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)

The clinical presentation and surgical and pathological findings of 46 children with unilateral tonsillar enlargement (UTE; age range 2 to 13 years, mean age 6.5) who underwent tonsillectomy for biopsy purposes between 1975 and 1995 were compared with those of 7 children who received treatment for tonsillar lymphoma (TL; age range 2 to 9 years, mean age 4.8) during the same period. There was no history of rapid tonsillar enlargement in children in the UTE group, and only 20 (43%) were symptomatic. Symptoms included recurrent sore throats in 10 patients (22%), snoring in 5 (11%), nasal obstruction in 4 (9%), and dysphagia in 1 (2%). No children had systemic symptoms or significant cervical lymphadenopathy. In contrast, tonsillar enlargement was observed to occur within a 6-week period in all children with TL, and 6 (86%) children had symptoms at presentation that included dysphagia in 5 (71%), snoring in 3 (43%), night sweats in 2 (29%), and fever and rigors in 2 (29%). Cervical lymphadenopathy greater than 3 cm was present in 6 (86%) children, while 1 child (14%) had hepatosplenomegaly. There was no histopathologic evidence of neoplasia in the UTE group, and a true discrepancy in size between the two tonsils was confirmed in only 21 of 44 (48%) cases. All 7 patients in the TL group had non-Hodgkin's lymphoma. All received chemotherapy, with 5 of the 7 cured and 2 dying of disease. The data suggest that tonsillectomy should be performed for biopsy purposes in UTE where there is a history of progressive enlargement, significant upper aerodigestive tract symptoms, systemic symptoms, suspicious appearance of the tonsil, cervical lymphadenopathy, or hepatosplenomegaly. The diagnosis of TL should also be considered when UTE is present in an immunocompromised child or one with a previous malignancy, when acute tonsillitis is asymmetric and unresponsive to medical treatment, or when rapid bilateral tonsil enlargement occurs. Observation is appropriate management for other cases of UTE.
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PMID:Unilateral tonsillar enlargement and tonsillar lymphoma in children. 1052 79

We studied a cohort of children with the human immunodeficiency virus (HIV) infection in Barbados in order to determine the prevalence of HIV infection, the clinical course including morbidity and mortality and the magnitude of the health care and social problems. Forty-seven children were diagnosed with HIV infection during the study period. The number of HIV infected children increased from 5 during 1981-85, to 14 during 1986-90, and to 21 during the 1991-95 period. The majority (91.5%) of infections resulted from perinatal transmission. Six (12.8%) cases remained asymptomatic and 41 (87.2%) were symptomatic with 19(46.3%) presenting in infancy, while 22 (53.5%) presented post-infancy. The median age at diagnosis (class P-2) was 13 months. Generalized lymphadenopathy (47.5%), hepatosplenomegaly (40.0%), failure to thrive (27.5%), persistent recurrent diarrhoea (15.0%), oral candidiasis (37.5%), Pneumocystis carinii pneumonia (37.5%), lymphoid interstitial pneumonia (12.5%) and progressive neurological disease (10.0%) were common HIV related conditions. Two children developed non-hodgkin's lymphoma. The median age at death for 23 children was 12 months, whereas the median survival after diagnosis was 4 months. Mortality was higher among those diagnosed in infancy (73.7%) as compared to those diagnosed post-infancy (42.8%). Pneumocystis carinii pneumonia was the most common (65.2%) cause of death. Paediatric HIV infection is rising and contributes considerably to infant mortality. In this study, children took longer to be symptomatic when compared to other reports. However, once symptomatic, they died early.
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PMID:HIV infection among children in Barbados. 1078 51

