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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A considerable reduction of hepatosplenomegaly and leucocytosis in leukemic rats of the Brown Norway Myeloid Leukemia (BNML) can be achieved by exposure to 50% nitrous oxide/50% oxygen. In this study the differential antiproliferative effect of nitrous oxide, inactivating vitamin B12, on normal and leukemic hemopoiesis was investigated in this rat model. Rats injected with leukemic cells and exposed to nitrous oxide for 10 days showed 30% reduction of hepatosplenomegaly and 50% reduction of leukocytosis. Similarly treated healthy rats showed no signs of impaired hemopoiesis as measured by peripheral blood parameters. Clonogenic assays of erythroid and myeloid progenitors from both healthy and leukemic rats revealed that exposure to nitrous oxide did not suppress normal bone marrow functioning. On the contrary, the reduction of leukemic proliferation by nitrous oxide retarded the leukemic infiltration of the bone marrow compartment.
Cancer Lett 1989 May
PMID:Nitrous oxide selectively reduces the proliferation of the malignant cells in experimental rat leukemia. 273 Nov 56

Malignant histiocytosis (MH: sinusoidal hematolymphoid malignancy) is a rare lymphoreticular disorder characterized by an aggressive clinical course in younger patients with weight loss, hepatosplenomegaly, generalized lymphadenopathy and pancytopenia. Five cases of MH were identified over a five-year period (1982 to 1987) at Indiana University Medical Center. The patients' ages were 12, 16, 20, 30 and 57 years; all presented with classic clinical symptoms. Four cases were diagnosed by fine needle aspiration biopsy; one case was diagnosed by the examination of ascitic fluid. All patients had confirmatory surgical biopsies. The salient cytologic features of MH included (1) a lack of background lymphoglandular bodies, (2) a population of variably sized dyscohesive cells, (3) a component of large bizarre cells with abundant eccentric, deep-blue cytoplasm on Wright-Giemsa-stained preparations, (4) prominent cytoplasmic vacuolization and (5) inconspicuous erythrophagocytosis occurring in the most benign-appearing histiocytic cells. Ancillary studies on cytologic and histologic material (immunostaining for alpha-1-antichymotrypsin and alpha-1-antitrypsin and staining for nonspecific esterases) confirmed the histiocytic nature of the malignant cells. Recognition of the distinctive morphology of MH and the performance of ancillary studies on cytologic preparations should facilitate the rapid diagnosis and early treatment of this aggressive disease.
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PMID:The cytology of malignant histiocytosis (sinusoidal hematolymphoid malignancy). 278 72

Three patients with peripheral T-cell lymphoma presenting with pyrexia, wasting, hepatosplenomegaly and pancytopenia in the absence of myelophthisic lymphomatous involvement are reported. Early in the course of the disease when there was no significant lymphadenopathy, these cases created enormous diagnostic confusion. Although the clinical features were suggestive of malignant histiocytosis (MH), marrow findings showed phagocytic histiocytes which did not appear atypical, and the criteria for diagnosis of MH could not be satisfied. Lymph node enlargement was detected only after 14, 5, and 8 weeks from the onset of symptoms, and the diagnosis of T-lymphoma was then made on lymph node biopsies. Treatment with multiple agent chemotherapy was attempted. Two patients died 3 days and 11 weeks after treatment was started and the third was lost to follow-up. In contrast with most of the cases reported in the literature, our cases show that a reactive hemophagocytic syndrome can be an early and prominent manifestation of an underlying T-cell lymphoma. Differentiation from other causes of hemophagocytic syndrome can be difficult and lack of histological proof of malignancy in the initial stage often delays definitive diagnosis and treatment.
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PMID:Peripheral T-cell lymphoma presenting as hemophagocytic syndrome. 278 35

