.
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PMID:Long-lasting fever of unknown origin preceding the diagnosis of intravascular lymphomatosis: a further case stimulates some remarks. 1458 55

A 52-year-old Japanese man presented with fever spikes, generalized fatigue, anorexia, and anasarca. The patient was referred for the evaluation of fever of unknown origin in association with swelling of cervical, axillary, and inguinal lymph nodes. He also manifested nephrotic syndrome, acute renal failure, hepatosplenomegaly, massive pleural effusion, ascites, disseminated intravascular coagulation, and hypergammaglobulinemia. C-reactive protein was positive and plasma vascular endothelial cell-derived growth factor (VEGF) and serum interleukin-6 levels were markedly elevated. Lymph node biopsy results showed that findings were compatible with Castleman's disease of hyaline vascular type associated with interfollicular plasmacytosis. In conjunction with the clinical findings, a diagnosis of multicentric Castleman's disease was made. The patient underwent renal biopsy because of nephrotic syndrome, and the results showed proliferation of mesangial cells, lobulation of glomeruli, and tram track pattern of the capillary wall without immune complex deposition. Electron microscopy showed widening of the subendothelial space. No electron-dense deposits were present in both mesangial and subendothelial regions. Pathologic features were compatible with glomerular microangiopathy and membranoproliferative glomerulonephritis-like lesions. With corticosteroid therapy, systemic symptoms disappeared; both VEGF and interleukin-6 levels were normalized, and he went into complete remission of nephrotic syndrome. In this article, the role VEGF plays in the pathogenesis of nephrotic syndrome and glomerular microangiopathy is discussed.
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PMID:Multicentric Castleman's disease associated with glomerular microangiopathy and MPGN-like lesion: does vascular endothelial cell-derived growth factor play causative or protective roles in renal injury? 1471 66

Primary MDS is a group of heterogenous clonal haematopoetic disorders. In a third of patients MDS terminates as acute myeloid leukaemia, usually resisitant to treatment, while the others succumb due to infections and haemorrhage. Conservative managements of MDS (chemotherapy, haematopoetic growth factors, modulation of cytokine network) are unsuccessful, while the bone marrow transplantation is the only definite treatment. We reviewed clinical and haematological presentations, frequency of dysplastic features, histological and cytogenetic findings in 29 children with primary MDS. Indications for haematological evaluation in our patients were symptoms and signs of isolated or combined cytopenias, fever of unknown origin and frequent infections. Hepatosplenomegaly was found in 19 (65%) patients, while this pattern was found in 10% of adult patients. Normochromic anaemia was found in 25 (86%) patients and thrombocytopenia in 23 (76%). Patients presenting pancytopenia had the lowest probability of survival. Degree of dysplasia, histology and kariotype of bone marrow had no influence on survival rates. Prognostic factors in paediatric MDS are of limited significance, as MDS in children is an absolute indication for bone marrow transplantation.
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PMID:[Primary myelodysplastic syndrome in children]. 1563 84

We report a 6-year-old boy who was diagnosed as having neuron-specific enolase (NSE)-positive pro-T cell type lymphoblastic lymphoma preceded with a variety of symptoms such as skin rash, giant splenomegaly, and hyper-gamma globulinemia. He first showed cervical lymphadenopathy in June 1999, followed by a fever of unknown origin with atypical erythema, hepatosplenomegaly, and a few lymphoblastoid cells present in the bone marrow in September. However, no specific treatments were started at this point because a cervical lymph node biopsy failed to show malignancy and the patient's signs and symptoms resolved spontaneously. Two months later, oral prednisolone therapy was started due to recurrence of the fever and erythema, but resulted in exacerbation of the skin lesions and generalized lymphadenopathy. A biopsy of the right inguinal lymph node performed in January 2000 revealed proliferation of lymphoblastic cells positive for CD3, CD5 and NSE with a rearrangement of T cell receptor gene Jdelta, leading to the diagnosis of lymphoblastic lymphoma. After intensified chemotherapy, he received an autologous peripheral blood stem cell transplantation and has been in complete remission for 4 years.
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PMID:[NSE-positive lymphoblastic lymphoma in a boy with cutaneous involvement, giant splenomegaly, and hyper-gamma globulinemia]. 1567 16

