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)

Three patients with megakaryoblastic leukaemia are described. All three presented with pancytopenia, a few blast cells in the peripheral blood and absence of overt hepatosplenomegaly. In two of them bone marrow aspiration yielded a dry tap. Histological investigation of the bone marrow indicated that the megakaryocytic cell line was the dominant proliferating lineage. Cytochemical and EM investigation supported these findings. The isomorphic isoenzyme pattern of the elevated serum lactic dehydrogenase might be of diagnostic importance. Despite chemotherapy, there was a rapidly fatal terminal leukaemic phase with high blast cell counts. The differentiation from other haematological malignancies, especially acute (aleukaemic) leukaemias and the accelerated phase of primary (chronic) myelofibrosis, is discussed. The picture appears to be identical with acute (malignant) myelofibrosis.
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PMID:Megakaryoblastic leukaemia (acute myelofibrosis): a report of three cases. 57 95

This report describes three cases with Down's syndrome. These cases initially had transient abnormal myelopoiesis (TAM), from which they recovered spontaneously. They finally developed into overt acute leukemia characterized by an increase of blasts, hepatosplenomegaly, and elevated lactic dehydrogenase. Of these three cases, one was thought to have ANLL, which broke out 5 months after spontaneous remission. The other two had ALL, each occurring 8 and 9 years later. Chromosomal abnormality, in addition to trisomy 21, was detected in blast cells from one of the patients with acute leukemia. All three patients with acute leukemia experienced complete remission. However, two of the three patients relapsed and died. It is noted in the literature that remission is permanent in most cases of TAM, and is rarely terminated by leukemic relapse. In view of our observations, the importance of following up on such patients who evidence apparent remission of their leukemia-like disorder is emphasized.
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PMID:[Transient abnormal myelopoiesis followed by acute leukemia in children with Down syndrome]. 215 Apr 19

A case of polymyositis associated with chronic active hepatitis was reported. A 53-year-old man, who had no previous history of blood transfusion nor hepatitis, noticed proximal dominant muscle weakness on January 29, 1985. He was admitted to Kyoto National Hospital on February 7, and laboratory studies disclosed the elevation of serum enzyme levels; creatine kinase (CK) 9845 IU/L (normal 54-263), glutamate oxaloacetate transaminase (GOT) 834 IU/L (9-31), glutamate pyruvate transaminase (GPT) 491 IU/L (4-34), lactate dehydrogenase (LDH) 2135 IU/L (248-464). Also serum gamma globulin was high (1.8 g/dl) and LE-like cell was found. The diagnosis of polymyositis was made and prednisolone therapy (60 mg/day) was started on February 23. The elevated serum enzymes decreased gradually, but severe muscle weakness persisted for about one month. On April 3, he was admitted to our hospital. Physical examination revealed moderate proximal dominant muscle weakness without skin eruption, jaundice or hepatosplenomegaly. The serum enzymes were still high; CK 1826, GOT 173, GPT 232 (GOT less than GPT), LDH 1548. However, alkaline phosphatase (ALP) and bilirubin were normal. Hepatitis B surface antigen (HBsAg) was not detected. Antinuclear antibody was positive. The electromyogram study showed myopathic change, and the muscle biopsy demonstrated myopathic change and cell infiltration, compatible with polymyositis. These results suggested liver dysfunction associated with polymyositis. Prednisolone therapy was continued and muscle weakness decreased. From December, 1985, serum enzymes (CK, GOT, GPT, LDH) elevated again and muscle weakness also slightly increased. Anti-smooth muscle antibody was positive. It was suggested that both polymyositis and liver dysfunction deteriorated.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of polymyositis associated with chronic active hepatitis]. 218 64

We have experienced and treated seven patients of pre-B cell leukemia in childhood. Clinical, cytological and ultrastructural characteristics of them were studied. Most of them had higher counts of white blood cells, hepatosplenomegaly, high value of lactic dehydrogenase and various karyotype abnormalities at onset. The chromosomal translocation t (1; 19) that is supposed to be specific to pre-B cell ALL was found in four of seven of our cases. In the seven patients, survival was studied in comparison to that of 27 common ALL patients at our hospital that are common in childhood acute leukemia. Although no difference in remission duration and survival time between pre-B cell ALL patients and common ALL group, there have been seen the tendency that remission and survival were of shorter duration for patients with pre-B cell ALL.
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PMID:[Clinical and laboratory studies in seven patients with pre-B cell leukemia in children]. 262 99

