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)

An unusual case of hepatosplenic gamma delta T-cell lymphoma with leukemic phase in a 39-year-old woman is reported. At the first presentation she had splenomegaly and pancytopenia diagnosed as hypersplenia treated by splenectomy. Subsequently, she developed hepatomegaly and progressive neoplastic lymphocytosis. The bone marrow showed a sinusoidal infiltrate of medium-sized cells. Flowcytometric analysis of peripheral blood mononuclear cells demonstrated expression of CD3, CD7, CD16, CD56 antigens and T-cell receptor gamma delta. A monoclonal TCR gamma- and beta-chain gene rearrangement were detected by polymerase chain reaction. The patient was treated by traditional chemotherapy and alpha-interferon, unsuccessfully. Therefore, 2-chlorodeoxyadenosine was introduced resulting in a complete remission for 6 months. The reported case demonstrates the usefulness of 2-chlorodeoxyadenosine in treatment of hepatosplenic gamma delta T-cell lymphoma.
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PMID:Hepatosplenic gamma delta T-cell lymphoma with leukemic phase successfully treated with 2-chlorodeoxyadenosine. 1280 28

A 61-year-old man was found to have mild lymphocytosis during a medical checkup and was referred to our hospital in 1993. Physical examination showed a mild hepatomegaly, and bone marrow examination revealed the proliferation of monoclonal mature B cells. He was diagnosed with B-CLL. Chlorambucil and then fludarabine were effective initially. However, large transformed cells started to increase in 2000, and the increment became uncontrollable and platelet transfusion dependence developed. He was admitted to our hospital in October 2001. He intermittently received a total of 32 administrations of rituximab. The total dose was 15, 500 mg. The toxicity was tolerable and he became transfusion independent for 6 months. However, systemic lymphoadenopathy and hepatomegaly did not respond to the drug. We conclude that rituximab treatment is useful for refractory B-CLL because of its excellent safety.
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PMID:[Drug-resistant B cell chronic lymphocytic leukemia safely treated with rituximab to control leukemic cell increment and platelet transfusion dependence]. 1499 66

Mantle-cell lymphoma (MCL) is a B-cell malignancy with distinct molecular genetics and pathological features. Peripheral blood involvement has been reported with variable frequency, but information on the natural history of cases presenting with leukemia is lacking. This study aimed to determine the clinical and prognostic features of such cases. We studied clinical features, tumor characteristics, prognostic factors and outcome in 58 patients with leukemic presentation of MCL. Diagnosis was based on morphology, immunophenotype, presence of t(11;14), histology and cyclin D1 expression. The median age was 62 years and male:female 2.4:1. Presenting features included splenomegaly (74%), lymphadenopathy (45%), hepatomegaly (17%) and, in a minority, gastro-intestinal involvement or involvement of Waldeyer's ring; 10% had lymphocytosis alone. Six patients developed central nervous system disease. Median lymphocyte count was 58 x 10(9)/l, 55% had anemia and 17% had thrombocytopenia. Morphology of peripheral blood showed small-cell MCL in 15% of cases, typical MCL in 46% and blastoid MCL in 39%. Immunological markers showed a typical phenotype (CD5+ CD23 -) in 68%, and atypical phenotypes, CD5- CD23- in 17% or CD5+ CD23+ in 15%. CLL scores were 0, 1 or 2 in 96%. Median overall survival was 36 months. Good response to first-line treatment (P = 0.0008) and splenomegaly (P = 0.03) were favorable prognostic factors, while other features including morphology and CD38 expression had no impact on survival or treatment response. This analysis demonstrates that except for splenomegaly, survival of MCL patients presenting with leukemia is not significantly influenced by clinical or tumor characteristics. Splenectomy is a useful treatment option in this group of patients.
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PMID:The leukemic presentation of mantle-cell lymphoma: disease features and prognostic factors in 58 patients. 1537 Feb 45

