Pelger's leukocyte anomaly

Pegler's leukocyte anomaly is a disorder of the formation of leukocyte cells, first described in 1947 by the Polish hematologist P. Pegler. The incidence of the disease is 0.5%. More common in men than women. Characterized by erythrocyte and platelet hemolysis, leukopenia. In a hemolyzed blood smear, segmented leukocytes are found, which usually have a regular round-oval shape, but are observed in the form of giant and rod-shaped due to swelling of their cytoplasm. Their size is three times larger than normal. The cytoplasm is basophilic, contains a nucleus, and hyperchromic cell forms are also common; the nuclei usually have a characteristic "transverse" striation. On microscopy of a blood smear, the total white blood cell count may be normal or decreased. The presence of extracellular and intracellular granularity is characteristic. Also noteworthy are plasmacytoid lymphocytes and process lymphocytes (“stigmatic” cells). There is also a decrease in the number of eosinophils and an increase in the number of neutrophils. In some patients with leukocyte anomaly, extramedullary hematopoiesis is observed in the liver, kidneys, stomach, and intestines.

Manifestations of the disease are increased body temperature, general weakness, and itchy skin. Possibly asymptomatic. One of the clinical signs is a triad of symptoms, which was established by P. Pshelensky. The triad includes fever, indigestion, and a feeling of heaviness in the stomach. Rarely, the disease is accompanied by jaundice, hepatosplenomegaly and neurological manifestations. The causes of anemia are intravascular hemolysis, transfusion siderosis, and idiopathic hypersegmental hematopoiesis. The initial symptom is a decrease in the number of leukocytes in the peripheral blood. Leukocytosis, especially neutrophilic, is possible. The ESR increases, which means an increase in purulent-inflammatory processes. To diagnose leukocyte anomalies, an important role is played by studying the blood picture in smears for the presence of atypical forms of leukocytes. Targeted immunological therapy using infusions of antileukotrienes (eg, josamycin) does not provide a complete therapeutic effect. In some patients, it is possible to use platelet aggregation inhibitors. Sometimes