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)

The levels of serum gamma-glutamyl transpeptidase (GGT) and, when appropriate, alkaline phosphatase (AP) and 5'-nucleotidase (NTD) have been measured as a routine in 276 patients with malignant haematological diseases during a 26-month trial period. GGT levels add no prognostic information to the routine haematological surveillance of leukaemia. Polychemotherapy does not appear to be an inducer of liver drug-metabolising microsomal enzymes. Polycythaemia rubra vera, myelofibrosis and chronic lymphocytic leukaemia may cause little change in GGT, AP and NTD levels despite marked hepatomegaly. A raised GGT in Hodgkin's disease and non-Hodgkin lymphoma is generally associated with active and widespread disease, but not necessarily a sign of malignant tissue in the liver. The elevations of GGT in myeloma may be secondary to liver infiltration though this group merits further detailed study.
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PMID:Evaluation of the usefulness of serum gamma-glutamyl transpeptidase levels in the management of haematological neoplasia. 2 19

In a retrospective study it was endeavoured to evaluate the effects of splenectomy in chronic lymphocytic leukaemia (CLL) characterised by splenomegaly. The material comprises 42 patients subjected to the operation in the course of the past 20 years. In the majority the spleen weighed more than 1000 g. The main indication for splenectomy was anaemia, while in 9 cases it was thrombocytopenia and in 14 cases hypercatabolism. Splenectomy is followed by a pronounced increase in the venous haemoglobin level and platelet count to higher values which have been recorded for up to 3 years after the procedure. In cases where data were available, there has been weight gain and a falling basal metabolic rate. Splenectomy is effective especially in cases predominated exclusively by splenomegaly, but even in cases with marked extrasplenic manifestations, splenectomy often greatly reduces the need for prednisone and cytostatics. Increasing hepatomegaly and lymphomas were not more common after splenectomy than in a control series, and the incidence of infections was not increased after the operation. For comparison, 37 non-splenectomised patients with splenomegaly were assessed. X-radiation of the spleen seems to be insufficient, since usually it has to be repeated. Splenomegaly does not decrease spontaneously and rarely after treatment with prednisone/cytostatics. The findings indicate that splenectomy of patients with CLL and increasing splenomegaly should be performed more often and presumably also earlier than recommended in the literature.
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PMID:Splenectomy in chronic lymphocytic leukaemia. 6 31

Two cases of T-cell chronic lymphoid leukaemia in adults were classified by recently developed immunological methods. Clinically there was splenomegaly, hepatomegaly, marked anaemia, para-aortic lymph nodes, cutaneous infiltration, little peripheral lymph-node enlargement and significant marrow infiltration. Immunological tests of membrane antigens revealed T-cell determinants. T-CLL may differ in its course and prognosis from B-CLL and may require different treatment.
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PMID:[T-cell leukaemia in adults: report of two cases (author's transl)]. 30 81

Continuous flow centrifuge leucapheresis (CFCL) provides a simple, mechanical technique for removing large numbers of circulating lymphocytes without cytotoxicity. Seven sequences of CFCL were performed on six patients with chronic lymphocytic leukaemia (CLL). In six, the median course consisted of six procedures in 10 days. A median total of 27.7 X 10(11) leucocytes were removed and a 70% drop in leucocyte counts (from 433 X 10(9)/l. to 130 X 10(9)/l.) was observed with little fall in haemoglobin and platelet counts. Tissue massess decreased in two patients. A similar effect was seen in one patient managed less intensively with 21 procedures being performed over 73 days, removing a total of 87.6 X 10(11) leucocytes. The peripheral leucocyte count decreased from 760 X 10(9)/l. to 114 X 10(9)/l. and hepatomegaly diminished markedly. One patient has survived for over 9 months since CFCL but stayed in control only briefly after each of two courses. Short courses of daily CFCL can produce short-term benefit in the management of advanced CLL but at present its main value is as an investigational form of therapy which provides a unique opportunity to study the cytokinetics of advanced CLL without the complications of superimposed cytotoxic therapy.
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PMID:The role of continuous flow centrifuge leucapheresis in the management of chronic lymphocytic leukaemia. 125 42

