Severe chronic neutropenia: Treatment and follow‐up of patients in the Severe Chronic Neutropenia International Registry

Abstract

Severe chronic neutropenia (SCN) is defined as an absolute neutrophil (ANC) of less than

Safety and efficacy of allogeneic PBSC collection in normal pediatric donors: The Pediatric Blood and Marrow Transplant Consortium Experience (PBMTC) 1996–2003

“…In spite of thousands of patient years of cytokine therapy, no patients with cyclic or idiopathic neutropenia treated with G-CSF and registered in the Severe Chronic Neutropenia International Registry have been reported with progression to cancer. 22 Approximately 10% of patients in that registry with congenital neutropenia (Kostmann syndrome) who have been treated with G-CSF progressed to MDS or AML, but this rate is similar to the rate of progression without G-CSF therapy and thus is likely to be related to the underlying disease, rather than the growth factor exposure. A similar lack of evidence of long-term adverse hematologic effects has been noted in a 2-year follow-up of neonates treated with G-CSF for sepsis.…”

Hematopoietic stem cell transplantation in severe congenital neutropenia: experience of the French SCN register

“…19 However, the success rate of HSCT for AML or MDS is low, as 17 of the 24 relevant patients included in the International Severe Chronic Neutropenia Registry died. 7 The need for chemotherapy prior to HSCT is controversial. 20 It can be useful for patients with overt leukemia, as in patients who have acute lymphoblastic leukemia, 13,21,22 but it can be deleterious in other patients as it may contribute to increasing infection risk.…”

“…6 Other major complications are leukemia and the myelodysplastic syndrome (MDS), the latter affecting about 10% of patients. 7 The use of G-CSF therapy has radically changed these patients' outlook. 8 However, a minority of patients is refractory to G-CSF, and some undergo malignant transformation.…”

Anti‐G presenting as a blood transfusion error

“…Three types of severe chronic neutropenia (SCN) are known: the congenital, the cyclic, and the idiopathic form [1][2][3][4][5][6][7]. All three types can be effectively treated with long-term G-CSF [8].…”