Severe congenital cyclic neutropenia: A case report
نویسندگان
چکیده
منابع مشابه
Severe congenital neutropenia: case report
Introduction Neutropenia is defined in the literature as absolute neutrophil counts in peripheral blood of less than 1500 cells/mm3 in more than one year old and less than 2000cells/mm3 in children in the first year old of life. Neutropenia is classified as mild, moderate or severe, and may be congenital or acquired, persistent or not. Kostmann syndrome is a severe neutropenia, the incidence va...
متن کاملA case report of familial cyclic neutropenia.
A 34-year-old female with cyclic neutropenia is reported. Family studies showed that her three sons and her mother were also involved. Oscillations in the blood neutrophil counts were almost regular, with a periodicity of 21 days. Numbers of colony-forming unit--granulocyte macrophage (CFU-GM) formed from the bone marrow cells of normal volunteers co-cultured with the patient's serum or mononuc...
متن کاملNeutrophil elastase in cyclic and severe congenital neutropenia.
Mutations in ELA2 encoding the neutrophil granule protease, neutrophil elastase (NE), are the major cause of the 2 main forms of hereditary neutropenia, cyclic neutropenia and severe congenital neutropenia (SCN). Genetic evaluation of other forms of neutropenia in humans and model organisms has helped to illuminate the role of NE. A canine form of cyclic neutropenia corresponds to human Hermans...
متن کاملA comparison of the defective granulopoiesis in childhood cyclic neutropenia and in severe congenital neutropenia.
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. We examined granulopoietic defects in CyN patients with those in SCN patients. DESIGN AND METHODS Three patients with CyN ...
متن کاملa case report of cyclic neutropenia associated with pyoderma gangrenosum
we present a 24-year-old female referred with non-healing wound of a few days duration on anterior aspect of her right foreleg. biopsy of the wound was reported to be pyoderma gangrenosum on pathologic report. further work up of the patient for high grade fever and occasional leukopenias revealed the diagnosis of cyclic neutropenia. treatment with granulocyte colony-stimulating factor (g-csf) r...
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ژورنال
عنوان ژورنال: International Journal of Applied and Basic Medical Research
سال: 2016
ISSN: 2229-516X
DOI: 10.4103/2229-516x.192598