Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The acute respiratory distress syndrome in childhood is a rare disease, but as in the past still plagued with a high mortality rate. It is caused by severe pneumoniaes or infectious diseases with multiorgan failure, aspiration, trauma or immunodepression. There are no therapeutic guidelines based on controlled studies. Therefore different therapies i. e. high frequency oscillatory ventilation, nitric oxide application, surfactant therapy, extracorporal membrane oxygenation or a combination of these methods are used. We present the case of a 4 (3)/ 12 year old boy, who suffered from an acute lymphatic leukaemia. Caused by immunosuppressive therapy he got a severe broncho-pneumonia. During ventilation therapy an acute respiratory distress syndrome occurred. Due to a surfactant application over 7 days with a doses of 360 mg/kg body weight this RDS could be dominated. The extubation was possible after 17 days of ventilatory support. 3 weeks later the lung function was normalized and the chemotherapy resumed.
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PMID:[Successful surfactant therapy of ARDS in an immunodepressed child]. 1201 42

GG-62 is a cell line previously thought to be derived from an atypical Ewing tumor (ET). Reverse-transcriptase polymerase chain reaction revealed an in-frame fusion between the Ewing sarcoma gene ( EWS) codon 325 and the activating transcription factor 1 gene ( ATF1) codon 65 which permits the production of chimeric EWS-ATF1 oncoproteins. We also identified the genomic breakpoint resulting from a reciprocal t(12;22)(q13;q12), which is the hallmark of malignant melanoma of soft parts (MMSP). We applied Affymetrix human cancer G110 arrays to compare the gene expression patterns of GG-62 and other cell lines derived from small blue round cell tumors of childhood. Hierarchical clustering of 463 differentially expressed genes distinguished GG-62 from the ETs, as well as the neuroblastomas, and revealed a cluster of 36 upregulated genes. Several of these genes are involved in signal transduction pathways that may be critical for maintaining cell transformation; some examples are avian erythroblastic leukemia viral oncogene homolog 3 ( ERBB3), neuregulin 1 ( NRG1), fibroblast growth factor 9 ( FGF9), and fibroblast growth factor receptor-1 ( FGFR1). Furthermore, genes near the chromosome-12q13 breakpoint exhibited increased expression of GG-62 including ERBB3, NR4A1 (nuclear receptor subfamily 4, group A, member 1), cyclin-dependent kinase 2 ( CDK2), and alpha 5 integrin ( ITGA5). Altogether our findings demonstrate the MMSP derivation of GG-62 and may shed light on the mechanisms of tumorigenesis in this rare disease.
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PMID:Characterization of the malignant melanoma of soft-parts cell line GG-62 by expression analysis using DNA microarrays. 1202 21

We reviewed Dutch patients and those described in the literature with congenital leukaemia in the past 25 years, with the intention to obtain an overview of the characteristics of this rare disease. Among the 117 patients reviewed, acute myeloid leukaemia (AML) was more frequent (64%) than acute lymphoblastic leukaemia (ALL, 21%). Most patients had a high leukaemic cell load with hepatosplenomegaly, leukaemia cutis and hyperleucocytosis. Cytogenetic abnormalities were found in the majority of the patients tested (72%); 11q23 abnormalities were found in less than half of them (42%). The probability of overall survival at 24 months was only 23%. When congenital AML and ALL were compared, clinical characteristics and overall survival were not significantly different. However, in patients at risk, the probability of event-free survival (EFS) and disease-free survival (DFS) were significantly higher in AML than in ALL, 43% versus 13% and 68% versus 0% respectively. Among the congenital AML cases, six spontaneous remissions have been described. In conclusion, the clinical characteristics of congenital leukaemia differ from those of leukaemia in older children and prognosis is generally poor. Once complete remission is achieved, patients with AML fare better than those with ALL. Chemotherapy for congenital leukaemia needs improvement to increase the sustained remission rate.
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PMID:Congenital leukaemia: the Dutch experience and review of the literature. 1202 17

IgG-secreting lymphoplasmocytoid leukemia is a rare disease associating splenomegaly and B cell proliferation. Lymphoid phenotype is usually different from chronic lymphocytic leukemia. We report a case disclosed by hyperviscosity syndrome. Diagnosis was confirmed at routine ophthalmologic study.
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PMID:[IgG-secreting lymphoplasmocytoid leukemia revealed by ophthalmologic hyperviscosity symptoms]. 1221 8

The hairy cell leukemia prolymphocytic variant, a subtype of hairy cell leukemia, is an extremely rare disease, especially in Japan. We report a case in which treatment with 2'-deoxycoformycin (DCF) improved the clinical features of the disease. The patient, a 70-year-old female, was first treated with 2-chlorodeoxyadenosine, but showed only transient improvement in the hematological findings. DCF was then administered every week. Following the start of this treatment, the leukemia cell count rapidly decreased and the platelet count simultaneously increased. This effect of DCF has so far been long term. More clinical studies are needed to confirm the therapeutic value of DCF.
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PMID:[Successful treatment of hairy cell leukemia prolymphocytic variant with 2'-deoxycoformycin]. 1222 30

