
Cyclic neutropenia - Wikipedia
Cyclic neutropenia (CyN) is a rare hematologic disorder and form of congenital neutropenia that tends to occur approximately every three weeks and lasting for few days at a time due to changing rates of neutrophil production by the bone marrow.
Cyclic Neutropenia, Congenital and Idiopathic Neutropenia | Blood ...
2017年12月7日 · Background: Cyclic neutropenia (CyN) is an autosomal dominant disease usually attributable to mutations in the gene for neutrophil elastase, ELANE. The diagnosis depends upon serial blood counts (at the minimum every other day or three days a week for 6 weeks) showing periods of severe neutropenia (absolute neutrophil count [ANC] < 0.2 x 10 9 ...
Cyclic Neutropenia, Congenital and Idiopathic Neutropenia
2017年12月8日 · Background: Cyclic neutropenia (CyN) is an autosomal dominant disease usually attributable to mutations in the gene for neutrophil elastase, ELANE. The diagnosis depends upon serial blood counts (at the minimum every other day or three days a week for 6 weeks) showing periods of severe neutropenia (absolute neutrophil count [ANC] < 0.2 x 10 9 ...
New insights into the pathomechanism of cyclic neutropenia
2020年2月21日 · Cyclic neutropenia (CyN) is a hematologic disorder in which peripheral blood absolute neutrophil counts (ANCs) show cycles of approximately 21-day intervals. The majority of CyN patients harbor ELANE mutations, but the mechanism of ANC cycling is unclear.
Allogeneic Transplant in ELANE and MEFV Mutation Positive …
2017年1月18日 · Cyclic neutropenia (CyN) is a rare autosomal dominant inherited disorder due to the mutation ELANE primarily affecting bone marrow stem cells and is characterized by recurrent neutropenia every 2 to 4 weeks.
Two cases of cyclic neutropenia with acquired
2016年5月26日 · Congenital neutropenia (CN) and cyclic neutropenia (CyN) are rare genetic disorders of hematopoiesis predominantly caused by ELANE mutations. 1-3 Due to overlaps in their genetic profiles, CyN can be distinguished from CN by cycling neutrophil counts, usually at 21-day intervals, in the former.
The Natural History of Cyclic Neutropenia: Long-Term Prospective ...
2012年11月16日 · Cyclic neutropenia (CyN) is an autosomal dominant and sporadically occurring hematological disease first described in 1910. Cyclic neutropenia is usually diagnosed in children before age 2 years based of regularly occurring fever, mouth ulcers, and recurrent skin or respiratory infections.
ELANE gene mutation-induced cyclic neutropenia manifesting as …
CyN is a rare hematological disease caused by gene mutations. The main clinical manifestations are recurrent fever, oral mucosal ulcer, lung infection and periodic decrease of peripheral blood ANC (ANC is often <0. 2 × 10 9 /L and lasts 3–5 days), with an average period of 21 days.
A comparison of the defective granulopoiesis in childhood cyclic ...
Background and objectives: Cyclic neutropenia (CyN) in childhood and severe congenital neutropenia (SCN) are congenital disorders that cause chronic neutropenia. Mutations in the neutrophil elastase gene, ELA2, have been reported in patients with CyN and in those with SCN.
(PDF) Cyclic Neutropenia, Mutations in the ELANE Gene
2021年8月7日 · Cyclic neutropenia (CyN) is a rare hematologic disorder and form of congenital neutropenia that tends to occur approximately every three weeks and lasting for few days at...
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