Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Contemporary treatments have resulted in 5-year event-free survival rates (EFS) of approximately 75% to 80% for childhood acute lymphoblastic leukemia (ALL). Relapses of ALL in children were more often in HR-ALL but also in very few non-HR-ALL. Thus current clinical study of ALL has focused on improving the outcome of a few subtypes of HR-ALL. Infants with ALL have a particularly high risk of treatment failure. Infant ALL Interfant-99 study found that MLL rearrangement, age younger than 6 months, poor response to a prednisone prophase and high WBC count were strong independent predictive factors for poor prognosis in infants with ALL. Treatments with hybrid protocol, including both lymphoid- and myeloid-directed treatment elements, also contain HD-MTX and high dose Ara-C(HD-Ara-C), will further improve the outcome for infant ALL. Children Philadelphia chromosome positive ALL (Ph+ALL) was associated with a high relapse rate when treated with chemotherapy alone. The Children's Oncology Group (COG) AALL0031 trial showed that the addition of tyrosine kinase inhibitors(TKIs) imatinib to intensive chemotherapy resulted in 3-year EFS more than historical control treated with chemotherapy alone. These findings create a new paradigm for integrating molecularly targeted agents with intensified chemotherapy. Children with T-ALL have had a worse outcome than with the precursor B-cell ALL previously. With more intensified chemotherapy , outcomes for children T-ALL were improved, approaching those for the precursor B-cell ALL. Recently, COG decided to treat children with T-cell ALL with separate protocols different from those for the precursor B-cell ALL, and the protocols of BFM for children with T-ALL have been the same as those of the precursor B-cell ALL. Early precursor T-cell ALL, a novel subtype of T-cell ALL, was identified by gene expression profiling, flow cytometry, and single nucleotide polymorphism array analyses. ETP-ALL, identified in 13% of T-cell ALL cases, is characterized by a distinctive immunophenotype (CD1a and CD8 negativity, with weak expression of CD5 and co-expression of stem cell or myeloid markers). A retrospective analysis suggested that ETP-ALL had a poor prognosis. The advent of high-resolution genome-wide analyses has provided new insights into leukemogenesis and identified many novel subtypes of leukemia. Ph-like ALL (BCR-ABL1-like ALL) patients lack a previously identified chromosomal rearrangement, but exhibit a gene-expression profile highly similar to that of Ph+ALL, and often have deletion/mutation of IKZF1, CRLF2 rearrangments and JAK1/2sequence mutations which are also common in Ph+ALL. The prognosis of Ph-like ALL is poor. Development of new agents targeted to leukemogenic pathways will promise to further improve the outcome of children ALL.
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PMID:[Therapeutic strategies for childhood high-risk acute lymphoblastic leukemia]. 2359 60

T-prolymphocytic leukemia (T-PLL) is a rare T-cell neoplasm with an aggressive clinical course. Leukemic T-cells exhibit a post-thymic T-cell phenotype (Tdt-, CD1a-, CD5+, CD2+ and CD7+) and are generally CD4+/CD8-, but CD4+/CD8+ or CD8+/CD4- T-PLL have also been reported. The hallmark of T-PLL is the rearrangement of chromosome 14 involving genes for the subunits of the T-cell receptor (TCR) complex, leading to overexpression of the proto-oncogene TCL1. In addition, molecular analysis shows that T-PLL exhibits substantial mutational activation of the IL2RG-JAK1-JAK3-, STAT5B axis. T-PLL patients have a poor prognosis, due to a poor response to conventional chemotherapy. Monoclonal antibody therapy with antiCD52-alemtuzumab has considerably improved outcomes, but the responses to treatment are transient; hence, patients who achieve a response to therapy are considered for stem cell transplantation (SCT). This combined approach has extended the median survival to four years or more. Nevertheless, new approaches using well-tolerated therapies that target growth and survival signals are needed for most patients unable to receive intensive chemotherapy.
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PMID:Advances in the understanding and management of T-cell prolymphocytic leukemia. 2926 69