Published online December 1, 2004
PEDIATRICS Vol. 114 No. 6 December 2004, pp. e757-e760 (doi:10.1542/peds.2004-0124)
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ELECTRONIC ARTICLE

Clostridium septicum Myonecrosis in Congenital Neutropenia

Chris Barnes, MB*, J. Ted Gerstle, MD{ddagger}, Melvin H. Freedman, MD* and Manuel D. Carcao, MD*

* Division of Haematology/Oncology, Department of Paediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
{ddagger} Division of Pediatric General Surgery, Department of Surgery, Hospital for Sick Children, Toronto, Ontario, Canada

Severe congenital neutropenia (SCN) and Clostridium septicum myonecrosis is an uncommon and life-threatening association requiring urgent combined aggressive medical and surgical management. We report 2 cases of SCN (1 with known Kostmann's syndrome and 1 not known at presentation to have a congenital neutropenic disorder but subsequently received a diagnosis of cyclic neutropenia) who presented with spontaneous C septicum myonecrosis. The cases highlight the importance of response to recombinant human granulocyte colony-stimulating factor in obtaining a satisfactory outcome for these patients. Early, empirical use of recombinant human granulocyte colony-stimulating factor in patients who are suspected of having a congenital neutropenia and who present with life-threatening sepsis is recommended.

Key Words: congenital neutropenia • clostridium septicum • rh-GCSF

Abbreviations: SCN, severe congenital neutropenia • rh-GCSF, recombinant human granulocyte colony stimulating factor

Accepted Jun 30, 2004.