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severe chronic neutropenia

Tuesday 18 November 2003

Etiology

- severe congenital neutropenia and cyclic neutropenia (mutations in the neutrophil elastase gene)
- myelokathexis (downregulation of the bcl-x protein)
- glycogen storage disease type 1b
- Chediak-Higashi syndrome
- Shwachman-Diamond syndrome
- dyskeratosis congenita
- Griscelli syndrome
- Barth syndrome
- Wiskott-Aldrich syndrome

- idiopathic neutropeni
- pure white-cell aplasia
- myelodysplasia
- aplastic anemia

Pathology

- severe infection
- perianal fistula (2683920)
- Crohn-like disease (1442691, 2066550, 7125837)

References

- Crohn’s disease associated with cyclic neutropenia. Lamport RD, Katz S, Eskreis D. Am J Gastroenterol. 1992 Nov;87(11):1638-42. PMID: 1442691

- Crohn’s disease associated with autoimmune neutropenia. Stevens C, Peppercorn MA, Grand RJ. J Clin Gastroenterol. 1991 Jun;13(3):328-30. PMID: 2066550

- [Chronic neutropenia and Crohn’s disease in childhood. Report of 2 cases]. Vannier JP, Arnaud-Battandier F, Ricour C, Schmitz J, Buriot D, Griscelli C, Rey J. Arch Fr Pediatr. 1982 Jun-Jul;39(6):367-70. PMID: 7125837