Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Post-transplant lymphoproliferative disorder (PTLD) is a complication that can develop after either solid-organ or hematopoietic stem cell transplantation (HSCT). T-cell PTLD is a rare disorder, especially following autologous HSCT. Here we report a case of T-cell PTLD which occurred after autologous peripheral blood stem cell transplantation (PBSCT) for relapsed angioimmunoblastic T-cell lymphoma (AILT). Three months after the transplant, the patient developed fever with elevated plasma Epstein-Barr virus (EBV)-PCR values. The patient subsequently developed pneumonitis, hepatomegaly and marked pancytopenia due to hemophagocytosis. The patient died of multi-organ failure, despite antiviral and steroid pulse therapy. Our post-mortem study confirmed the marked proliferation of EBV-infected T-cells that differed from the original AILT clone and macrophages/histiocytes were observed in the marrow, liver, lymph nodes and lungs. Phagocytosis was most evident in the bone marrow. The patient's AILT remained in complete remission. To the best of our knowledge, this is the first case of fulminant EBV-associated T-cell lymphoproliferative disorder (LPD) following autologous HSCT.
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PMID:Fulminant Epstein-Barr virus (EBV)-associated T-cell lymphoproliferative disorder with hemophagocytosis following autologous peripheral blood stem cell transplantation for relapsed angioimmunoblastic T-cell lymphoma. 1633 97

The thrombophilia which can be either congenital or acquired in adult life has major implications in the abdominal vessels. The resulting portal vein thrombosis, Budd-Chiari syndrome and mesenteric vein thrombosis have a variety of consequences ranging from acute abdomen to chronic hepatomegaly and even totally asymptomatic patient in whom the only finding is pancytopenia. The complications like esophageal varices, portal gastropathy, ascites, severe hypersplenism, liver failure requiring liver transplantation are well known. Interesting features of collateral venous circulation showing itself as pseudocholangiocarcinoma sign and its possible clinical reflection as cholestasis are also known from a long time. The management strategies for these complications of intraabdominal vessel thrombosis are not different from their counterpart which is cirrhotic portal hypertension, but the prognosis is unquestionably better in former cases. In this review we presented and discussed the abdominal venous thrombosis, etiology and the resulting clinical pictures. There are controversial issues both in nomenclature, and management including anticoagulation problems and follow up strategies. In light of the current knowledge, we discussed some controversial issues in literature and presented our experience and our proposals about this group of patients.
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PMID:Etiology and consequences of thrombosis in abdominal vessels. 1653 66

We report a 19-year-old woman who was presented with B-symptoms, massive splenomegaly, hepatomegaly and hypersplenism. She underwent diagnostic/therapeutic splenectomy. Microscopically, the spleen showed a vaguely micronodular and diffuse proliferation of lymphoid cells in the white pulp that also involved the red pulp. On immunohistochemical staining, this proliferation consisted predominantly of CD3(+), CD7(+) small T cells with the presence of a minor population of CD15(-),CD30(-), CD20(+) large atypical B cells. A liver biopsy also showed a similar morphology to that seen in the spleen. After splenectomy, only the pancytopenia improved. A combined immunochemotherapy regimen (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone) was utilized, which resulted in a complete remission.
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PMID:T-cell-rich B-cell lymphoma of the spleen presenting with severe hypersplenism. 1710 97

In this study, the clinical and laboratory features of 26 infectious mononucleosis (IMN) cases who have been diagnosed between the years of 1984-2005 were evaluated retrospectively. The mean age of the patients was 26+/-11 years, the rate of being hospitalized was 65%, and mean hospitalization period was 9.2+/-6 days. Fever (81%), weakness (50%), sore throat (50%), headache (50%) and swollen neck (35%) were the most common symptoms, while in the physical examination cervical lymphadenopathy (81%), splenomegaly (69%), hyperemic pharynx (65%), hepatomegaly (54%) and tonsillitis (50%) were observed. Laboratory results yielded leukocytosis in 21%, leucopenia in 12%, anemia in 44%, thrombocytopenia in 5% and elevated transaminase levels in 84% of the patients. Of the patients 15 (57.7%) had the history of using antibiotics before the diagnosis. Serological diagnosis was performed by Paul-Bunnel test and/or IgM positivity against Epstein-Barr virus (EBV) viral capsid antigen (VCA). Tonsillo-pharyngitis secondary to edema and respiratory distress due to lymphadenopathy pressure were detected in four patients, whereas pancytopenia was established only in one patient, as complications. This study emphasized that, although IMN is a self-limited infection, the diagnostic difficulties may arise when the clinical course is atypical, and rarely seen life-threatening complications may also develop during IMN course.
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PMID:[Retrospective evaluation of patients who were diagnosed as infectious mononucleosis between 1984-2005]. 1742 57

Histoplasmosis occurs in approximately 5% of acquired immunodeficiency syndrome (AIDS) patients in endemic areas and often evolves to a disseminated picture if diagnosis is delayed and/or CD4 count falls below 150 cells x mm(3) without high active antiretroviral therapy (HAART). This report presents clinical features of patients with histoplasmosis admitted from 1992 to 2005. Of the 57 individuals, 45 (79%) were male, aged 20-40 years; 30 (52.6%) presented histoplasmosis together with HIV diagnosis and 35 (61.4%) referred illness course up to 4 weeks. Fever, hepatomegaly and/or splenomegaly, dyspnea and skin lesions were noticed in 50 (87.7%), 38 (66.7%), 30 (52.6%) and 25 (43.9%) patients respectively. High levels of lactic acid dehydrogenase, X-ray lung interstitial pattern, pancytopenia and CD4 count <100 cells x mm(3) were observed in 48 (84.2%), 35 (66%), 34 (59.6%) and 33 (94%) patients respectively. Mycological diagnosis was performed by one or more methods in all patients. Thirty nine (68.4%) received amphotericin B and/or itraconazole. A cure rate was observed in 76.9% and nine (23.1%) died early during therapy. Otherwise death occurred in 18 (31.6%) before diagnosis was completed. Despite free HAART disposal in public Brazilian health services, histoplasmosis still occurs as the first AIDS baseline condition in patients without antiretroviral therapy, many of whom are not receiving any medical care for HIV infection.
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PMID:Disseminated histoplasmosis in acquired immunodeficiency syndrome patients in Uberaba, MG, Brazil. 1825 50