A 33-year-old man was hospitalized because of thrombocytopenia and severe splenomegaly. On admission 78% of peripheral lymphoid cells were abnormally large, with pale cytoplasm. Flow cytometry of the abnormal lymphocytes showed that they expressed CD 2, CD 3, CD 11, CD 16, and CD 56, but not CD 4 nor CD 8, so they were T-cell large granular lymphocytes (T-LGL). Abnormal lymphocytes obtained from a lymph node expressed CD 2, CD 16, CD 38, and CD 56, but not CD 3, CD 4, and CD 8, so they were natural killer(NK) cells. Splenectomy was performed and the operative specimen showed diffuse infiltration of pleomorphic lymphocytes, probably chronic lymphocytic leukemia cells. After splenectomy, the platelet count returned to normal but the lymphocytosis continued. Two years after discharge, chemotherapy was done because of thrombocytopenia and hepatomegaly. The patient died of disseminated intravascular coagulation arising from sepsis. The differences and similarities between peripheral and lymph-node lymphocytes suggest that LGL and NK cells may be differentiated from the same kind of cell, somewhat differentiated from stem cells.
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PMID:[Chronic lymphocytic leukemia with peripheral T lymphocytes expressing CD 2+, CD 3+, CD 4-, CD 8-, CD 16+, and CD 56+ and lymph-node lymphocytes expressing CD 2+, CD 3-, CD 4-, CD 8-, CD 16+, CD 38+, and CD 56+]. 171 68

A review was carried out of 187 previously untreated cases of chronic lymphocytic leukaemia diagnosed between 1969 and 1988. The median age of the patients was 65 years (range, 36-87). There were 118 males (M/F ratio, 1.7). In accordance with Rai's staging system the patients were distributed as follows: 0.29%; I, 20%; II, 25%; III, 13%; IV, 13%, and according to Binet's staging the distribution was: A, 55%; B, 21%; C, 24%. The most frequently found physical findings were lymph node enlargement (55%), splenomegaly (32%) and hepatomegaly (28%). Anaemia was present in 20% of the cases and thrombocytopenia in 13%. The mean lymphocyte count was 62 x 10(9)/L (range, 6-475 x 10(9)/L). Bone marrow infiltration of over 80% was seen in 46% of the patients. Bone marrow biopsy was performed on 97 patients, the diffuse pattern of involvement being most commonly found (44%). Increased BUN (55%), alkaline phosphatase (42%) and LDH (38%) were the most frequent biochemical alterations. Hypogammaglobulinaemia was present in 55% of the patients, IgM being the most commonly affected immunoglobulin (66%). Monoclonal gammopathy was seen in 4% of the cases. LDT, measured in 75 patients, was less than a year in 32%. No antileukaemic drugs were needed in 34% of the patients. When concluding this study, 100 patients had died. The median survival was 57 months and death was related to chronic lymphocytic leukaemia in 53% of such patients.
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PMID:[Chronic lymphatic leukemia. I. Clinico-biological features and survival analysis. Study of 187 patients]. 177 6

Chronic lymphocytic leukaemia was diagnosed in 1957 in a 29-year-old male patient who lived 31 years after that time. At the time of diagnosis he was in good general condition with the enlarged liver (4 cm) and spleen (5 cm). In peripheral blood the leukocyte count was 47 x 10(9)/1 with 80% lymphocytes (38 x 10(9)/1), Hb 106 g/1; the bone marrow showed hypercellularity with predominance of lymphocytes. Treatment with corticosteroids was during 1958-1966. In 1967 Chlorambucil was given for 3 months, in 1972 for one month, inducing normalization of leukocyte count, 5.7 x 10(9)/1 with 40-50% of lymphocytes (3 x 10(9)/1). In 1977 the bone marrow showed slight lymphocytosis (38%). Numerous infections were recorded. Splenomegaly disappeared in 1980. In 1982 macrocytic anaemia was observed. Diagnosis of pernicious anaemia was established based on megaloblastic bone marrow and positive Schilling test. Vitamin B12 treatment resulted in reticulocyte response (crisis) and normalization of haemoglobin level. In 1988 light anaemia and melena appeared. Gastroscopy revealed lesions in the fornix histologically identified as ventricular carcinoma. It can be presumed that the appearance of pernicious anaemia as an autoimmune disease is analogous to other autoimmune complications in chronic lymphocytic leukaemia (haemolytic anaemia, thrombocytopenia).
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PMID:[Chronic lymphocytic leukemia complicated by pernicious anemia during long-term remission]. 213 9

