Myelodysplastic syndrome in children

Myelodysplastic syndromes (MDS) (preleukemia, malocellular leukemia) are a heterogeneous group of clonal disorders, characterized by abnormal growth of the myeloid components of the bone marrow. Myelodysplastic syndromes are characterized by a violation of normal maturation of the hematopoietic cells and signs of ineffective hematopoiesis. In myelodysplastic syndromes, clonal proliferation occurs at the level of stem cells in the bone marrow, in most cases the production of granulocytes, erythrocytes and platelets is disrupted, which leads to the development of pancytopenia. Another feature of myelodysplastic syndromes is its frequent evolution into acute leukemia, the development of which is preceded by a prolonged cytopenic syndrome. Myelodysplastic syndromes are referred to refractory anemia, more advanced stages of the disease reflect the transformation into myeloid leukemia.

[1], [2], [3]

Symptoms of Myelodysplastic Syndrome

Symptoms of myelodysplastic syndrome are a consequence of pancytopenia and include signs of anemia, bleeding due to thrombocytopenia and infection due to neutropenia. Cellular bone marrow, as a rule, is increased, but it is normal or decreased. The general risk of transformation into myeloid leukemia is 10-20%, patients with PAC have the lowest risk of leukemic transformation (5%), RAAP - T - the largest (50%).

FAB classification of myelodysplastic syndromes

• Refractory anemia (<5% of blasts in the bone marrow).
• Refractory anemia with ringed sideroblasts (<5% of blasts in the bone marrow).
• Chronic myelomonocytic leukemia (> 20% of blasts in the bone marrow).
• Refractory anemia with increased blast content (5-10% of blasts in the bone marrow).
• Refractory anemia with increased blast content in the transformation stage (10-30% of blasts in the bone marrow).

[4], [5], [6], [7], [8], [9], [10],