We analyzed the clinical features and prognostic factors for transformation of immunoglobulin Mrelated disorders (IgM-RDs) to malignant lymphoproliferative disease (MLD) in 83 patients with IgM-RDs. We studied 19 patients with type I cryoglobulinemias, 56 patients with type II cryoglobulinemias, 5 patients with peripheral neuropathies (PNs), and 3 patients with idiopathic thrombocytopenic purpuras. Fourteen patients with cryoglobulinemias presented with mild to moderate hepatomegaly with or without splenomegaly. Fourteen patients with type II cryoglobulinemias had arthralgias and/or vascular purpura (12 receiving corticosteroids), and 7 presented with PN. These latter patients and those with PNs without cryoglobulinemia were treated with steroids, cyclophosphamide, or polychemotherapy with/without plasma-exchange. Cumulative probability of evolution to MLD at 5 years was 15% (95% CI; 5%-25%). At a median of 62 months (12-195 months), 8 cases of IgM-RDs (8.4%) evolved to overt Waldenstrom's macroglobulinemia (n = 6), 1 case to non-Hodgkin's lymphoma, and 1 case to B-cell chronic lymphocytic leukemia. At univariate analysis, male sex (P = 0.02), IgM level > or = 3 g/dL (P < 0.0001), detectable Bence Jones proteinuria (P = 0.0005), lymphocytosis (P = 0.049), and high erythrocyte sedimentation rate (P = 0.003) significantly correlated with the evolution risk. Age, blood cell counts, b2-microglobulin level, degree of marrow lymphoplasmacytic infiltration, type of cryoglobulinemia, and hepatitis C virus positivity did not correlate with transformation. Although IgM-RDs represent a distinct clinical entity frequently requiring treatment in view of the IgM-related symptoms, their evolution probability and prognostic factors for malignant transformation seem to widely overlap those described for asymptomatic IgM monoclonal gammopathies.
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PMID:Clinical characteristics and outcome of immunoglobulin M-related disorders. 1579 61

A 73-year-old woman was presented with altered mental status and disorientation. She was diabetic and hypertensive, and she had experienced an ischemic cerebrovascular accident 3 years ago. Physical examination revealed the findings of chronic obstructive pulmonary disease, cor pulmonale and congestive heart failure. Hepatomegaly, splenomegaly and ascites were found and might be associated with postsinusoidal portal hypertension secondary to congestive heart failure. Laboratory tests showed uremia, lymphocytosis and thrombocytopenia. Neurologic findings were related with uremia and hypoxia. Multiple pathologic lymphadenopathies were seen in abdominal ultrasonography and thoracic computed tomography. Bone marrow histology indicated chronic lymphocytic leukaemia (CLL). The reason for acute renal failure was leukaemic infiltration of the kidneys due to CLL that was shown with renal biopsy. Blood urea nitrogen (BUN) and serum creatinine responded well to cyclophosphamide and methyl prednisolone treatment. In CLL, direct renal involvement is frequently seen in autopsy studies especially in advanced disease, however, renal failure due to leukaemic infiltration is extremely rare.
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PMID:Acute renal failure due to leukaemic infiltration in chronic lymphocytic leukaemia: case report. 1587 23

A previously healthy 11-month-old girl presented with fever and rash for 6 days. Physical examination revealed an irritable infant with a high fever, injected conjunctivae, red cracked lips, posterior auricular lymphadenopathy, hepatomegaly, generalized erythematous maculopapular rash and petechial hemorrhage on trunk, face and extremities. Complete blood count showed atypical lymphocytosis and thrombocytopenia. Dengue infection was initially diagnosed. The persistent fever and clinical manifestations of Kawasaki disease (KD) were observed on day 8 with high erythrocyte sedimentation rate (56 mm/hr). Treatment of KD included intravenous immunoglobulin on day 9 of the illness. Desquamation of the fingers was found on day 15 of the illness. Ectasia of left coronary artery with small aneurysmal dilatation was detected by echocardiography on day 15 of the illness. Hemagglutination-inhibition test and enzyme-linked immunosorbent assay for dengue virus eventually showed a four-fold rising. According to the literature review, this is the second reported case of dengue infection concomitant with KD. The natural course of each disease may be modified and causes some difficulties in diagnosis and management.
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PMID:Concomitant dengue infection and Kawasaki disease in an infant: a case report and literature review. 1596 58

