Severe congenital neutropenia unresponsive to G-CSF.

Abstract:

:Severe congenital neutropenia (SCN) is an inherited disorder characterized by severe neutropenia and recurrent infections from an early age, with bone marrow showing a maturational arrest of granulopoiesis at the promyelocyte stage. Since the introduction of G-CSF therapy the prognosis for affected children has improved dramatically. We describe two patients with SCN who were clinically unresponsive to G-CSF therapy. The results of in-vitro colony assays from these two patients are presented together with the results from the mother of one of these patients who also has a chronic neutropenia, and a further child with SCN who responded to treatment with G-CSF.

journal_name

Br J Haematol

authors

Ryan M,Will AM,Testa N,Hayworth C,Darbyshire PJ

doi

10.1111/j.1365-2141.1995.tb05242.x

subject

Has Abstract

pub_date

1995-09-01 00:00:00

pages

43-5

issue

1

eissn

0007-1048

issn

1365-2141

journal_volume

91

pub_type

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