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Severe Chronic Neutropenia

Abstract

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NORD is very grateful to Laurence A. Boxer, MD, Henry and Mala Dorfman Family Professor of Pediatric Hematology/Oncology, University of Michigan, for assistance in the preparation of this report.

Synonyms of Severe Chronic Neutropenia

  • SCN

Disorder Subdivisions

  • chronic congenital neutropenia
  • chronic idiopathic neutropenia
  • cyclic neutropenia

General Discussion

Severe chronic neutropenia (SCN) is a rare blood disorder characterized by abnormally low levels of certain white blood cells (neutrophils) in the body (neutropenia). Neutrophils play an essential role in fighting bacterial infections by surrounding and destroying invading bacteria (phagocytosis). Symptoms associated with severe chronic neutropenia include recurring fevers, mouth sores (ulcers), and/or inflammation of the tissues that surround and support the teeth (periodontitis). Due to low levels of neutrophils, affected individuals may be more susceptible to recurring infections that, in some cases, may result in life-threatening complications. SCN may last for months or years and can affect both children and adults. Clinicians recognize three forms of the disorder: congenital, cyclical, and idiopathic neutropenia. The term idiopathic neutropenia is used when severe chronic neutropenia occurs for unknown reasons.

Organizations related to Severe Chronic Neutropenia

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