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)

Forty-seven consecutive patients with chronic lymphocytic leukemia (CLL) followed in the Clinica Medica and the Sezione di Ematologia of Ferrara from January 1966 to June 1979 were classified according to the staging system of Rai and to age, sex and the occurrence of enlarged spleen or liver or both. Analysis of actuarial survival curves revealed the following: 1) the method of staging proposed by Rai was confirmed to be a reliable predictor for prognosis of CLL; 2( neither age nor sex do appear to be prognostic factor; 3) the enlarged liver did not appear to make worse the prognosis of CLL, probably by hepatomegalies due to other aetiologies. Furthermore the bound of peripheral lymphocytosis of 15,000/mm3, also according to other authors, seems to be too high: numerous cases of undoubted CLL with smaller lymphocytosis ae left out of this and other statistics published on CLL.
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PMID:[Prognosis of chronic lymphatic leukemia. Retrospective study of the survival period 47 patients]. 720 44

We report 26 elderly patients (median age 68.3 years) who met diagnostic criteria for B-cell chronic lymphocytic leukaemia (B-CLL) but whose lymphocytes lacked CD5 expression. Haematological and clinical features of this CD5- series were compared with those of 333 CD5+ B-CLL patients from the same institute. No significant differences were observed regarding peripheral blood (PB) and bone marrow (BM) lymphocytosis, Hb level, platelet count, incidence of adenomegaly, hepatomegaly or splenomegaly or diffuse BM pattern. Due to an absence of nodal enlargements or to general clinical condition, lymph node biopsy was performed in only three patients, while spleen histology was examined in two cases following splenectomy. All histological results confirmed the clinical diagnosis of CLL. The distribution of the CD5- subjects according to the different staging categories proposed by Rai, Binet and Mandelli was similar to that of CD5+ subjects. Ten patients received standard chemotherapy with Chlorambucil (CHL) and Prednisone (PDN). All achieved partial remission, although one of these patients later died of disease progression; 80 months after diagnosis. We conclude that rare cases of CD5- lymphocytosis fulfilling all criteria for B-CLL may occur. Haematological and clinical features at presentation and the response to conventional treatment with Chlorambucil support our hypothesis of considering this disease as a less frequent subgroup of B-CLL.
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PMID:CD5 negative lymphocytosis mimicking typical B-chronic lymphocytic leukaemia. Description of 26 cases. 769 97

A series of 20 patients with granular lymphocyte proliferative disorders (GLPD) is reported. The criterion of inclusion was presence of persistent (> or = 6 months) granular lymphocytosis in the absence of any causative illness. Diagnoses made upon analytical control in half the patients of splenomegaly (25%) and hepatomegaly (25%) were infrequent. Clinical course was nonprogressive in 17/20 patients, but two developed high-grade NHL several years later and one showed progressive disease. Actuarial probability of survival at 5 years was 85%. Granular lymphocyte morphology was relatively homogeneous, and peripheral blood counts were preserved in the most patients. Bone marrow lymphocytic infiltration was low, as assessed by bone marrow aspiration and/or biopsy. Eosinophilia was an outstanding feature in eight cases. Ultrastructurally, all cases showed parallel tubular arrays; cytoplasmic granules and numerous short microvilli were noticed. The lymphoid phenotype was heterogeneous, the most common being CD2+CD3+CD4-CD8+, but six patients (30%) were CD4+ with variable expression of natural killer-associated antigens. Chromosomal analysis was abnormal in 4/10 patients [trisomy 19, t(5;6); inv(14) and inv(10)]. The study of beta-chain of the T-cell receptor revealed clonal rearrangements in 14 (78%), restricted to CD3+ patients (92%). In vitro culture of myeloid precursors showed decreased CFU-GM in 5/6 patients. Virological studies for HTLV-I and II were negative. In conclusion, the presence of a clonal proliferation was not correlated with the clinical course or an associated disease.
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PMID:Granular lymphocyte proliferative disorders: a multicenter study of 20 cases. 791 27