De novo erythroleukemia (EL) is a rare disease. Reported median survival are poor and vary from 4 to 14 months. The value of hematopoietic stem cell transplantation (HSCT) for EL is unknown. This EBMT registry study reports on the largest series of patients with EL treated with HSCT in first complete remission-103 autologous and 104 HLA identical sibling allogeneic HSCT. Outcome and identification of prognostic factors for each type of transplantation were evaluated. For autologous HSCT, outcome at 5 years showed a leukemia-free survival (LFS) of 26% +/- 5%, a relapse incidence (RI) of 70% +/- 6%, and a transplant-related mortality (TRM) of 13% +/- 4%. By multivariate analysis, the only prognostic factor was age. For allogeneic HSCT, outcome at 5 years showed an LFS of 57% +/- 5%, an RI of 21% +/- 5%, and a TRM of 27% +/- 5%. By multivariate analysis, prognostic factors were graft-versus-host disease and age. This study represents the largest series of de novo EL treated with HSCT and shows that allogeneic HSCT is by far the most effective treatment.
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PMID:Hematopoietic stem cell transplantation for de novo erythroleukemia: a study of the European Group for Blood and Marrow Transplantation (EBMT). 1238 10

Congenital leukemia is a rare disease developing within the first 4 to 6 weeks of life. We report a female infant born with facial mass and multiple subcutaneous nodules. The facial mass was discovered by ultrasound during a routine prenatal examination at the 36th week of gestation. Biopsies were consistent with the diagnosis of acute monoblastic leukemia (AML, FAB M5b). Cytogenetic studies showed 46 XX, t(11;19)(q23;p13.1), which is only found in acute monoblastic leukemia and involves the gene. The infant died at 12 days of age and autopsy revealed a large leukemic tumor burden in several body organs. The discovery of the facial mass prenatally and massive extramedullary leukemic burden support the notion of the in utero development of congenital leukemia.
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PMID:Prenatal presentation supports the in utero development of congenital leukemia: a case report. 1257 68

Hematopoietic stem cell transplantation (SCT) is the only proven cure for chronic myeloid leukemia (CML), a rare disease in childhood. We report outcomes of 314 children with Philadelphia-chromosome-positive (Ph+) CML undergoing SCT from HLA-matched siblings (n = 182) or volunteer-unrelated donors (VUD; n = 132). Three-year overall survival (OS) and leukemia-free survival (LFS) rates were 66% and 55% (n = 314). For 156 children in first chronic phase (CP1) who underwent transplantation from HLA-identical siblings, OS and LFS rates were 75% and 63%. For 97 children who underwent SCT in CP1 from VUD, 3-year OS and LFS rates were 65% and 56%, reflecting higher transplantation-related mortality (TRM) after VUD SCT (35% vs 20%; multivariate hazard ratio [HR], 1.9; 95% confidence interval [CI], 1.0-3.5; P =.05). In a multivariate model for OS and LFS, outcomes were superior in CP1 than in advanced phase (AP/CP1) (OS HR, 2.0; 95% CI, 1.3-3; P =.001; LFS HR, 1.8; 95% CI, 1.2-2.6; P =.003). For relapse, donor source (VUD/sibling) (HR, 0.38; 95% CI, 0.19-0.76; P =.006) and disease stage (AP/CP1) (HR, 2.4; 95% CI, 1.36-4.3; P =.003) were significant. This is the first large series to show that SCT confers long-term LFS in most children with CML and helps assess alternative therapy, including tyrosine kinase inhibitors.
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PMID:Stem cell transplantation for chronic myeloid leukemia in children. 1271 25

We report a case of pulmonary tuberculosis, which was preceded by skin tuberculosis. 65-year old male was admitted to our hospital complaining of skin eruption which last one year. Skin biopsy proved granuloma with acid-fast bacilli. Mycobacterium tuberculosis was detected by PCR examination using skin biopsy and skin tuberculosis was confirmed. Chest roentogenography demonstrated small nodules with bilateral infiltrates compatible with pulmonary tuberculosis. M. tuberculosis was attained by culture examination using sputa sample. In this case, skin tuberculosis was a first clinical sign to suggest pulmonary tuberculosis. Peripheral blood test showed that he has developed adult T-cell leukemia and this could be an important factor for developing skin tuberculosis. Although skin tuberculosis becomes rare disease, physician should pay attention for this disease as differential diagnosis of lasting eruption.
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PMID:[A case of pulmonary tuberculosis preceded by skin tuberculosis]. 1451 55

The case of a patient with the aplastic variant of hairy cell leukaemia, successfully treated with the drug Deoxycoformycin(Pentostatin), is presented. It is very important to be aware of this rare variant of a rare disease so that the right treatment can be offered.
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PMID:Successful treatment of aplastic variant of hairy-cell leukaemia with deoxycoformycin. 1497 88


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