Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We investigated the effects of L-asparaginase (L-asp) on Epstein-Barr virus (EBV)-positive T/NK lymphoproliferative diseases (EBV-T/ NK-LPDs). Seven doses of L-asp (6,000 U/m2) were administered intravenously, with one dose administered on every alternate day. Five consecutive patients were enrolled. Three patients completed the treatment. The clinical symptoms resolved in 1 patient who started the administration 8 months after the onset, being the earliest among the 5 patients. Her EBV-DNA level in whole blood markedly decreased to 0.08 times of that before treatment, and the level in plasma became undetectable. In the other 2 patients whose administration was started 3 and 3.5 years after the onset, however, a remarkable improvement was not detected. Treatment was discontinued in 2 patients because of disease progression or idiopathic dystonia. The mRNA levels of asparagine synthetase in EBV-infected cells were examined. The level from the patient who responded to L-asp treatment was low, but it did not correlate with the effects in the other patients. Liver dysfunction (grades 2 and 3) was observed in 2 patients and neutropenia (grade 3) was noted in 1 patient. In conclusion, the effect of L-asp as monotherapy in EBV-T/NK-LPDs is limited, and early treatment initiation might be effective.
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PMID:L-Asparaginase monotherapy for EBV-positive T/NK lymphoproliferative diseases: A pilot Study. 2611 30

The autoimmune cytopenias are a related group of disorders in which differentiated hematopoietic cells are destroyed by the immune system. Single lineage disease is characterized by the production of autoantibodies against red cells (autoimmune hemolytic anemia [AIHA]), platelets (autoimmune thrombocytopenia [ITP]) and neutrophils (autoimmune neutropenia [AIN]) whereas multilineage disease may include various combinations of these conditions. Central to the genesis of this disease is the breakdown of central and/or peripheral tolerance, and the subsequent production of autoantibodies by both tissue and circulating self-reactive B lymphocytes with support from T helper lymphocytes. These disorders are classified as primary (idiopathic) or secondary, the latter associated with an underlying malignancy, systemic autoimmune disease, infectious disease or a specific drug. Non-specific immunosuppression with corticosteroids remains the first-line therapy for many of these disorders, and although associated with high response rates, is compromised by significant toxicity and high relapse rates. Management of patients with chronic refractory autoimmune cytopenias who have failed first-line and second-line (cytotoxic immunosuppressant therapy and or splenectomy) is particularly complex, with definitive treatment in select patients requiring hematopoietic stem cell transplantation. Given the toxicity concerns of non-selective immunosuppressants, development of therapeutic regimens that avoid steroids has progressed rapidly in recent decades. [Full article available at http://rimed.org/rimedicaljournal-2016-12.asp].
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PMID:Autoimmune Cytopenias: Diagnosis & Management. 2790 98