Yuriy N. Kuznetsov

• Medical Doctor at First Pavlov State Medical University of St. Petersburg

Aim. To evaluate the hematological response within 4 weeks of iptacopan monotherapy in patients with paroxysmal nocturnal hemoglobinuria and suboptimal response to long-term eculizumab therapy. Materials and methods. The analysis included 8 patients, median age 32 years, with persistent anemia on long-term therapy with eculizumab. The hematological...

BACKGROUND. Inherited bone marrow failure syndromes (IBMFS) is a heterogenous group of rare genetically determined diseases with variable hematologic and nonhematologic manifestations. The implementation of highly specific methods of genetic diagnosis advanced the understanding of IBMFS and allowed its application also beyond pediatrics. That presu...

Here we present a rare case of Fanconi anemia in a 31-year-old Syrian male patient at the early stage of MDS/AML transformation. His bone marrow cells showed 1q and 3q gains combined with severely sup- pressed TP53 gene expression, along with low WT1 and BAALC gene expression.

A discovery of nonrandom recurrent interstitial aberration at the long arm of chromosome 5 was made by Van den Berghe et al. in 1974. For a long time, this entity was classified as myelodysplastic syndrome (MDS). Meanwhile, its definition as well as classification criteria were repeatedly changed due to both clinical studies and advances in new tec...

Portable non-invasive ventilation (PNIV) is a modern option of respiratory support in the treatment of acute and chronic respiratory failure. Efficiency of PNIV was previously noted in patients with obstructive sleep apnea syndrome, ventilation-type respiratory failure in chronic obstructive pulmonary disease, bronchial asthma and lung damage in t...