Twenty-six cases of adult T-cell leukemia/lymphoma (ATLL) were identified between 1983 and 1991 in Martinique (French West Indies). There were 14 men and 12 women, all of mixed racial descent and born in Martinique. Their ages ranged from 23 to 95 years. The main clinical and laboratory features at initial presentation were peripheral lymphadenopathy (22 cases), hepatomegaly (11 cases), splenomegaly (10 cases), cutaneous lesions (12 cases), hypercalcemia (16 cases), refractory infection by Strongyloides stercoralis (12 cases), and pre-existing autoimmune disorders (4 cases). All patients had absolute lymphocytosis with circulating pleomorphic abnormal lymphocytes. The prognosis was poor, with most patients (20 cases) surviving for less than 6 months. Although the overall clinicopathologic features of ATLL in this series are similar to those described in previous reports, we observed three additional points of interest: a high association with Strongyloides infection, an increased incidence of tropical spastic paresis/HTLV-1 associated myelopathy (TSP/HAM) among the relatives of the patients (5 cases), and the presence of prior collagen vascular diseases.
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PMID:Adult T-cell leukemia-lymphoma: a clinico-pathologic study of twenty-six patients from Martinique. 811 52

Twenty-nine juvenile, captive-reared African black-footed penguins (Spheniscus demersus) were hematologically monitored every 2 wk over the period of 24 wk during their first outdoor exposure. Blood samples taken from the penguins were screened for 12 blood evaluation parameters. Parasitemic penguins were medically treated. Eighteen birds (62.1%) experienced naturally acquired malaria and 11 birds (37.9%) remained nonparasitemic. A total of 32 avian malaria episodes were noted; 25 (78.1%) were identified as Plasmodium elongatum, 5 (15.6%) as Plasmodium relictum, and 2 (6.3%) as Plasmodium spp. One P. elongatum (3.4%) and 3 P. relictum (10.3%) infections were fatal. All deaths occurred during the first episode of parasitemia. Gross lesions of the birds that died included hepatomegaly and splenomegaly. Interstitial pneumonia with schizonts was observed on histological examinations. The range, mean, and SD of 12 hematological parameters were determined for nonparasitemic and parasitemic penguins. Differences between these groups in total white blood cell (WBC) counts and relative lymphocytosis (LYMPHS) were not significant. The combined classes of total WBC counts (> 20.0 x 10(3)/microliters) and LYMPHS (> 60.0%) are not indicative of avian malaria infection in African penguins. No correlations were found between changes in the values of blood parameters with season or age of penguins. Treatment of parasitemic birds significantly reduced expected mortality from 50.0% to 13.8%.
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PMID:Hematologic characteristics of avian malaria cases in African black-footed penguins (Spheniscus demersus) during the first outdoor exposure season. 815 74

Low-grade malignant non-Hodgkin lymphoma [NHL] and chronic lymphocytic leukemia [CLL] as its special form are slowly progressing malignancies which may present with lymphadenopathy, splenomegaly or, more rarely, hepatomegaly. The diagnosis is made by bone marrow or lymph node histology, while laboratory tests are relatively unspecific and may only hint towards the diagnosis. In contrast to high-grade malignant lymphoma, low-grade malignant NHL is often associated with the appearance of malignant lymphoma cells in peripheral blood. These malignant lymphocytes may be differentiated microscopically from normal lymphocytes, so that the diagnosis of NHL may be suspected not only because of clinical symptoms or lymphocytosis, which may present late in the natural history of the disease, but also on morphological grounds. Three types of low-grade malignant NHL cells may be recognized in peripheral blood: A mature appearing lymphocytic type with only slight alterations of the nucleus, a lymphoplasmocytic type, and a lymphocytic type with prominent alterations of the nucleus. The appearance of smudge cells and a monotony in lymphocyte morphology may serve as further diagnostic aids. Once the diagnosis has been suspected on morphological grounds, it may be verified in the case of B-cell lymphomas by flow cytometry. The clonality of T-cell lymphoproliferative disorders in addition has to be proven by demonstrating a clonal rearrangement of the T-cell receptor in Southern blots. An early diagnosis of low-grade malignant NHL may not only provide new insights into the natural history of monoclonal cytopathies but may also be of importance in the clinical management of patients.
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PMID:[Value of the blood picture and flow cytometry immunotyping in the early diagnosis of low-grade lymphoma]. 862 61