We performed molecular studies in five cases of Philadelphia (Ph) translocation-negative chronic myelogenous leukemia (CML). Among the five, one case showed a 7q - anomaly; the remaining four had normal karyotypes. The 5' or 3' breakpoint cluster region (bcr) DNA probes detected rearrangements in two of the five cases. The two cases with bcr rearrangement showed clinical and hematologic manifestations similar to those with Ph-positive CML; for example, basophilia in the peripheral blood and marked hepatosplenomegaly. On the other hand, the three Ph-negative CML cases without bcr rearrangement manifested somewhat different clinical manifestation; that is, they did not respond to busulfan therapy in the chronic phase. Our observations suggest a heterogeneity in Ph-negative CML with at least two subtypes: one with a rearranged bcr gene showing clinical and hematologic features akin to those of CML with a Ph translocation, and the other without such a rearrangement and with a somewhat different clinical feature. Furthermore, the present data point to the possibility of the existence of Ph-negative CML without bcr rearrangement.
Cancer Genet Cytogenet 1988 Jul 01
PMID:Molecular and clinical investigations in Philadelphia chromosome-negative chronic myelogenous leukemia. 283 55

A girl with common acute lymphoblastic leukemia associated with t(4;11)(q21;q23) is presented. The physical examination revealed hepatosplenomegaly, and the hematologic analysis showed a high count of white cells. Chemotherapy induced a complete remission (CR), and the patient is still in CR (8 months). At the present time, 43 cases (including ours) of acute leukemia with t(4;11) have been published. This disease more often affects children and women than adult men and is characterized by a high blood white cell count and a short survival. The t(4;11)-associated acute leukemia is a heterogeneous group, including acute undifferentiated leukemia, acute undifferentiated lymphoblastic leukemia, and "common" acute lymphoblastic leukemia.
Cancer Genet Cytogenet 1986 Mar 01
PMID:4;11 translocation-associated acute leukemia: a comprehensive analysis. 293 46

Twenty four patients with angio-immunoblastic lymphadenopathy (AILD) presenting between 1974 and 1985 have been reviewed. Clinical features at presentation included rash, fever, lymphadenopathy and hepatosplenomegaly in 75% of patients. Polyclonal hypergammaglobulinaemia was seen in 19/20 patients; 5 had normal immunoglobulin levels. Combination chemotherapy with MVPP was the optimal treatment with 6/7 patients achieving complete remission. Duration of remission ranged from 9 months to 4 years and was significantly longer in patients achieving complete as opposed to partial remission. In 6 patients phenotype studies were performed on single cell suspensions and immunoperoxidase studies on frozen sections of 7 lymph nodes. There was a reversal of the normal T suppressor/helper cell ratio with a predominance of T suppressor cells. Loss of normal B follicles was observed histologically in all except one lymph node. Germline configuration of the beta B-chain of the T cell receptor was observed in lymph nodes of 4 patients with AILD, and a rearranged T cell receptor was observed in 1 patient in whom a second lymph node biopsy had shown alteration of the histological picture to that of T-zone lymphoma. Frozen sera of 15 patients were screened for antibodies to HTLV I and III and were found to be negative.
Br J Cancer 1987 Apr
PMID:Angio-immunoblastic lymphadenopathy: a clinical, immunological and molecular study. 295 85

To date, the acquired immunodeficiency syndrome (AIDS) has been identified in over 50 children in the US, including those with associated hemophilia, high-risk environmental factors (Haitian background, parental intravenous drug abuse, or prostitution), and blood transfusions. The evaluation of an infant or young child in whom AIDS is suspected requires exclusion of congenital disorders of immune function. A specific test is not currently available, but inclusion criteria for childhood AIDS have been developed. The diseases accepted as indicative of underlying cellular immunodeficiency children are the same as those used in defining AIDS in adults, with the exclusion of congenital infections such as toxoplasmosis or herpes simplex virus infection in the 1st month of life or cytomegalovirus infection in the 1st 6 months of life. Specific conditions that must be excluded in children are primary immunodeficiency diseases (e.g., DiGeorge syndrome, Wiskott-Aldrich syndrome, ataxia-telangiectasia, neutrophil function abnormality) and secondary immuno-deficiency associated with immunosuppressive therapy, lymphoreticular malignancy, or starvation. Almost all young children with AIDS have hepatosplenomegaly, interstitial pneumonitis, and poor growth. The average age of 36 US child AIDS victims studied in detail was 5 months at presentation with findings suggestive of severe immunodeficiency. Mucocutaneous candidiasis was present in 75% of these 36 children, and Pneumocystis carinii and cytomegalovirus were each isolated from 30% of cases. Normal T4:T8 ratios occur in about 15% of pediatric AIDS cases. Laboratory evidence of polyclonal hypergammaglobulinemia generally supports the AIDS diagnosis. Recurrent infection and malnutrition are major problems in the clinical management of child AIDS patients.
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PMID:Acquired immune deficiency syndrome in childhood. 298 8