The T cell chronic leukemias encompass a broad spectrum of diseases involving mature post-thymic T cells. With the development of highly specific marker studies, clear patterns of immunophenotypic and functional characteristics of the involved cells have emerged. These studies, along with the development of molecular probes for the T cell receptor gene loci, have helped to elucidate the pathogenetic basis for the highly variable clinical course which has been described for patients with these disorders. The T gamma lymphocytosis syndrome has been identified as a benign chronic illness which is nevertheless usually a monoclonal neoplastic proliferation of large granular lymphocytes. These patients represent a distinct clinical entity characterized by splenomegaly, neutropenia, and peripheral blood lymphocytosis. The cells of TGLS are large granular lymphocytes and display many of the immunophenotypic and functional characteristics of NK and K cells. These cells have been implicated pathogenetically in the associated cytopenias seen in the illness, but a clear link has not been established. Although the lymphoproliferative manifestations of the disease are usually easily controlled with low-dose alkylating agents, therapy of the neutropenia has been relatively unsuccessful. Separating these patients from the rest of the spectrum of the T cell chronic leukemias has provided insight into the other disorders as well. It has established that T-CLL and T-PLL are, in fact, extremely rare. T-CLL is similar to its B cell counterpart, except that patients have a higher incidence of skin infiltration. Available data suggest that the prognosis in T-CLL is actually less variable, and somewhat worse, than generally believed when those patients were viewed in conjunction with the patients with the more benign TGLS. T-PLL is an extremely aggressive disease characterized by massive splenomegaly, lymphadenopathy, and skin infiltration. It is refractory to most forms of therapy. These illnesses are again phenotypically distinct from the retrovirus-associated ATLL. Most of the early cases of T-CLL reported from Japan were probably ATLL; this disease is characterized by pronounced splenomegaly, hepatomegaly, lymphadenopathy, and skin infiltration. It has an extremely aggressive natural history, and survival is usually less than 1 year from diagnosis. The rapid development of sophisticated immunologic and molecular techniques for analyzing T cell proliferations has allowed highly specific distinctions to be made among the cells of origin of the different T cell chronic leukemias. It is hoped that increased understanding of the immunologic and functional characteristics of these diverse T lymphoid populations will provide further insights which will have an impact on directed therapeutic interventions in the future.
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PMID:T gamma lymphocytosis and T cell chronic leukemias. 218 3

A 48-year-old male was admitted to our hospital on April 20, 1989 because of general fatigue and abdominal fullness. Physical examination showed hepatomegaly, massive splenomegaly, and systemic lymphadenopathy. Hematological findings revealed WBC 73,000/microliters, RBC 289 x 10(4)/microliters, Hb 8.0g/dl, and platelet 9.1 x 10(4)/microliters. WBC differential count demonstrated a mixture of 63% matured small lymphocytes and 32% prolymphocytoid cells. Bone marrow aspiration was unsuccessful with a dry tap. Surface marker analysis of peripheral blood lymphoid cells disclosed that they were positive for anti-HLA-DR, CD 5, CD 19, CD 20, CD 21, CD 25, Sm-IgM, Sm-IgD, and Sm-K. He was diagnosed as B-CLL/PL, and treated with VEPA with partial remission. CLL/PL which was advocated by Melo in 1986 is regarded as a distinct clinical entity intermediate between CLL and PLL in clinical and laboratory features. Our case is interesting with regard to good response to combination chemotherapy, though most cases of CLL/PL have a resistance to standard chemotherapy.
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PMID:[B-chronic lymphocytic leukemia/prolymphocytic leukemia (CLL/PL)--a case report]. 228 70

Sixty-two previously untreated patients with B-cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep-rosette, mouse-rosette, slg, and HLA/DR, CD20, FMC7, CD5, and CD9 antigens, had a significant influence on survival. On the other hand, several clinical and haematological characteristics were identified as being associated with survival: 1) clinical features--presence of lymphadenopathies (P less than .05) and hepatomegaly and/or splenomegaly (P less than .04); 2) haematologic parameters--presence of anaemia and/or thrombopenia (P less than .05), the absolute peripheral blood lymphocyte count (P less than .03), and the presence of hypogammaglobulinemia (P less than .08); 3) biochemical parameters--serum uric acid (P less than .03); and 4) bone marrow histopathological features--biopsy pattern (P less than .04) and the percentage of lymphocytes in bone marrow aspirate (P less than .03). Both the Rai staging and the International Workshop on CLL staging systems were effective in identifying groups of patients with significantly different prognoses (P less than .05). Multivariate regression analysis demonstrated that the combination of three clinicopathologic characteristics (bone marrow histopathologic pattern, absolute peripheral blood lymphocyte count, and the presence or not of hypogammaglobulinaemia) had the strongest predictive relationship with survival time. In summary, our findings show that the clinicobiological and anatomopathologic parameters have much more prognostic relevance than the immunological markers analysed in the present study.
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PMID:B-cell chronic lymphocytic leukaemia: prognostic value of the immunophenotype and the clinico-haematological features. 270 40


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