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 initial features, response to therapy, complications, cause of death, and prognostic factors of 171 consecutive children with ANLL are described and compated to historical data for adults with ANLL and for children with ALL. Major differences between children and adults with ANLL include a higher frequency of CNS leukemia and a lower frequency of early deaths in the children. The most important differences between children with ANLL and ALL are the absence of a peak age of incidence in ANLL and the far better response to therapy in ALL. Among features present at 100,000/mm3 or above, and no palpable hepatomegaly had significantly longer survivals, while patients with platelet counts below 10,000/mm3 had significantly shorter survivals. The frequency and duration of remission were significantly better with three protocols used since 1968 than previously. However, even with these protocols, the results were far from satisfactory, with a complete remission frequency of 66%, a median duration of hematological remission of 6 months, and a median duration of survival of 10 months. The striking contrast of these results in childhood ANLL with current results in childhood ALL underscores the need for novel, imaginative therapeutic approaches for ANLL.
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PMID:Acute nonlymphocytic leukemia in 171 children. 78 98

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

To study the survival and prognostic factor of childhood acute lymphoblastic leukemia, 78 newly-diagnosed cases between January 1982 and June 1987 in National Taiwan University Hospital were reviewed and analyzed. They were stratified into two groups, i.e. standard-risk (SR) and high-risk (HR), according to their pre-treatment leukocyte count and age. Following induction therapy, 97% of the SR patients and 80% of the HR patients attained complete remission. In the SR group, the 2- and 3-year failure-free survival rates were 37% and 24%, with a median survival of 16 months. In the HR group, failure-free survival at the second and third year were 11% and 4%, respectively, with a median survival of 5.3 months. Three factors are strongly related to induction failure, i.e. high leukocyte counts (greater than 50*10(9)/1), massive hepatomegaly and large lymph nodes. Univariate analysis of failure-free survival showed six variables with significant detrimental effects on eventual outcomes, i.e. high leukocyte counts (greater than 50*10(9)/1), meningeal leukemia, marked lymphadenopathy, age younger than 2 years and older than 10 years, massive hepatomegaly (greater than 6 cm), and high LDH level (greater than 800 u/1). However, statistical survival models should also determine the joint effects of the prognostic factors so that the relative importance of each factor can be assessed. High initial leukocyte count, disclosed by multivariate analysis, was the single most important factor detrimental to the continuance of complete remission (P = 0.0004). Preliminary results also revealed poor compliance and early relapse in this study. Possible causes of early failure are discussed. Conceptual education for family members, as well as management with effective cytoreductive therapies are urgently needed.
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PMID:An analysis of risk factor and survival in childhood acute lymphoblastic leukemia. 263 11

A variety of oncogenes are activated by specific chromosomal translocations, which are associated with distinct subtypes of leukemia. The identification of these rearrangements provides critical diagnostic and prognostic information, which may contribute to the selection of specific anti-leukemic therapy. The translocation t(9;22), the equivalent of the BCR/ABL rearrangement, is associated with a poor prognosis. We therefore used RT-PCR to detect this molecular event in a prospective study including 890 children. 673 of them suffered from acute lymphoblastic leukemia (ALL) at primary diagnosis and a transcription of the chimeric gene was detected in 21 of 648 with a successful analysis (3.2%). All children were treated by one of the two German multicenter childhood ALL therapy studies ALL-BFM-90 or COALL-05-92, respectively. Comparison of clinical features between BCR/ABL-positive and -negative children showed no significant differences regarding WBC, percentage of blasts, splenomegaly, hepatomegaly and age. Immunophenotypic studies at diagnosis in 21 BCR/ABL-positive children identified common ALL in 16 patients (76.2%), pre-B-ALL in four (19.0%), and an early T-lineage ALL in one (4.8%). Coexpression of myeloid antigens (CD13 and/or CD33) was observed in six of 16 common ALL patients as well as in the one child with early T-lineage ALL phenotype. The type of breakpoint (m-BCR/ABL: n = 14; M-BCR/ABL: n = 7) showed no correlation with clinical parameters. A comparison of cytogenetic and molecular data was performed in 16 positive patients and was concordant in all of them. We analyzed the response to the prednisone pretreatment and found a higher incidence of poor responders among the BCR/ABL-positive children. Regarding the event-free survival (EFS) of BCR/ABL-positive (0.53) and -negative patients (0.79) after a follow-up of 2 years, significant differences (P < 0.05) between both groups could be demonstrated.
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PMID:Incidence and clinical outcome of children with BCR/ABL-positive acute lymphoblastic leukemia (ALL). A prospective RT-PCR study based on 673 patients enrolled in the German pediatric multicenter therapy trials ALL-BFM-90 and CoALL-05-92. 866 52

The SCID mouse represents a valuable tool for assessing growth characteristics and drug sensitivity of human leukemic cells. We have examined differences in the engraftment patterns in SCID mice of primary human leukemic cells isolated from children (< 21 years old) with either t(1;19)+/E2A-PBX1+ or t(9;22)+/BCR-ABL+ acute lymphoblastic leukemia. Leukemic cells from 13/24 t(1;19)+/E2A-PBX1+ patients caused overt leukemia in SCID mice. Macroscopic lesions were evident in 6/13 cases, with multiple sites involved in some mice: hepatomegaly,(3) splenomegaly(4), thymic enlargement; liver tumors(1), kidney tumors(1), abdominal tumors(1). Microscopic lesions in SCID mouse organs were present in all 13 cases and involved the bone marrow, brain, heart, gut, liver, kidney, lung, ovary, pancreas, skeletal muscle, spleen, and thymus. Leukemic cells from 5/20 t(9;22)+/BCR-ABL+ patients caused overt leukemia in SCID mice. Notably, macroscopic lesions (splenomegaly; leukemic bones; hepatic tumors) were observed in only 1 case. In all 5 cases, microscopic lesions were found in the mouse bone marrow. Additional microscopic lesions were restricted to skeletal muscle, spleen, and mesentery (1 case) or thymus (1 case). These findings differ markedly from those of t(1;19)+/E2A-PBX1+ leukemic cells due to the lack of involvement of major organs such as liver, pancreas, kidney, skin, or brain. These data illustrate the biological heterogeneity of childhood ALL and suggest that the differential risks associated with t(1;19)+/E2A-PBX1+ and t(9;22)+/BCR-ABL ALL might arise from unique engraftment and proliferation capabilities of the respective leukemic cell populations.
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PMID:Distinct in vivo engraftment and growth patterns of t(1;19)+/E2A-PBX1+ and t(9;22)+/BCR-ABL+ human leukemia cells in SCID mice. 1003 3

Acute lymphoblastic leukaemia (ALL) is a common paediatric cancer with a tendency to relapse, usually within 3 years of remission. Most patients present with hepatomegaly, splenomegaly, pallor, fever and bruising. Localised muskuloskeletal presentation is extremely rare. Here, we present a case of leukaemia relapse in the bone marrow of a 28-year-old man 9 years after achieving remission, presenting only with ankle pain and normal routine labs besides mild hypercalcemia, and no signs of disease in common bone marrow biopsy sites. This highly localised presentation is unusual and would hopefully inform clinicians to have a high index of suspicion for relapse in an adult patient who has had childhood ALL.
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PMID:Childhood acute lymphoblastic leukaemia relapse with atypical localised presentation mimicking ankle trauma in a 28-year-old man. 3111 75