Fifty four cases of CLLB were studied from 1st of April. 1990 to October 30, 1993; 35 male and 19 female (M:F = 1.8:1) in age 39-76 years (median age = 62 years). 81% patients had lymphadenopathy, 30%--hepatomegaly, 31% splenomegaly, 24% had allergic symptoms, 24% had anaemia (7% AINH). 13% thrombopoenia (2% autoimmunologic thrombopoenia). In all cases immunological phenotype of peripheral blood lymphocytes was determined, 100% patients had B cells CD5+, 70% lymphocytes sIg+, 97%-CD19+, 73%-CD23+, 67%-CD22+, 82%-HLADr, 10%-71(TR90), CD10 was negative. There was negative correlation between B CD5+ cells and life span (p < 0.03). There was positive correlation, between CD23 and bulky diseases (p < 0.01). Percentage of T cells with CD2+, CD3+, CD4+, CD8+ and CD4:CD8 was diminished. Lymphocytosis T with antigens CD2+, CD3+, CD4+, CD8+ was enhanced. There was found a positive correlation between lymphocytosis CD2+ (p < 0.00009), CD4+ (p < 0.008), CD8+ (p < 0.0008) and blood lymphocytosis and positive correlation between T lymphocytosis CD2+ (p < 0.02), CD4+ (p < 0.002) and lymphocytosis bone marrow.
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PMID:[Chronic lymphatic leukemia from B CD5+ cells: characteristics, clinical and laboratory features, and immunophenotyping]. 865 49

The case of a 7-year-old boy with virus-associated hemophagocytic syndrome (VAHS) and serologically proven parvovirus B-19 infection is described. The patient with VAHS presented with fever, hepatosplenomegaly, pancytopenia, and hyperlipidemia type IV. After induction therapy with VP-16 and prednisone, partial remission was achieved. Despite maintenance therapy, reinductions, and the addition of cyclosporine A for 3 months, several relapses occurred. The therapy was stopped because of life-threatening complications (Klebsiella sepsis, neutropenic enterocolitis, and stercoral peritonitis). The complications were treated successfully. The patient status was stabilized after splenectomy. However, hepatomegaly progressed slowly and the hyperlipidemia endured. Ten months after the diagnosis leukocytosis with absolute T lymphocytosis appeared. Reactivation of VAHS was suspected and intravenous immunoglobin and then antilymphocyte immunoglobulin ALG therapy were started. The resultant decrease in leukocytosis was prompt, but lymphopenia did not occur. Virostatic treatment with foscarnet was introduced based on human herpesvirus-6 seroconversion. Twenty-six months after the diagnosis, the patient is well, without any sign of VAHS or lymphoproliferation.
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PMID:Infection-associated hemophagocytic syndrome complicated by infectious lymphoproliferation: a case report. 872 Oct 28

Despite intensified chemotherapy protocols, including autologous bone marrow transplantation (ABMT), stage IV neuroblastoma has a poor prognosis, and modern therapeutic trends are aimed at the eradication of minimal residual disease, which is though to be the main factor leading to relapse. In this pilot study, we report the systemic administration of high doses of interleukin-2 after ABMT in four patients. Five day cycles of IL-2 at a dose of 18 x 10(6) IU/m2/day were administered at variable time intervals as frequent as it was necessary to maintain the levels of natural killer (NK) cytotoxic activity higher than the median control value (40 LU/ml blood) throughout 1 year from the start of first IL-2 treatment. After IL-2 infusion, NK and LAK activities increased significantly (median 742 x 10(-3) LU/ml blood and 186.8 x 10(-3) LU/ml blood, respectively). Toxicities were transient and no life-threatening complications were observed. Fever, anorexia, skin rash and enlarged liver were always present. Anaemia, thrombocytopenia, leukocytosis, lymphocytosis and and eosinophilia occurred following most of the IL-2 courses. Although the small number of patients does not allow an estimation of the immunomodulatory-antineoplasic effects of IL-2, the results seem promising for the management of neuroblastoma patients.
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PMID:High-dose systemic interleukin-2 therapy in stage IV neuroblastoma for one year after autologous bone marrow transplantation: pilot study. 888 13

One hundred four Saudi patients with brucellosis who were admitted to Abha General Hospital in the Asir region of southern Saudi Arabia were studied. All the patients had Brucella melitensis infection; 61.5% were females while 38.5% were males. Their mean age was 32 years. Most of the patients (61.5%) lived in the lowland (Tihama) and the majority were shepherds (84.6%). The most common presenting symptoms were fever (100%), sweating (96.2%), headache (76.9%), joint pains (76.9%), and backache (73.1%). Physical findings included fever (96.2%), hepatomegaly (46.2%), splenomegaly (42.3%), tenderness over the spine (30.8%), arthritis (26.9%), and lymphadenopathy (19.2%). Mild anemia, leukopenia, and relative lymphocytosis were common. A history of raw milk ingestion was an important factor in disease transmission (84.6%), followed by close animal contact (73%) and raw liver consumption (63.3%). The study shows the effectiveness of several drug combinations in the treatment of brucellosis and the low relapse rate if the treatment is prolonged for not less than six weeks.
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PMID:A clinical study of brucellosis in adults in the Asir region of southern Saudi Arabia. 915 43


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