Farhoodi A, Ahangari Gh, Chavoshzadeh Z, Ramyar A, Movahedi M, Ghareghozlou M, Heydarzadeh M, Fazlolahi M, Bemanian M H, Zandieh F, Mansori M,
Volume 65, Issue 7 (10-2007)
Abstract
Background: Mutations of ELA2, the gene encoding neutrophil elastase (NE) are known to be associated with cyclic neutropenia (CN) and severe congenital neutropenia (SCN). However, high variability of these mutations has been reported. This study was designed to describe the analysis of the ELA2 gene, clinical manifestations and demographic characteristics in patients with CN and SCN.
Methods: A series of 21 patients with CN or SCN were selected, based on SCINR criteria, from the immunology ward of the Pediatric Medicine Center, Tehran, Iran, from March 2004 to August 2005. The ELA2 gene, isolated from blood samples, was analyzed using RT-PCR and automated capillary sequencing. Informed consent was obtained under the tenets of the Helsinki Declaration and the Ethical Committee of the Tehran University of Medical Sciences.
Results: Kostmann's syndrome and CN was diagnosed in three and 18 patients respectively. Of all the patients, one or two mutations were found in 18 cases (85.7%), including all three patients with SCN and 15 of the patients with CN. Exons two and four had the most mutations (eight and seven cases, respectively). Seven patients had double mutations in two distinct exons. Overall, 16 different mutations were found. At the time of presentation, the mean age of patients was 13.4 ±17.6 months, ranging from one month to seven years. Overall, 61.9% of patients had consanguineous parents. The mean absolute neutrophil count was 830.5 ±419.4 (150-2000)/mm3. On average, each patient had been admitted to the hospital 2.2 ±1.6 times. The neutrophil counts of the SCN patients were significantly higher than those of the CN patients. However, there was no significant difference in the neutrophil counts between patients with mutations and those without mutations. All patients with SCN had two or more infectious complications, although the prevalence of infectious or non-infectious complications did not correlate with ELA2 mutations or the neutropenic disorders.
Conclusion: Mutations in ELA2 appear to play an important role in the phatogenetic mechanisms of CN and SCN. Patients with CN had significantly higher neutrophil counts than SCN patients with CN. Although it possible for the gene encoding neutrophil elastase to have more than one mutation in distinct exons, we found no association between the mutations in ELA2 and their complications in CN and SCN patients.
Ahmadinejad Z, Soleimani Ar,
Volume 68, Issue 3 (6-2010)
Abstract
Background: Fever in neutropenic patients is a medical emergency which may happen in patients undergoing chemotherapy. The definition of neutropenia varies from institution to institution but is usually defined as an absolute neutrophil count (ANC)
< 500 cells/l or < 1,000 cells/l with a predicted nadir of < 500 cells/l. Bacterial and fungal infections are the most important in neutropenic patients. Viral infections with agents such as herpes simplex virus and cytomegalovirus are common but less than other pathogens.
Case presentation: We report a patient with fever & neutropenia following cytomegalovirus infection during chemotherapy course for breast cancer.
Conclusion: Although fever and neutropenia after cytomegalovirus infection is not very common but prompt diagnosis and treatment of this disease reduces the mortality and morbidity associated with cytomegalovirus. For this reason, screen testing for CMV
infection in high risk patients including patients with cancer and preemptive therapy in patients with viremia, for prevention of CMV disease could be considered as a strategy for prevention of CMV infection.
Sogol Shirzad, Zeinab Karimi, Mehdi Mohsen Zadeh , Masoud Mohammadi,
Volume 81, Issue 7 (10-2023)
Abstract
Background: Neutropenia refers to a decrease in the absolute number of neutrophils in the blood circulation, certain drugs are used in connection with the treatment of neutropenia. Therefore, the aim of this study is to investigate and compare the efficacy of filgrastim and lenograstim drug treatment in patients with neutropenia in a systematic review.
Methods: This study is a systematic review study conducted in connection with the comparison of the effectiveness of filgrastim and lenograstim in neutropenic patients based on the search in Google scholar, PubMed, ScienceDirect, Irandoc, SID, Magiran databases in the time range of January 2000 to August 2023. This systematic review was based on the criteria of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement, including systematic search of databases, organization of documents for review, selection of studies, information extraction and finally presentation of the final report. The keywords used for searching in this study were selected based on published primary studies and MESH, and after a detailed examination of the study questions, they were selected according to the PECO criteria.
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Results: 1099 articles were identified in the review of the investigated databases, and after removing duplicate articles, unrelated articles, as well as articles that did not have access to their full text or did not have the required information, eight studies were the final phase, and were checked. Out of the eight selected articles, three articles declared the effectiveness of lenograstim more than filgrastim. Two articles mentioned the greater effect of filgrastim and three articles published in recent years declared the effect of two drugs to be the same. Among these articles, the studies that considered the drug dosage to be the same in the investigated groups and the studies that had a larger statistical population in order to generalize to the society are more important.
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Conclusion: According to studies conducted in recent years, both filgrastim and lenograstim recombinant drugs have the same effectiveness in the treatment of neutropenia.