The clinical and pathologic findings of four patients with Chediak-Higashi syndrome in the accelerated phase were studied in order to clarify the nature of this enigmatic process. Fever, lymphadenopathy, hepatosplenomegaly, and cytopenias were present in every patient. All cases demonstrated extensive parenchymal infiltrates in many organs composed of benign-appearing histiocytes manifesting hemophagocytosis accompanied by lymphocytes and plasma cells. Studies in one patient suggested a viral etiology with the findings of a low blood lymphocyte OKT4 to OKT8 ratio, acquired loss of lymphocyte response to mitogens, the presence of Epstein-Barr virus genome in the mononuclear cells of lymph node, blood, and bone marrow, and possible clinical responses to acyclovir. It is concluded that the accelerated phase of Chediak-Higashi syndrome may be the clinicopathologic expression of the virus-associated hemophagocytic syndrome.
Cancer 1985 Aug 01
PMID:The accelerated phase of Chediak-Higashi syndrome. An expression of the virus-associated hemophagocytic syndrome? 298 47

Human T-cell leukemia/lymphoma virus I can transform mature T-lymphocytes in vitro and is associated with the human T-cell cancer, adult T-cell leukemia/lymphoma. Adult T-cell leukemia/lymphoma is a distinct clinicopathological entity associated with leukemia, lymphadenopathy, hepatosplenomegaly, skin lesions, hypercalcemia, and lytic bone lesions. Although morphologically diverse it pursues an aggressive clinical course. Human T-cell leukemia/lymphoma virus III is associated with acquired immunodeficiency syndrome, which in its early stages shows follicular lymphoid hyperplasia; however, lymphoid atrophy is progressive and ultimately results in virtually total lymphoid depletion of lymph nodes. Patients with human T-cell leukemia/lymphoma virus III infections appear to have an increased risk of high-grade B-cell lymphomas and perhaps Hodgkin's disease.
Cancer Res 1985 Sep
PMID:Lymph node pathology of HTLV and HTLV-associated neoplasms. 299 Jul 5

A patient with osteosclerotic myeloma and POEMS syndrome, unresponsive to pulse prednisone and melphalan therapy, was admitted to the hospital for a trial of plasma exchange therapy. The presentation included IgG lambda monoclonal gammopathy, peripheral neuropathy, hepatosplenomegaly, hyperpigmentation and thickening of the skin, edema, and tense ascites. Laboratory tests confirmed hypothyroidism, hypogonadism, and adrenal insufficiency. Six exchange procedures failed to affect the clinical course, and the patient died. Greater-than-one-plasma-volume exchanges (patient's measured plasma volume, 2,703 cc) were performed. When IgG and cholesterol removal were compared to the predicted removal, based on the volume of plasma removed, significantly less reduction in concentration than predicted was measured. IgG concentrations increased postapheresis and, at 2 weeks, three-fourths of the removed IgG had reaccumulated. A reduced efficiency of removal of both IgG and cholesterol can be explained by postulating increased vascular permeability with free exchange of soluble substances from one compartment to another. If an abnormal product is produced by the disease and is responsible for the clinical syndrome, a more intensive schedule of plasma exchange therapy may be needed to achieve a sustained depletion of the responsible soluble substance. Alternatively, neither increased vascular permeability or the clinical manifestations are responsive to removal of a soluble substance or are caused by a soluble substance produced by the malignancy.
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PMID:Therapeutic trial of plasma exchange in osteosclerotic myeloma associated with the POEMS syndrome. 299 55


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