Bernard Syndrome

Bernard syndrome

Bernard syndrome is a rare hereditary disease of the blood system, characterized by decreased blood clotting.

This disease was first described in 1961 by French hematologist Jean Bernard (1907-2006). Hence the name - Bernard syndrome.

The cause of the disease is a deficiency of the protein factor IX, which is involved in blood clotting. This deficiency leads to increased bleeding.

The main symptoms are frequent and heavy bleeding, including nosebleeds, gum bleeding, hemorrhages in muscles and joints.

Treatment consists of regular administration of clotting factor IX drugs. The prognosis with timely diagnosis and treatment is favorable.

Thus, Bernard syndrome is a rare hereditary bleeding disease described by the French hematologist Jean Bernard. If the doctor's recommendations are followed, patients can lead a normal life.



**Bernard's syndrome** (b-n B.) A rare hereditary disease of the hematopoietic system, characterized by the proliferation of erythrokaryocytes and impaired maturation of reticulocytes in the patient's bone marrow, as well as an increase in the size of the spleen and liver, is one of the most interesting and rare evolutionary pathologies. B. Vernaille first described and characterized a rare type of hemoblastosis in 1892. The main reason for the development of this theory is the work of scientists - J. P. Bordier and E. Deicher. These researchers regarded this type of tumor as lymphoma [1-3].

On June 1, 1916, two French hepatologists, Drs. Cassen and Dr. Cunnet, read in the Societe des hopitaux de Paris a report of several new cases of a disease that J.B. later called "Bern's syndrome." Based on an analysis of previous cases of Bernadian transformation, they came to the conclusion that it represents a malignant transformation of a normal blood cell. They also described the main features of the blood rearrangement: the most important feature was that it was a rare form of cancer with an unusual morphology and clinical presentation. Transposition of blood vessels