Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective study on acute lymphoblastic leukemia (ALL) was conducted to assess the pattern of childhood ALL at the Subdivision of Pediatric of Hematology, School of Medicine, University of North Sumatera/Dr. Pirngadi Hospital, Medan, in a period of 8 years (1980-1988). There were 120 cases, consisting of 63 (52.5%) males and 57 (47.5%) females. By the FAB classification (Bennett, 1976) (77.5%) were found as FAB L 1, 25 (20.8%) as FAB L 2, and 2 (1.7%) as FAB L 3. The youngest was 4 months old. The majority of signs and symptoms appeared in the forms of pallor 102 (85%), fever 84 (70%), hemorrhage 52 (43.3%), hepatomegaly 64 (53.3%), splenomegaly 54 (45%) and lymphadenopathy 18 (15%). On first admission, 76 (63.33%) cases were with a leukocyte count of less than 20,000/microliters, and 72 (60%) with Hb content of less than 5 g/dl. Twenty one cases died in the first year. The received cytostatic protocol; 11 (52.38%) were treated regularly and first remission were found in 8 (72.73%) cases. The average of admissions per year for the age group of 2-8 years was higher than the age groups of 0-2 years and 8-16 years (p less than 0.05).
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PMID:Acute lymphoblastic leukemia in the Department of Child Health, School of Medicine, University of North Sumatera/Dr. Pirngadi Hospital Medan (1980-1988). 179 87

Leukemic cell expression and serum levels of CD4, CD8, and interleukin-2 receptor (IL-2R) were determined at diagnosis for children or adolescents with acute myeloid leukemia (AML). Cellular expression of CD4 was detected in 18 of 62 cases, CD8 in none of 60 cases, and IL-2R in one of 33 cases tested. Myeloblasts of the M4 and M5 subtypes expressed CD4 significantly more frequently than other FAB subtypes (p = 0.0001). Serum levels of the three soluble factors (tested for 91 patients) were positively correlated with each other. Increased serum CD4 levels were significantly associated with cellular CD4 expression, high leukocyte count, M5 leukemia, spleen enlargement, and age less than 1 year. High serum CD8 levels correlated significantly with splenomegaly, extramedullary disease, absence of Auer rods, and high leukocyte count. Cases with high serum IL-2R levels were less likely to have Auer rods and more likely to have splenomegaly and M5 leukemia; serum levels greater than 750 U/ml were associated with a higher probability of treatment failure (p = 0.05), even after adjustment for other potential prognostic factors. Further studies of serum CD4, CD8, and IL-2R levels may help to clarify the immunoregulatory role of T-cells in patients with AML.
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PMID:Serum CD4, CD8, and interleukin-2 receptor levels in childhood acute myeloid leukemia. 190 14

Two patients with acute lymphocytic leukemia of B-cell phenotype (B-ALL) are described. They were 77-year-old female and 34-year-old male. One patient presented with marked splenomegaly, and the other with rupture of spleen on admission. Leukemic morphology revealed a typical L3 profile by FAB classification system in both cases. Immunologic analysis showed the presence of surface immunoglobulins in both cases, and one phenotype was IgM kappa, whereas the other was IgG kappa. Cytogenetic study revealed the typical translocations (8; 14) in both cases. Following chemotherapy, complete remission was achieved in one case, but the other died 36 days after admission. Including our two cases, we studied 12 cases of B-ALL and 77 cases of Burkitt's lymphoma reported in Japan, investigating the clinical prognosis as well as the biological features. We concluded that there are no significant difference of survival between B-ALL and Burkitt's lymphoma. We estimated it is due to oncogenesis from the same original cell in despite of the difference in main tumor site between two diseases.
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PMID:[Clinical, cytogenetic and immunological studies of 2 cases with B-cell acute lymphocytic leukemia. Comparison between B-ALL and Burkitt's lymphoma reported in Japan]. 210 43

