Abstract

GIL-SIERRA, MD  and  BRICENO-CASADO, MP. Management at different levels of neutropenia secondary to glycogenosis: a case report. Rev. OFIL·ILAPHAR [online]. 2021, vol.31, n.4, pp.431-432.  Epub Sep 19, 2022. ISSN 1699-714X.  https://dx.doi.org/10.4321/s1699-714x20210004000019.

Glycogen storage disease type Ib is a rare hereditary metabolic pathology caused by deficiencies in the microsomal transporter of glucose-6-phosphatase. Considering the low incidence of this disease, clinical cases are the main source of information on its management. Neutropenia secondary to glycogenosis type Ib is associated with the appearance of infections and inflammatory bowel disease. The treatment is daily administration of colony stimulating factors (CSF). Previous literature describes the abrupt and immediate drop in the neutrophil count after discontinuation of CSF treatment. We present a case of a woman treated with filgrastim every 48 hours using a lower dose that has not been previously described. The neutrophil count was stabilized above 1 x 109 cells/L without the appearance of infections. On the other hand, the negative impact of public contact along with measures to prevent the appearance of infections during the COVID-19 pandemic was showed. Therefore, this case report can provide information at different levels on the management of patients with glycogen storage disease type Ib.

Keywords : Glycogenosis; filgrastim; neutropenia; COVID-19.

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