Severe idiopathic neutropenia

Severe Idiopathic Neutropenia

Neutropenia is a condition in which the body doesn’t have enough neutrophil, a type of white blood cell necessary for fighting infection. Severe Idiopathic Neutropenia (SIN) is a rare, inherited or acquired form of neutropenia, which can cause an increased risk of infections and other major health problems.

Causes

The cause of SIN is not known in most cases. In some cases, SIN is inherited, and can be caused by a change (mutation) in certain genes that have been linked to the condition. In other cases, SIN can be acquired, usually due to an underlying medical condition, such as bone marrow failure, use of certain medications, chemotherapy or radiation, or a viral infection.

Symptoms

Infections caused by SIN can vary in severity. Individuals with SIN can experience:

  • Fever
  • Chills
  • Fatigue
  • Cough
  • Sore throat
  • Diarrhea
  • Skin rashes or lesions
  • Shortness of breath
  • Joint pain

Diagnosis

SIN can be diagnosed through a complete blood count and other laboratory tests to measure the number and types of white blood cells. Additional tests may be needed to look for an underlying cause, such as a viral infection or genetic mutation.

Treatment

Treatment for SIN depends on its underlying cause, severity, and the individual. Treatment can include antibiotics to treat or prevent infections, medications to stimulate neutrophil production, such as granulocyte-macrophage colony-stimulating factor (G-CSF), and other medications to reduce the risk of infection and support general health.