Sometimes called Kostmann Syndrome this type of Neutropenia is generally present at birth. It is usually very severe since neutrophils are often completely absent. Patients usually show what is known as a maturation arrest in the early stages of neutrophil development in the bone marrow. This means that their neutrophils rarely fully mature into the cells that are capable of fighting infections. This type is known to often benefit from G-CSF treatments.
This type of Neutropenia may occur at any time in life for unknown reasons. The onset of idiopathic Neutropenia is possible in both, children and adults. Most patients respond well to G-CSF treatment but require long-term treatment.
Cyclic Neutropenia is another inherited type of Neutropenia. This type shows a cyclic pattern of varying neutrophil counts 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. The frequency of bacterial infections depends on the length of the neutropenic period that the patient experiences. Those who have a longer neutropenic period within the cycle suffer more frequently from infections.
Cyclic Neutropenia occurs because of fluctuating rates of cell production by the bone marrow stem cells. Other blood cells, such as platelets or red cells can also show oscillations with a cyclical pattern. Cyclic Neutropenia can occur sporadically, but there are families in which cyclic Neutropenia is inherited with one parent and more than one child affected. As in Kostmann syndrome, patients with cyclic Neutropenia also benefit from G-CSF treatment.
This type involves antibodies in the blood that actually attack the body’s own neutrophils. This is the most common cause for Neutropenia of this age group. Although these infants lack peripheral blood neutrophils, they usually do not suffer from severe bacterial infections.
Granulocyte-specific antibodies are detectable by different immunological blood tests that can be performed in specialized laboratories. If these antibodies are identified, the patients should be kept under medical care, but may not necessarily require treatment with antibiotics or G-CSF.
Depending on the frequency of infections and the neutrophil counts, prophylaxis with an oral antibiotic may be considered by the treating physician. In some children where severe infections occur, treatment with G-CSF is indicated. In most children the blood counts normalise during the first 2-3 years.
Autoimmune Neutropenia is occasionally seen in young people (20 – 40 year age group) predominantly women and in this setting is often associated with other disorders or conditions.
Neutropenia and Other Diseases
Chronic neutropenia may be present in the following diseases. For more information click on the links below.