A 49-year-old woman was admitted to our hospital because of fever of unknown origin. The patient had long-lasting spiking fever, hepatosplenomegaly, pleural effusion, and skin rash. Laboratory tests showed marked leukocytosis and an extremely high serum ferritin level (240 000 ng/ml) accompanied by disseminated intravascular coagulation and hemophagocytic syndrome. Most of the patient's features were compatible with a diagnosis of adult-onset Still's disease (AOSD), the rash, however, was not a typical rheumatoid rash but multiforme erythema. Biopsy of a breast nodule revealed breast cancer, leading us to a diagnosis of paraneoplastic syndrome mimicking AOSD. Although this is a rare disorder, cases resembling the present one have been reported, indicating the importance of including paraneoplastic syndrome in the differential diagnosis of AOSD.
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PMID:A case of paraneoplastic syndrome mimicking adult-onset Still's disease. 1714 3

Hyper-reactive malarial splenomegaly (HMS) or Tropical splenomegaly syndrome(TSS), occurs in areas of high transmission of malaria. These children usually presents with gross splenomegaly and abdominal discomfort, while fever is not the usual manifestation in majority of them. It is a disease of young adults and rarely reported below 8 years of age. Here it is reported a three-year-old child who presented as pyrexia of unknown origin with hepatosplenomegaly, diagnosed as HMS.
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PMID:Hyper-reactive malarial splenomegaly: rare cause of pyrexia of unknown origin. 1747 89

We report a case of a previously healthy 38-year old lady who presented with prolonged fever and hepatosplenomegaly. Intensive investigations were performed for pyrexia of unknown origin which revealed negative. CT scan of the abdomen showed multiple hypodense lesions which did not respond to broad-spectrum antibiotics. Percutaneous biopsy of the splenic lesion revealed granuloma formation and Langhan's giant cells suggestive of TB. She responded well with anti- TB medication but required extended treatment duration of 24 months due to persistence of the splenic lesion on repeated CT scans. This case illustrates a very rare clinical entity of isolated splenic TB with a therapeutic dilemma following incomplete resolution, despite prolonged treatment.
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PMID:Splenic tuberculosis presenting as pyrexia of unknown origin. 1768 77

Hemophagocytic lymphohistiocytosis (HL) is a rare syndrome, although more common in children, that may be underdiagnosed. The clinical presentation can be aggressive, and patients may rapidly develop lethal multiple organ failure....HL simulates the presentation of infectious sepsis, although the response to treatment and evolution are worse. HL should be suspected in young children with persistent fever of unknown origin, general malaise, hepatosplenomegaly, cytopenia, elevated triglycerides and ferritin, and decreased fibrinogen. Brain MRI shows diffuse leptomeningeal and perivascular enhancement, patchy areas of hyperintensity in the white matter of both cerebral hemispheres on T2-weighted sequences, and cerebral atrophy. Diffusion-weighted sequences are useful for staging the lesions. We present a fatal case of familial HL and review the literature about the clinical, histological, and radiological characteristics of this disease.
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PMID:[Familial hemophagocytic lymphohistiocytosis: Neuroradiological findings]. 2004 6

Disseminated tuberculosis can present in various ways including prolonged fever / pyrexia of unknown origin, hepatosplenomegaly, lymphadenopathy, meningitis and rarely extreme forms of hematological abnormalities such as pancytopenia and leukemoid reaction. We hereby report a case who presented with short history of fever, associated with vomiting and altered sensorium. He also had evidence of meningitis on neuroimaging with equivocal CSF finding. During his stay, he showed a spectrum of interesting hematological findings, including severe pancytopenia on peripheral smear, hemophagocytosis, epithelioid cell granuloma with Langhans' giant cells and focal necrosis consistent with tuberculosis on bone marrow examination. He showed an excellent clinical as well as hematological response to four drug antitubercular treatment (RHZE). The report highlights the significance of hematological picture in final confirmation of tuberculosis which may otherwise be passed off as nutritional or other unrelated causes.
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PMID:Disseminated tuberculosis: interesting hematological observations. 2104 80

Fever of unknown origin remains a diagnostic challenge with up to 50% of unexplained cases. Adult-onset Still disease typically present high fever, arthralgia, rash, pharyngitis and serositis, lymphadenopathy and hepatosplenomegaly, inflammatory markers and hepatic enzymes elevation and negative immunological study. The authors present a 46-year old man with previous admissions for fever of unknown origin with a 3-week history of fever and systemic symptoms. The study disclosed anaemia, inflammatory markers and hepatic enzymes elevation, splenomegaly and negative serological and immunological studies. The patient posteriorly presented polyarthralgias and cutaneous rash. The introduction of corticotherapy resolved symptoms and laboratories alterations. Adult-onset Still disease is a heterogeneous and rare disease and the lack of serologic markers as a true gold standard makes diagnosis difficult.
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PMID:[Adult-onset Still disease as the cause of fever of unknown origin]. 2114 36


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