A 56-year-old male was admitted to the Nihon University Hospital because of general fatigue and anemia on September 21st, 1985. He had mild hepato-splenomegaly. Hematological findings showed RBC 286 x 10(4)/microliters, Hb 6.0/dl, reticulocyte count 2.5%, platelet count 9.3 x 10(4)/microliters and WBC 2,400/microliters. An erythroblast per 100 leukocytes counted in a blood film was found. Bone marrow was erythroid hyperplasia with megaloblasts. The erythroblasts were PAS positive but not ringed sideroblasts. Other laboratory data including hemolysis were all negative. This case seemed to be diagnosed as refractory anemia (RA) according to the FAB classification. Chromosomal analysis of marrow cells, however, all revealed 46, XY, 20q- at diagnosis and 46, XY, 7q- 20q- after 22 months. Furthermore, Hb electrophoresis ahd family study indicated the presence of acquired HbH disease. Neither erythroid bursts (BFU-e) nor late erythroid progenitors (CFU-e) were detected. He has had progressive anemia without proliferation of blasts for over 2 years. From these findings, we postulate that the entity of erythremia should be distinguished from RA including many heterogeneous diseases.
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PMID:[An erythremia with acquired HbH disease and chromosomal abnormality]. 221 93

We report on 12 patients who fulfilled the FAB diagnosis criteria of chronic myelomonocytic leukemia (CMML). Some clinical findings (splenomegaly) as well as biological parameters (WBC count, cellularity of bone marrow sections, presence of dyserythropoiesis in bone marrow smears) were subjected to particular analysis. This allowed the assignment of each patient onto one of three separate hematological syndromes: (1) RA or RAEB with monocytosis, (2) myelomonocytic dysplasia (a novel syndrome) and (3) true CMML. Such separation may have distinct prognostic significance and may, therefore, predict life expectancy.
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PMID:FAB classification of chronic myelomonocytic leukemia: heterogeneity revealed on the basis of bone marrow findings. 321 30

We report 60 cases of chronic and subacute myelomonocytic leukaemias (CMML and SMML) in the adult, using the FAB group criteria. The M/F sex ratio was 3.3 and the mean age 67.5 years. Splenomegaly was found in 32% of cases, hyperleucocytosis in 52% of cases and mean blood monocytosis was 4.3 X 10(9)/l. Marrow smears showed an excess of blasts in 57% of patients, a moderate increase in monocytes in most cases and frequent myelodysplastic features. An increase in serum lysozyme and polyclonal hypergammaglobulinaemia were usual and clonal cytogenetic anomalies found in about half of the patients tested. Treatment was usually palliative and the median survival was 28 months, a blastic transformation being responsible for a third of the deaths. Prognostic factors at diagnosis were analysed retrospectively in the 46 patients who had sufficient follow up. Percentage of marrow blasts haemoglobin level and blood monocytosis at diagnosis, were subject to multivariate analysis, resulting in a discriminant 'score'. This allowed assignment of each patient into one of two prognostic subgroups (10.9% probability of error): a poor prognosis one, with a life expectancy of less than 1 year and a high risk of acute transformation (subgroup termed SMML) and a better prognosis subgroup (termed CMML), with some CMML patients surviving over 5 years.
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PMID:Chronic and subacute myelomonocytic leukaemia in the adult: a report of 60 cases with special reference to prognostic factors. 346 95

In an attempt to characterize the prognostic significance of the main initial clinical, haematological and biochemical features of chronic myelomonocytic leukaemia according to the FAB criteria, 29 such patients were analysed by means of univariate and multivariate statistical methods. At the time of the analysis 21 patients had died, with a median survival for the overall series of 8.2 months. The univariate analysis identified three parameters associated with poor prognosis: high monocyte counts, low platelet counts and splenomegaly. When all the initial features were included in a multiple regression model, only high monocyte counts and spleen enlargement retained their unfavourable prognostic influence (P = 0.002 and P = 0.02, respectively). Based on the presence or not of these prognostic factors, two populations of patients with different survival (median survival 5.6 and 16.5 months, respectively, P less than 0.005) could be identified. It seems therefore that monocytic proliferation serves not only as the diagnostic marker for chronic myelomonocytic leukaemia but also as the most important feature in the assessment of the patient's prognosis.
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PMID:A multivariate analysis of prognostic factors in chronic myelomonocytic leukaemia according to the FAB criteria. 347 Dec 68