The study aimed at the diagnosis of toxoplasmosis in 73 children with malignancy; 31 with lymphoma (22 with Hodgkin's and 9 with non-Hodgkin's lymphoma) and 42 with leukemia (34 with acute lymphoblastic leukemia and 8 with acute myelogenic leukemia). In positive cases toxoplasmosis was manifested by any of the following; fever, lymph node enlargement, neurological manifestations and/or hepatosplenomegaly. The indirect hemagglutination test (IHA) for toxoplasmosis detected 4 (5.4%) positive cases with malignancy, 2 with Hodgkin's lymphoma, one with non-Hodgkin's lymphoma and one with acute lymphoblastic leukemia. The immunoglobulin M enzyme-linked immunosorbent assay (IgM ELISA) detected only one (1.4%) case with Hodgkin's lymphoma. Immunoglobulin G (IgG) ELISA detected 6 (8.2%) positive cases, 3 with Hodgkin's lymphoma, one with non-Hodgkin's lymphoma and 2 cases with acute lymphoblastic leukemia. Polymerase chain reaction for detection of parasite DNA in blood (PCR) was the most useful in diagnosing toxoplasmosis with malignancy, as it was able to detect 9 (12.3%) positive cases; 5 (6.8%) with Hodgkin's lymphoma, one (1.4%) with non-Hodgkin's lymphoma and 3 (4.1%) with acute lymphoblastic leukemia. No positive toxoplasmosis cases were detected with acute myelogenic leukemia by any of the above methods.
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PMID:Diagnosis of toxoplasmosis in children with malignancy. 1094 13

A 38-year-old male presented with fever and hepatosplenomegaly. Cells that had infiltrated to the bone marrow were consistent immunophenotypically and genotypically with natural killer (NK) cells. Oligoclonal Epstein-Barr virus infection was detected in the bone marrow cells. The patient was diagnosed as a case of aggressive NK cell leukemia/lymphoma. Combined chemotherapy was not effective and death occurred shortly after presentation. Although the karyotype of this case was too complicated to be accurately identified only by G-banding, spectral karyotyping (SKY) analysis not only identified all chromosomal materials of unknown origin, but also detected the cryptic translocation on the apparently normal chromosome. Moreover, SKY analysis identified der(4)t(4;14)(q12;q11.2). The chromosomal band 14q11.2 is a recurring breakpoint in T-cell non-Hodgkin's lymphoma, and is also the locus of the delta chain of the T-cell receptor. To our knowledge, t(4;14)(q12;q11.2) in T-cell or NK-cell malignancies has not been previously reported.
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PMID:Aggressive natural killer cell leukemia/lymphoma: a comprehensive cytogenetic study by spectral karyotyping. 1104 24

The presence of serum cold agglutinin can be the initial presentation of lymphoproliferative diseases. Conditions with persistent cold agglutinins are a spectrum of diseases that vary from benign lymphoproliferation of the "autoimmune-like chronic cold agglutinin disease" to malignant lymphoma. We report a case of a 72-year-old woman who presented with severe anaemia, hepatosplenomegaly and episodes of peripheral haemagglutination precipitated by cold exposure. The haemoglobin was 5.6 g/dL with a cold agglutinin titer of 1:256 at 4 degrees C and 1:8 at room temperature (30 degrees C). The cold agglutinin showed anti-I specificity and kappa light chain restriction. Peripheral blood showed atypical lymphoid cells with a B-cell immunophenotype. Immunoglobulin gene rearrangement study by polymerase chain reaction (PCR) showed an amplified band at 100 bp, consistent with a clonal proliferation of B-lymphocytes. We believe that our patient had cold antibody haemolytic anaemia as the initial presentation of a low-grade non-Hodgkin's lymphoma. The association of cold antibody haemolytic anaemia with low-grade B-cell lymphoma is unusual.
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PMID:Cold agglutinins in low-grade B-cell lymphoma. 1104 70

A case of angiotropic B-cell lymphoma associated with hemophagocytic syndrome (HPS) has been reported. In addition to fever, pancytopenia, hepatosplenomegaly, and lack of lymphadenopathy, unique clinical features, such as syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and pulmonary infarction, were manifested. Both soluble interleukin-2 receptor (sIL-2R) and IL-6 were elevated in the patient's sera in addition to an increase of serum lactate dehydrogenase and ferritin. In contrast, tumor necrosis factor-alpha and interferon-gamma were within normal ranges. Serum antibodies against Epstein-Barr virus and cytomegalovirus showed a past infection pattern. An autopsy examination revealed systemic intravascular proliferation of lymphoma cells with a B-cell phenotype, confirming the diagnosis of angiotropic B-cell lymphoma. Moreover, SIADH was suggested to result from the infiltration of tumor cells into the pituitary gland. Triple association of angiotropic B-cell lymphoma, HPS and SIADH is quite rare. Therefore, the present case seems to be helpful for clarifying the mechanism for HPS of non-Hodgkin's lymphoma with B-cell origin.
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PMID:Angiotropic B-cell lymphoma with hemophagocytic syndrome associated with syndrome of inappropriate secretion of antidiuretic hormone. 1110 Jul 51

