Neutropenia D70.7

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 29.10.2020

Definition
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Lack of peripherally circulating neutrophil granulocytes. In mild neutropenia, the absolute neutrophil count is between 1000 and 1500/μl, in moderately severe neutropenia between 500 and 1000/μl and in severe neutropenia below 500/μl blood. To calculate the absolute number of neutrophil granulocytes, the leucocyte count is multiplied by the result of the differential blood count (percentage of segment-nuclear and rod-nuclear neutrophil granulocytes and their immature precursors) in the peripheral blood.

Classification
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Mild neutropenia: 1000-1500 neutrophils/µl blood.
Medium neutropenia: 500-1000 neutrophils/µl of blood.
Severe neutropenia: < 500 neutrophils/µl of blood.
Differentiation between acute (< 3 months) and chronic neutropenia (> 3 months).

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Etiopathogenesis
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Hematological causes and immunodeficiencies:
- Panmyelopathy (aplastic anaemia)
- Acute graft-versus-host disease
- Wiskott-Aldrich Syndrome
- Chédiak Higashi Syndrome
- dyskeratosis congenita
- Cartilage-hair hypoplasia
- Hyper-IgM Syndrome
- Xanthogranuloma, necrobiotic with paraproteinemia
- WHIM Syndrome
- Cyclic neutropenia.
autoimmune diseases:
- Systemic lupus erythematosus
- Mixed connective tissue disease (Sharp syndrome)
- Felty syndrome.
infections:
- HIV infection
- Cytomegaly
- Erythema infectiosum (Parvo B19 infection)
- EBV
- Acute viral hepatitis
- Malaria
- Measles.
Metabolic causes:
- Vitamin B₁₂ deficiency.
- Folic acid deficiency
- M. Gaucher.
Other causes:
- Toxins
- Drugs, among other things. .:
  - Caspofungin
  - Ganciclovir
  - Gold Preparations
  - Griseofulvin
  - Infliximab
  - Interferons
  - Lamivudine
  - Metronidazole
  - Retinoids
  - Rifabutin
  - Ticlopidine
  - Zidovudine
  - Numerous cytostatic drugs
- Irradiation.

Clinical features
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Neutropenia > 1000/µl are mostly asymptomatic; neutropenia < 1000/µl are clinically characterized by chronic and recurrent infections (mostly bacterial) such as aphthous stomatitis, abscesses, otitis media, tonsillitis, pneumonia. Fever attacks, sepsis. Signs of inflammation are attenuated.

Diagnosis
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Anamnesis and clinic, determination of the absolute neutrophil count, in case of chronic neutropenia 2 blood count controls/week over 6 weeks.
In case of clinical symptoms or severe infections, further diagnostics to exclude immune defects, malignant diseases, HIV infection, SLE.
Detection of autoantibodies against neutrophil granulocytes with simultaneous anaemia and/or thrombopenia or blasts in the peripheral blood smear. Bone marrow aspiration e.g. of a malignant infiltration of the KM.

Therapy
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Stopping suspicious medication, treatment of underlying diseases, antibiotic therapy (after taking blood cultures and smears). Subcutaneous application of granulocyte colony stimulating factor (G-CSF); in severe congenital forms: stem cell or KM transplantation.