Review of 5406 children with acute lymphoblastic (ALL) or nonlymphoblastic leukemia (ANLL) registered with Childrens Cancer Study Group (CCSG) since 1972 identified 115 patients (2.1%) with Down syndrome. The proportion of patients with Down syndrome was the same for ALL (2.1%) and ANLL (2.1%). Patients with ALL with and without Down syndrome did not differ significantly with respect to age at diagnosis, sex, race, morphology (FAB classification), cell surface markers, initial white blood cell count, pretreatment hemoglobin value, hepatomegaly, lymphadenopathy, presence of mediastinal mass, CNS disease at diagnosis, or prognostic group as defined by age and initial white blood cell count. Patients with ALL-Down syndrome less frequently had splenomegaly, had lower pretreatment platelet counts, and more often had normal or elevated IgG or IgA levels. In addition, they had a significantly lower rate of remission (81% versus 94%), a higher mortality during induction therapy (14% versus 3%), and a poorer overall survival with 5-year life table rates of 50% versus 65% (P less than 0.001). If an initial remission was achieved, there were no significant differences with respect to remission duration, survival, or disease-free survival. Patients with ANLL-Down syndrome were younger at diagnosis than those without Down syndrome. There was no significant difference in the remission rates between these patients. Analysis of findings in patients with ANLL provided results similar to those obtained for patients with ALL with regard to clinical outcome after achievement of an initial remission.
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PMID:Down syndrome and acute leukemia in children: a 10-year retrospective survey from Childrens Cancer Study Group. 623 37

40 children (23 males, 17 females) have been diagnosed have ANLL during the period from february 1970 to september 1981. According to FAB classification, 24 cases were M1,-M2, 9 M3, 3 M4, 3 M5 and 1 M6. At diagnosis, 20 patients (50%) had leukocytes less than 10.000/mmc, 6 (15%) had leukocytes greater than 50.000mmc. Hb levels was 7 g% in 16 patients (40%); 10 children had hepatosplenomegaly (25%), 7 splenomegaly (18%) and 5 lymphoadenomegaly (13%). 4 patients had cutaneous or mucous infiltrates. None had meningeal involvement at diagnosis. According to the year of diagnosis, 3 groups can be identified. In the group I (1970-73), 11 patients have been treated with not codified combination chemotherapy as ARA-C, 6-TG, DNR, CTX, Metil-GAG. In the group II (1974-76) and in the group III (1977-81), the patients (respectively 12 and 17) have been treated according to the following protocols: LAM-5 (3), TRAP (5), COAP (1), LAM 80 (2), AIL 7402 (8), AIL 7604, AIL 7801 (6). Immunotherapy has been performed in 7 cases. CNS prophylaxis (MTX i.t. +/-ARA-C +/- RT) was given in 5 patients of group II and in 6 of group III. I patients of group I (45%), 6 of group II (50%) and 13 of group III (76%) achieved CR. Median duration of remission was 5 months in the group I and in 17 in group II and III.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Acute non-lymphatic leukemia in children]. 654 20

Thirty-five patients who fulfilled the FAB diagnosis criteria of chronic myelomonocytic leukemia (CMML), i.e., myelodysplastic features, monocytosis over 10(9)/liter, bone marrow monocyte infiltration, blast cells less than 5% in the peripheral blood and less than 30% in the bone marrow, are analyzed. CMML appears as an entity distinct from myelodysplastic and myeloproliferative disorders. Splenomegaly, anemia, thrombocytopenia, leukocytosis with monocytes and granulocytic cells in all stages of development, increased blood and urine lysozyme levels without renal failure, and polyclonal hyperimmunoglobulinemia are its main clinical and biologic features. With conventional cytotoxic drugs (6-mercaptopurine, hydroxyurea), the prognosis of CMML appears poor (median survival 475 days). None of the clinical hematologic or biologic parameters tested had a significant effect on prognosis. As other chemotherapy trials seemed necessary, we recently administered small doses of cytosine-arabinoside (ARA-C) to six patients over several consecutive days and obtained a complete remission in four. These preliminary results must be confirmed by larger series using the diagnostic criteria proposed by the FAB cooperative group.
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PMID:Chronic myelomonocytic leukemia according to FAB classification: analysis of 35 cases. 658 39


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