A 25-year-old man was admitted to our hospital because of hematuria, anemia and thrombocytopenia. Laboratory examinations revealed an increased number of bone marrow megakaryocytes and an increased level of platelet-associated immunoglobulin G, suggesting immune thrombocytopenia. Computed tomography of the abdomen showed enlargement of the bilateral kidneys with multiple low-density areas, although neither lymphadenopathy nor hepatosplenomegaly was evident. After amelioration of the thrombocytopenia by prednisolone therapy, open renal biopsy was performed and a diagnosis of diffuse large B-cell non-Hodgkin's lymphoma was made. The patient achieved complete remission after CHOP therapy. This was thought to be a rare case of primary renal non-Hodgkin's lymphoma initially presenting as immune thrombocytopenia, which was treated successfully by chemotherapy.
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PMID:[Primary renal non-Hodgkin's lymphoma presenting as immune thrombocytopenia]. 1123 33

Two children with non-Hodgkin's lymphoma (NHL) as the presenting illness of acquired immunodeficiency syndrome (AIDS) are described. There was a delay in diagnosing the underlying AIDS in both cases. In the first case, an 18-month-old boy with stage IV, high-grade,T-cell NHL, the diagnosis of underlying AIDS was suspected only when he developed recurrent and profound opportunistic infection during chemotherapy. The second case, an eight-month-old female infant presented initially with hepatosplenomegaly and thrombocytopenia of undetermined cause. She had progressive abdominal distension and swelling of her right eye one year later due to high grade B-cell NHL. She was later found to be sero-positive for HIV during pre-chemotherapy screening. As the prevalence of HIV infection continues to increase, HIV infection should be considered in the differential diagnoses of childhood hepatosplenomegaly and thrombocytopenia, and as a possible underlying cause of childhood cancer, especially NHL.
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PMID:Acquired immunodeficiency syndrome presenting as childhood non-Hodgkin's lymphoma. 1187 80

Malignant lymphomas have been reported previously to coincide with adenocarcinomas of the stomach and, rarely, the kidney, breast, colon, liver, or lung. Here, we describe the first case to our knowledge of a malignant lymphoma and an extensive disease small cell cancer of the lung. A 60-year-old male was admitted for severe back pain and was found to have multiple enlarged lymph nodes, hepatosplenomegaly, and bilateral pleural effusions. A B-cell non-Hodgkin's lymphoma (NHL) was diagnosed from biopsies of the stomach and liver. Further staging revealed a dense infiltration of the bone marrow by both a small cell lung cancer and a malignant lymphoma. Both tumors responded well to chemotherapy. This unique case report demonstrates that the simultaneous occurrence of small cell lung cancers and malignant lymphomas is extremely rare and may effectively be treated with chemotherapy.
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PMID:Effective treatment of simultaneous small cell lung cancer and B-cell lymphoma. 1200 73

A rare simultaneous occurrence of multicentric Castleman's disease, non-Hodgkin's lymphoma, and Kaposi's sarcoma was diagnosed in a 70-year-old man who presented with fever, polyarthralgia, weight loss, vascular purpura, anemia, generalized lymphadenopathy, and hepatosplenomegaly. He had no risk of HIV infection and serological tests for HIV were negative twice, but a low number of T-cells and a reversed CD4/CD8 ratio were observed. During hospitalization, he developed Kaposi's sarcoma at the right sole. Lymph node biopsies revealed multicentric Castleman's disease together with a large B-cell lymphoma, which showed monotypic IgM-lambda lymphocytes. To our knowledge, this is the first report in which systemic manifestations of all three diseases occurred simultaneously prior to any specific treatment. The altered immune status and human herpesvirus-8 infection might have played a role in the pathogenesis of this occurrence.
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PMID:Multicentric Castleman's disease, non-Hodgkin's lymphoma, and Kaposi's sarcoma: a rare simultaneous occurrence. 1240 98


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