To elucidate the clinicopathologic features of non-Hodgkin's lymphoma (NHL) in Taiwan, 123 adult patients with proven NHL were studied. They were classified according to the international working formulation as: low grade (LG), 12.2%; intermediate grade (IG), 42.3%; and high grade (HG) lymphoma, 45.5%. The most common subtypes were diffuse large cell (26.8%) and large cell immunoblastic (26.8%) lymphomas. Follicular lymphoma accounted for only 8.9% (11 cases). Complete remission rates for LG, IG and HG lymphomas were 53%, 35% and 34%, respectively. LG lymphoma had a significantly better survival than that of IG and HG lymphomas. The IG lymphoma encompassed a heterogeneous group of patients with varying prognoses but the overall survival curve was indistinguishable from that of HG lymphoma. Clinically, 66% of HG, 77% of IG and 86% of LG lymphoma presented with advanced disease. LG lymphoma had high frequencies of hepatosplenomegaly (30-50%) and bone marrow involvement (53%), whereas skin, bone and central nervous system involvement occurred exclusively in IG and HG lymphomas. Mild anemia was common and occurred in 40-50% of the patients. Hyperimmunoglobulinemia was found in 50-60% of all 3 grades of lymphoma, monoclonal gammopathy in 3 cases of IG lymphoma, and hypercalcemia in 4 cases of IG and HG lymphomas. Elevated serum lactate dehydrogenase occurred mainly in IG and HG lymphomas and was an important prognostic factor. In conclusion, the characteristic features of NHL in Taiwan include: (1) a high proportion of HG lymphoma and low proportions of LG and follicular lymphomas; (2) a heterogeneous patient composition of IG lymphoma with an unfavorable overall prognosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Adult non-Hodgkin's lymphoma in Taiwan area: a clinicopathologic study of 123 cases based on working formulation classification. 276 10

We describe five patients with adult T-cell leukemia/lymphoma (ATL) with neither integration of human T-cell leukemia virus type I (HTLV-I) into their leukemia cells nor anti-HTLV-I antibody in their sera. These findings indicate that HTLV-I may not have been involved in leukemogenesis in these patients. The clinicohematological, cytopathological, and immunological features of HTLV-I-negative ATL were exactly the same as those of HTLV-I-associated ATL. Leukemia cells with pleomorphic nuclei, generalized lymphadenopathy, hepatosplenomegaly, skin lesions, hypercalcemia, and elevated lactate dehydrogenase levels, all of which are characteristic features of typical ATL, were also seen in these patients with HTLV-I-negative ATL. Leukemia cells expressed T3, T4, and pan-T-cell antigens in three cases, and T3 and pan-T-cell antigens in two. All five patients had lived in ATL-nonendemic areas. The finding of HTLV-I-negative ATL suggests that factor(s) other than HTLV-I infection may be involved in ATL leukemogenesis.
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PMID:Adult T-cell leukemia/lymphoma not associated with human T-cell leukemia virus type I. 301 71

Three hundred twenty-five previously untreated patients with chronic lymphocytic leukemia were analyzed to identify significant prognostic factors for survival. Univariate analysis identified the following characteristics associated with survival: (1) clinical characteristics: age, race, sex, performance status, lymphadenopathy, and hepatosplenomegaly; (2) hematologic parameters: WBC count, absolute lymphocyte and granulocyte counts, hemoglobin level, and platelet count; and (3) biochemical parameters: serum albumin, calcium, uric acid, lactate dehydrogenase, alkaline phosphatase, BUN, and creatinine. Multivariate regression analysis in a randomly selected training subset of 217 patients demonstrated that the combination of uric acid, alkaline phosphatase, lactate dehydrogenase, external lymphadenopathy, and age had the strongest predictive relation to survival time. The resulting model was validated in the remaining independent subset of 108 patients and led to classification of patients into low, intermediate, and high-risk groups with five-year survival rates of 75%, 59%, and 14%, respectively, and with distinctively different annual mortality rates (P less than .01). Both the regression model and Rai staging were highly effective in identifying risk groups among the entire patient population (P less than 0.001). Overall the regression model was superior to Rai staging in defining prognostic risk groups. In addition, it was able to separate patients into significantly different risk categories within each Rai stage, thus improving on the prognostic prediction of individual patients with chronic lymphocytic leukemia.
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PMID:Prognosis of chronic lymphocytic leukemia: a multivariate regression analysis of 325 untreated patients. 381 21

