What is Neutropenia?
Neutropenia is a condition, in which the number of blood neutrophilic granulocytes (neutrophils), as a subgroup of the white blood cells or leukocytes, respectively, is diminished.
The level of neutropenia may vary considerably. In general, the blood of healthy adults contains about 1500 to 7000 neutrophils per mm3 (1.5 – 7.0 x 109/l). In children under 6 years of age, the neutrophil count may be lower. The severity of neutropenia generally depends on the absolute neutrophil count and is described as follows:
Mild neutropenia, when the ANC falls below a lower limit of 1500 per mm3 (1.5 x 109/l), but remains higher than 1000 per mm3 (1.0 x 109/l)
Moderate neutropenia, when the ANC falls between 500 per mm3 and 1000 per mm3 (0.5 x 109/l - 1.0 x 109/l)
Severe neutropenia, when the ANC falls below 500 per mm3 (0.5 x 109/l)
The duration of the neutropenia may be short-lived. In short-lived cases, the patient is described as suffering from acute neutropenia. "SCN" stands for "severe chronic neutropenia", summarizing a group of conditions, in which neutropenia persists for more than three months. Neutrophils are very important in defending the body against bacterial infections. Therefore patients with too few neutrophils are more susceptible to bacterial infections.
Congenital neutropenia is a general term used to describe a group of serious but rare inherited hematological disorders, which cause neutropenia with less than 500 neutrophils/mm3 on a continuing basis for many months or years:
- Kostmann-syndrome
- Myelokathexis
- Shwachman-Diamond-syndrome
- Glycogen storage disease type 1b
- Barth's syndrome
- Unclassified congenital neutropenia
In cyclic neutropenia, neutrophil counts show a cyclic pattern with a typical cycle length of 21 days. These cycles vary from patient to patient with some individuals being neutropenic during the whole cycle and others who have low neutrophil counts for only a few days and normal blood counts during the rest of the cycle.
Idiopathic neutropenia describes a group of acquired neutropenias including:
- Virus-induced neutropenia
- Neutropenia due to anti-granulocyte antibodies
- Neutropenias of unknown origin
Therapy options
The decision on what therapy is best for the patient depends directly on an accurate diagnosis of neutropenia. The SCNIR provides assistance and information on the diagnosis of neutropenia on request.
In addition to antibiotic treatment of bacterial infections caused by neutropenia, cytokines may be applied. The majority of patients with severe chronic neutropenia benefits from a G-CSF therapy. Patients with severe chronic neutropenia may call for an outpatient appointment at the neutropenia consultation of the Department of Pediatric Hematology and Oncology of the Medical School Hannover.