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)

A generalized form of cytomegalic disease in newborns is relatively rare. According to the author's data, only one case of cytomegalia was observed during post-mortem investigations of 1724 newborns and young children. This was a full-term child which was born with jaundice, generalized petechial rash and hepatosplenomegaly. Histological investigations revealed cytomegalic cells in the lungs, liver, kidneys and parotid salivary glands; in the brain there was noted calcification of the walls of the lateral ventricles. In the stroma of organs lymphoid infiltration with proliferation of histiocytes was observed.
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PMID:[A case of cytomegalic disease of the newborn]. 17 8

We observed two sisters with ataxia telangiectasia, one of whom developed an atypical subacute lymphocytic leukemia characterized by atypical lymphocytes and absence of palpable lymphadenopathy or hepatosplenomegaly. The lack of organomegaly in this patient may have been due to the underlying ataxia telangiectasia, which was associated with lymphoid hypoplasia. Cytogenetic studies showed a marker chromosome 14 [t(14q11:14q34)] in both patients. The sister with leukemia had other complex chromosomal aberrations in addition to the marker chromosome 14 that were stable for more than 14 mo before the patient's death from complicating infection. The development of atypical T cell leukemia has not been previously described in ataxia telangiectasia. This case further illustrates the interesting interrelationships amoung immunosuppressed states, development of lymphoid malignancy, and an emerging pattern of a propensity to chromosome 14 abnormalities in various lymphoid malignancies.
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PMID:Atypical lymphoid leukemia in ataxia telangiectasia. 69 87

A patient with acute lymphocytic leukemia of T (thymic-derived) cell origin was successfully treated and was maintained in remission for four months by combined chemotherapy. He died following a seven-week, fulminant course with fever, refractory pancytopenia, and marked hepatosplenomegaly. The autopsy showed lymphoid leukemic infiltration and extensive histiocytic medullary reticulosis in various organs. The possible relations between these two lymphoreticular diseases are discussed.
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PMID:Histiocytic medullary reticulosis in acute lymphocytic leukemia of T cell origin. 108 96

An inappropriate, sustained and absolute lymphocytosis in a young man was investigated using technics which attempted to define the more detailed features of these cells and their T and B subpopulations. The results of the tests for lymphocyte RFC (E) and complement (EAC) rosette formation, immunofluorescence, in vitro phytohemagglutin (PHA) stimulation and 14C-cyclo-phosphamide binding assays, when evaluated in a combined and interrelated fashion, indicated that the lymphocytosis was mainly but not solely, due to an absolute elevation in circulating T lymphocytes. Moreover, an aneuploid cell line with 47 chromosomes, presumably T cells, also was detected in PHA-stimulated cultures. These data, associated with the patient's anemia, lymphocyte-infiltrated bone marrow, hepatosplenomegaly, pharyngeal lymphoid tumor and the clonal proliferation of lymphocytes bearing an abnormal karyotype, furnish evidence that the lymphocytosis is more than an unremitting leukemoid reaction and is potentially neoplastic. The detection of the chromosomal abnormality at this patient's age and point in disease may make him especially unique in that he could be a clinical prototype for other patients with lymphoreticular tumors which remain undetected at their onset.
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PMID:Persistent lymphocytosis with chromosomal evidence of malignancy. 109 66

A 54-year-old man who had been known to have a high prolymphocyte count for four years was admitted to our hospital because of dyspnea in September, 1990. Physical examination revealed skin eruption, lymphadenopathy and hepatosplenomegaly. Chest X-ray demonstrated bilateral pleural effusions. The leukocyte count was 232,900/microliter with 99% lymphoid cells possessing single nucleoli. The cells expressed the phenotype CD2+, CD3-, CD4+, CD7+, and CD8-. Southern blot analysis of DNA from these cells revealed monoclonal rearrangement of T-cell receptor beta-chain genes. Anti-human T-cell lymphotropic virus type 1 (HTLV-1) antibody and HTLV-1 proviral DNA were not detected. A biopsy specimen from the skin lesions showed infiltration of the leukemic cells which were positive for anti-MT1 antibody. Histological finding of the axillary lymph node was malignant lymphoma, diffuse, medium-sized, T-cell type. Combination chemotherapy resulted in the improvement of skin eruption, lymphadenopathy, hepatosplenomegaly and pleural effusions, although his prolymphocyte count increased to 910,000/microliters. He died of cerebral bleeding in July, 1991. We diagnosed this case as T-cell prolymphocytic leukemia, observed for five years.
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PMID:[A case of T-cell prolymphocytic leukemia]. 143 23

A 60-year-old man was admitted with general fatigue and jaundice of one year's duration in February, 1981. The hemoglobin (Hb) was 11.4 g/dl and reticulocytes were 1.7%. A diagnosis of chronic cold agglutinin disease (CCAD) was made from the presence of cold agglutinin (CA) 1:2,048, increased serum IgM 267 mg/dl and indirect bilirubin 1.4 mg/dl. His Hb was approximately 11 g/dl in summer and 9 g/dl in winter for the subsequent ten years without therapy. In July, 1990, he was readmitted because of exacerbation of anemia and hepatosplenomegaly. The Hb was 4.6 g/dl, indirect bilirubin 3. 1 mg/dl, CA titer 1:232,144 and reticulocytes were 20%. Serum IgM was 1,065 mg/dl, and immunoelectrophoresis showed IgM-kappa M-protein. Peripheral blood lymphoid cells expressed surface membrane immunoglobulin (SmIg) M and kappa. The bone marrow showed an increased number of lymphoid cells which also expressed SmIg M and kappa. These findings were compatible with those of the features of primary macroglobulinemia (PMG). The M-2 protocol resulted in decrease in serum IgM and CA, but he died of heart failure in February, 1991. The relationship between CCAD and PMG in relation to the pathogenesis was discussed.
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PMID:[Chronic cold agglutinin disease terminating in primary macroglobulinemia after a 10 year history]. 146 90