20 children, diagnosed with scrub typhus who attended Chiang Rai Regional Hospital during a period of 6 months from June 2003 to December 2003, were studied prospectively. All cases were serologically proved to be scrub typhus by using Dipstick or indirect immunofluorescent antibody (IFA) technique. The most common clinical feature was eschar (75%). Others included hepatomegaly (65%), cough (60%), lymphadenopathy (40%), tachypnea (35%), constipation (25%), abdominal pain (20%), edema (20%), splenomegaly (15%), vomiting (15%), rash (15%) and petichia (5%) respectively. Chest radiography showed abnormalities in 85% with mostly bilateral interstitial infiltrations. Elevated of SGOT: SGPT were detected in 18 (90%) and 15 (75%) cases. Hypoalbuminemia was detected in 12 (60%) cases. Complete blood count showed PMN leukocytosis (> 60%) in 12 (60%) cases, lymphocytosis (> 40%) and atypical lymphocytosis (> 5%) in 1 (5%) case each and thrombocytopenia in 16 (80%) cases. The Weil-Felix test was positive in 1 (5%) case. Complications were pneumonia with or without pulmonary edema, meningitis and shock. Chloramphenicol and doxycycline were successfully treated and roxithromycin was not effective.
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PMID:Clinical study of 20 children with scrub typhus at Chiang Rai Regional Hospital. 1651 87

HCL typically presents in middle-aged men, and is characterized by splenomegaly and cytopenias. Hepatomegaly may be present, but it usually is not a salient feature. Peripheral adenopathy is uncommon. Other organ manifestations occur, but are unusual. patients are now presenting with a less tumor burden, as a result of earlier diagnosis. Leukocytosis/lymphocytosis should suggest HCl variant. Infectious complications, which were common in the past and the major cause of death, have become rare in the era of purine analog therapy. Whether there is a true increased risk for second malignancies remains controversial.
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PMID:Clinical presentations and complications of hairy cell leukemia. 1699 Jan 7

Pathological findings in the liver sinusoids are mostly caused by extrahepatic or systemic diseases. Unclear fever, hepatosplenomegaly, portal hypertension or a mild elevation of liver enzymes are reasons for a liver biopsy leading to path-breaking diagnoses. Reactive intrasinusoidal lymphocytosis, especially with Epstein-Barr virus infections, has to be differentiated from predominantly intrasinusoidal lymphoproliferative malignancies. Intrasinusoidal megakaryocytes can be the first sign of a myeloproliferative or myelodestructive disease. Intrasinusoidal carcinosis and melanomatosis might present radiologically without tumor lesions and are easily overlooked histologically, in particular, if the critical cells have a similar size to hepatocytes. This also applies for intrasinusoidal storing macrophages. Gaucher's disease type I, and some other subtypes of inborn storage diseases might present for the first time in adulthood by hepatomegaly and Kupffer cell hypertrophy. Accompanying perisinusoidal fibrosis and immunohistochemical staining (CD68) can help to detect the large pale intrasinusoidal macrophages. In immunocompromized patients with fever, particular attention must be paid to intracellular agents, especially atypical mycobacteria and yeasts in non-granulomatous nested or dispersed Kupffer cells. Leishmaniasis with amastigotes in macrophages is accompanied by reactive sinusoidal plasmocytosis.
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PMID:[Pathology along the liver sinusoids: intrasinusoidal findings]. 1821 Jan 15

Most cases of acute acquired toxoplasmosis (AAT) are oligosymptomatic and self-limited. Therefore, these infections rarely indicate treatment. Prospective studies of AAT patients are rare in the medical literature. The frequency of systemic manifestations has not been sufficiently studied. In order to search for risks factors for systemic and ocular involvement, 37 patients were submitted to a diagnostic investigative protocol. The most frequent findings were lymph node enlargement (94.6%), asthenia (86.5%), headache (70.3%), fever (67.6%) and weight loss (62.2%). Hepatomegaly and/or splenomegaly were present in 21.6% of cases (8/37). Liver transaminases were elevated in 11 patients (29.7%) and lactic dehydrogenase in 17 patients (45.9%). Anaemia was found in four patients (10.8%), leucopoenia in six patients (16.2%), lymphocytosis in 14 patients (37.8%) and thrombocytopenia in one patient (2.7%). Fundoscopic examination revealed retinochoroiditis in four patients (10.8%). No statistical association was found between any one morbidity and retinochoroiditis. Nevertheless, a significant association was found between the presence of more than eight morbidity features at evaluation and long-lasting disease. An ideal diagnostic protocol for AAT would include evidence of systemic involvement. Such a protocol could be used when planning treatment.
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PMID:Acute acquired toxoplasmosis: clinical-laboratorial aspects and ophthalmologic evaluation in a cohort of immunocompetent patients. 1943 Jun 71


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