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Kostman's syndrome


Kostman's agranulocytosis - the disease very rare, is described for the first time in 1956 in Sweden (Kostmann). At this disease at patients considerable decrease or lack of mature neutrophils in blood and marrow is observed.  These patients are very inclined to  infections.

Reasons of a syndrome of Kostman:

Kostman's syndrome has autosomal and recessive inheritance.

Symptoms of a syndrome of Kostman:

The heavy recuring purulent infections from first months of life are characteristic. Combined manifestations: sometimes delay of intellectual development, nanocephalia, cataract and low-tallness. Marrow with a myeloid hypoplasia with a stop at a promyelocyte stage.
The triad of symptoms is characteristic of a disease: inborn agranulocytosis, lymphopenia, lack of cellular and humoral immunity. At babies the heavy recuring purulent infections from first months of life develop. Lack of lymph nodes, almonds, a thymus gland come to light, anemia and thrombocytopenia sometimes take place. Marrow is miyelokletoch-ny, with lack of myeloid and lymphoid elements.

Внешний вид больного с синдромом Костмана

Outward of the patient with a syndrome Kostman

Treatment of a syndrome of Kostman:

Transplantation of marrow — a choice method at Kostman's syndrome. Antibacterial therapy, anti-staphylococcal globulin is in addition appointed.

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