An adult T cell leukemia (ATL) accompanied with Isospora belli infection was described. A 65-year-old male was admitted to our hospital because of a two month history of watery diarrhea. On admission, physical examination showed slight pallor but no detectable superficial lymphadenopathies. Hepatosplenomegaly was not observed. Laboratory examination revealed a leukocyte count 5,500/microliters with 10% abnormal lymphoid cells. A majority of the abnormal lymphoid cells expressed both CD 4 and CD 8 antigens. The patient was diagnosed as chronic ATL, since anti-HTLV-1 antibody in his serum and monoclonal integration of HTLV-1 proviral DNA in his peripheral mononuclear cells were detected. Isospora belli was found in his feces thereafter, and trimethoprim/sulfamethoxazole was effective for diarrhea. In Japan, there have been only 9 reported cases of lymphoproliferative disorders (including five ATL patients) accompanied with Isospora belli infection. From the descriptions in those reports, these 9 cases might all be ATL patients.
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PMID:[Isospora belli infection in a patient with adult T-cell leukemia]. 163 21

Ten cases of pediatric fulminant hemophagocytic syndrome, encountered between 1986 and 1989, are described. They occurred in the summer, and the patients presented with fever, jaundice, hepatosplenomegaly, pancytopenia, coagulopathy, and abnormal liver function. Bone marrow studies revealed infiltration by atypical T-lymphoid cells, rare B immunoblasts, and mature histiocytes with hemophagocytosis. Initially, histiocytic medullary reticulosis was suspected in six cases. The clinical course was characterized by rapid deterioration, with a mean period of 16 days from onset of fever to death. The main causes of death were coagulopathy with multiple organ failure and opportunistic infection. In seven of eight cases studied by serologic assay and Southern blot hybridization, acute or active Epstein-Barr virus (EBV) infection was documented. It is suggested that an atypical or fulminant form of primary EBV infection distinct from classic infectious mononucleosis was prevalent in previously healthy children in Taiwan. Younger age involvement and seasonal clustering were characteristic of the disorder described.
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PMID:Fulminant childhood hemophagocytic syndrome mimicking histiocytic medullary reticulosis. An atypical form of Epstein-Barr virus infection. 165 May 32

Adult T-cell leukemia/lymphoma (ATLL) is an HTLV-I associated lymphoid malignancy frequently seen in Japan. Abdominal involvement in 40 patients with ATLL were assessed by ultrasonography and the findings seen in four clinical types, acute, chronic, lymphoma and smoldering, were compared. Splenomegaly was frequently found in the cases of acute and lymphoma types, and the sizes of the spleens measured by ultrasonography correlated well with the disease activity. Hepatomegaly was also found more frequently in acute and lymphoma types, and hepatosplenomegaly was proved to be due to the infiltration by ATL cells. Nodular lesions in spleen and liver and abdominal lymph node swelling were also found frequently in the lymphoma type but rarely in the other types. Ascites, pleural effusion, and pericardial effusion were found in the active stage of acute and lymphoma types. Ultransonography also could detect findings associated with therapies. Thus, ultrasonography studies were found to be very useful for assessing the clinical classification, examining various pathological conditions associated with ATLL, and monitoring the disease activity.
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PMID:Assessment of abdominal involvement of adult T-cell leukemia/lymphoma by ultrasonography: comparison among four clinical types. 165 79

Ten leukemia cases with mixed phenotype were investigated in terms of clinical characteristics and cellular origin. Three patients were infants and six patients were older children. Six of them had a high leukocyte count and a mediastinal mass was found in three cases. All but one showed hepatosplenomegaly and/or lymphoadenopathy. In spite of intensive chemotherapy, most of them responded poorly. Cytochemical analysis of their leukemic cells revealed a low percentage of positivity for myeloperoxidase reactivity (less than 25%) in two cases and electron microscopic platelet peroxidase reactivity was found in one of three analyzed cases. Phenotypically, these cells all expressed CD7, and other T-lineage-associated, B-lineage-associated, and/or myeloid-associated antigens were also detected to some extent. In addition, three cases expressed CD41 and one case expressed CD56. The T-cell receptor (TCR) genes and immunoglobulin gene were in the germline configuration in seven cases. In three rearranged cases, two showed only the TCR-delta gene rearrangement, and one had both TCR-gamma and delta gene rearrangements. Cell culture studies with 12-0-tetradecanoyl-phorbol-13-acetate (TPA) revealed differentiation to the T-lineage in two cases and to a myeloid lineage in one case. Megakaryocytic differentiation was detected in two cases in culture without TPA. These results suggest that the cells from these cases arose from stem cells capable of both lymphoid and nonlymphoid differentiation. Although the cells were heterogeneous with regard to their potency of differentiation, they have similar clinical characteristics. Because of poor prognosis, it is important to identify this type of leukemia, and allogenic or autologous bone marrow transplantation should be considered.
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PMID:Clinical significance of CD7-positive stem cell leukemia. A distinct subtype of mixed lineage leukemia. 171 22


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