Successful treatment of neutropenia in the hyper-immunoglobulin M syndrome with granulocyte colony-stimulating factor.

PATIENT

A young boy with hyper-immunoglobulin M (IgM) syndrome had recurrent severe infections, failure to thrive, and chronic neutropenia for 2 years despite treatment with i.v. gammaglobulin (IVIG).

METHODS AND RESULTS

With the addition of granulocyte colony-stimulating factor (G-CSF; Filgrastim, Amgen, Inc., Thousand Oaks, CA), increased doses of IVIG, and prophylactic trimethoprim-sulfamethoxazole, his absolute neutrophil count increased from 0.64 x 10(9)/L to 3.36 x 10(9)/L, and he has been free of significant infection for the past 22 months.

CONCLUSIONS

The use of G-CSF merits consideration in patients with hyper-IgM syndrome and severe neutropenia.