Periodic neutropenia

June 26, 2018 20:00 | Disease Treatment
This is a rare disease that can occur in children in infancy and older age, as well as at any age in adults. Typical symptoms of this symptom complex include recurrent granulocytopenia, recurring in 2-3-4 weeks. In some cases, general leukopenia is noted, while in others, with a normal number of leukocytes, a selective decrease in the number of neutrophilic granulocytes. Sometimes the deficit of nuclear leukocytes is compensated by monocytosis.

The periods of neutropenia, which can last from several to 10 days, are accompanied by various clinical symptoms( general weakness and weakness, chills, fever, furunculosis, sore throat, upper respiratory tract infection, conjunctivitis, pyoderma).In some cases, there are joint pains and ulceration of the mucous membrane of the throat and rectum, sometimes there is a transient increase in the spleen, lymph nodes, pain and abdominal cavity. It should be noted that these symptoms can be 2-3 days ahead of the fall in the number of granulocytes.

The study of bone marrow in the neutropenia period shows a pronounced weakening of granulopoiesis in the form of delayed maturation of granulocytes and a decrease in the number of segmented forms. During the periods of remission, children feel well, with relapses of leukopenia their condition deteriorates sharply.

The pathogenesis of of this symptom complex is unknown.

Treatment of reduces to the use of antibiotics, blood transfusion or leukocyte mass, as well as ACTH and corticosteroids. Hormone therapy can lead to the disappearance of clinical symptoms of the disease, but after stopping hormones, the symptoms of the disease are resumed. There were attempts to remove the spleen. In some cases, after splenectomy, there was a marked improvement in the clinical picture, which was sometimes held up to 1/2 year. It should be emphasized, however, that splenectomy did not have a distinct effect on the periodic nature of leukopenia, it only softened the accompanying clinical symptoms.

Read more Chronic granulocytopenia of childhood
Female journal www. BlackPantera.en: Magdalena Berger