Review of records for last 5 years has shown 4 cases of Type I Gaucher's disease in our institute. The cases were diagnosed on bone marrow aspiration, examination of splenectomy specimen, liver biopsy and post mortem in one case. The age range was 2 years to 22 years. Male to female ratio was 3:1. Splenectomy was performed in one case and one case received enzyme replacement therapy with high dose, low frequency regimen for six months without any favorable effect. All were Hindus. Family history of similarly affected and treated twin brother was available in one case. The predominant clinical presentation was pancytopenia and splenohepatomegaly with splenomegaly greater than hepatomegaly. Remarkable constitutional inferiority was noted in one case, which succumbed to death following acute illness and bleeding diathesis. Post mortem performed showed infiltration of spleen and liver with Gaucher cells, fibrosis and myeloid metaplasia in liver and lung.
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PMID:Gaucher's disease: report of 4 cases. 1830 46

An 82-year-old man was admitted with a 1-week history of chilling fever and dry cough. Laboratory tests revealed pancytopenia and elevated levels of C-reactive protein and lactic dehydrogenase (LDH). Screening for infectious diseases was negative. A bone marrow biopsy showed aspecific findings. The combination of pancytopenia, persistent fever, elevated LDH and hepatomegaly (demonstrated by ultrasound examination of the abdomen) was suggestive of the haemophagocytic syndrome. This was confirmed by very high levels of ferritin and soluble interleukin-2 receptor in the blood. In addition, re-examination of the bone marrow showed several haemophagocytic histiocytes. A polymerase chain reaction for Epstein-Barr virus (EBV) revealed a very high viral load. Since the patient had a history of an increased level of anti-EBV immunoglobulin-G, this was explained by a reactivation of the EBV infection. On the sixth day in hospital the patient developed signs of bilateral pneumonia and subsequent multiple organ failure. Despite intensive treatment the patient died. Autopsy revealed no haematological or other malignancies, but did show haemophagocytosis in many organs. It was then concluded that the patient had a virus-associated haemophagocytic syndrome, due to a reactivation of EBV, for which no underlying cause was found.
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PMID:[Clinical reasoning and decision-making in practice. A patient with fever and pancytopenia]. 1878 29

The spectrum of hepatic disease in systemic lupus erythematosus (SLE) ranges from subclinical elevations of liver enzymes to rare reports of hepatic arteritis and infarction. Whether liver disease occurs as a consequence of (or is merely coincident with) lupus activity is not known. In our example, an episode of unexplained acute hepatitis occurred in association with pancytopenia. Both responded to increased doses of prednisone and were believed to be manifestations of SLE. The importance of hepatic disease and its contribution to morbidity and mortality in patients with SLE is controversial. Based on a critical review of the literature, we conclude that, although liver disease in SLE occurs more frequently than previously believed, it is usually limited to asymptomatic hepatomegaly and/or isolated elevations in liver function tests.
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PMID:The clinical significance of liver disease in systemic lupus erythematosus. 1907 23

Haemophagocytic syndrome (HPS) or haemophagocytic lymphohistiocytosis (HLH) is a rare disease caused by a dysfunction of cytotoxic T cells and NK cells. This T cell/NK cell dysregulation causes an aberrant cytokine release, resulting in proliferation/activation of histiocytes with subsequent haemophagocytosis. Histiocytic infiltration of the reticuloendothelial system results in hepatomegaly, splenomegaly, lymphadenopathy and pancytopenia ultimately leading to multiple organ dysfunctions. Common clinical features include high fevers despite broad spectrum antimicrobials, maculopapular rash, neurological symptoms, coagulopathy and abnormal liver function tests. Haemophagocytic syndrome can be either primary, i.e. due to an underlying genetic defect or secondary, associated with malignancies, autoimmune diseases (also called macrophage activation syndrome) or infections. Infectious triggers are most commonly due to viral infections mainly of the herpes group, with EBV being the most common cause. HPS can be fatal if untreated. Early recognition of the clinical presentation and laboratory abnormalities associated with HPS and prompt initiation of treatment can be life saving. HPS triggered by viral infections generally does not respond to specific antiviral therapy but may be treated with immunosuppressive/immunomodulatory agents and, in refractory cases, with bone marrow transplantation.
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PMID:Viral infections associated with haemophagocytic syndrome. 2012 50

The response rates and overall survival of hairy cell leukemia has changed remarkably with cladribine. Twenty patients diagnosed as hairy cell leukemia over a 5 year period were evaluated. Median age of the patients was 52.5 years. Splenomegaly was seen in 85% and hepatomegaly in 50% patients. At presentation, pancytopenia was seen in 45% and bicytopenia in 20% patients. Tartrate resistant acid phosphatase was positive in 73.68%. Cladribine was given in 13 patients. Overall survival was 84.6%. Median duration of follow-up was 17 months (4-108 months). Direct Coombs' test positive autoimmune hemolytic anemia was seen in two patients (15.38%) on cladribine therapy who required treatment with steroids.
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PMID:The clinico-hematological profile of hairy cell leukaemia: a single centre experience. 2055 75


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