We have observed five patients with smoldering adult T-cell leukemia (ATL) who had skin lesions as premonitory symptoms. The illness developed slowly, but flared up after several years. Skin lesions appeared in the form of erythema, papules, or nodules. Infiltration of the skin by ATL cells was slight, and the proportion of ATL cells in the peripheral blood was 0%-2%. The serum lactate dehydrogenase (LDH) value was within normal range and was not associated with hypercalcemia; lymphadenopathy, hepatosplenomegaly, and bone marrow infiltration were very slight. In most cases, hypergammaglobulinemia was seen, and in one case, monoclonal hypergammaglobulinemia was observed. All five patients had lived in an area in which ATL was endemic, and their anti-ATLA antibodies were positive; none had ever received a blood transfusion. One patient developed typical ATL after more than 13 yr of illness and died of renal insufficiency. Another patient developed typical ATL after 5 yr of illness and died of cryptococcus meningitis. Based on clinical and pathologic differences, we believe that these cases should be distinguished from typical ATL cases for the purposes of prognosis and treatment.
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PMID:A proposal for smoldering adult T-cell leukemia: a clinicopathologic study of five cases. 622 22

Serum lactate dehydrogenase (LDH) activity is increased in many tumor-bearing patients and can be used as a prognostic marker. We studied serum LDH concentration in 94 consecutive patients with non-Hodgkin's lymphoma who were histologically classified according to the Kiel Classification and were grouped according to the Non-Hodgkin's Lymphoma Pathologic Classification Project Working Formulation. 74 patients were studied at diagnosis, and 20 of them (27%) had an LDH level higher than 250 U/l. High LDH levels were more frequent in cases of true histiocytic, high-grade, and intermediate-grade malignancy lymphoma (4 of 7, 7 of 14, and 7 of 20, respectively) than in cases of low-grade lymphoma (2 of 33). A close relationship of LDH to several prognosis-related disease features was found, including general symptoms, bulky disease, big mediastinal tumor, huge hepatosplenomegaly, bone marrow involvement, and a leukemic syndrome. LDH was higher than normal in a high proportion of cases who were studied in relapse (13 of 20, 65%). These data suggest that in non-Hodgkin's lymphomas the LDH serum concentration is not independent of other disease features, so that the prognostic value of LDH is probably lower than expected from previous studies. Serum LDH activity decreased to normal in all cases of complete remission, but also in cases of partial remission, suggesting that measuring enzyme activity is of a limited usefulness for detecting and monitoring minimal residual disease. For that purpose, LDH isoenzyme studies would be more appropriate.
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PMID:Serum LDH concentration in non-Hodgkin's lymphomas. Relationship to histologic type, tumor mass, and presentation features. 643 91

We have observed five patients with smoldering adult T-cell leukemia (ATL) who had skin lesions as premonitory symptoms. The illness developed slowly but flared up after several years. Skin lesions appeared in the form of erythema, papules or nodules. Infiltration of the skin by ATL cells was slight, and the proportion of ATL cells in the peripheral blood was from 0% to 2%. The serum lactic dehydrogenase value was within normal range, and was not associated with hypercalcemia, lymphadenopathy, or hepatosplenomegaly, and bone marrow infiltration was very slight. In most cases, hypergammaglobulinemia was seen, and in one case monoclonal hypergammaglobulinemia was observed. All five patients had lived in an area in which ATL was endemic, and their sera were positive for anti-ATL-associated antigen antibodies. None of them had ever received a blood transfusion. One patient developed typical ATL after more than 13 yr of illness, and died of renal insufficiency. Another patient developed typical ATL after 5 yr of illness, and died or cryptococcus meningitis. These cases were clinically and pathologically different from typical ATL cases already reported, and we feel it necessary to make distinctions from the viewpoints of prognosis and treatment. In discussing these cases, we compared smoldering ATL with typical ATL, and deliberated upon the causes of both.
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PMID:A proposal for smoldering adult T-cell leukemia--diversity in clinical pictures of adult T-cell leukemia--. 660 27


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