Özet:

Purpose: Myelodisplastic syndrome (MDS) is the clonal root cell disease of the bone marrow characterized by cytopines as a result of the dysplasia of hematopoetic primary cells in the bone marrow. In the bone marrow, both the three erythrostic, megacaryostic and granulostic series can be affected. Paroxysmal pointal hemoglobinuria (PNH) is a life-threatening, non-malign hematopoetic root cell disease of the bone marrow. This study is planned to assess both the level and frequency of PNH clone in patients with low-risk MDS diagnosis. Method: The study was planned as a retrospective observation study. The normal distribution of the data was assessed by the Kolmogorov-Smirnov test. The normal distribution of the numerical variables is indicated as the average±standard deviation, the number and percentage of the categorical variables. According to the existence of PNH, the difference of numerical variables was assessed by the Studen T test and Mann Whitney U. Results related to mortality were evaluated by univariable Cox regression analysis. Between 2010-2019 patients with myelodisplastic syndrome followed in the Hematology BD of the Mersin University Medical School. Results: The study consisted of 13 patients with PNH clones and 176 patients without PNH clones of a total of 189 MDS patients. The number of patients with PNH clone was estimated at 7%. The average age of patients is 64.5±13 years, the female ratio is 51.8%, the hyposellularity of the bone marrow is higher compared to those with non-PNH clone (61.5% vs 16.5%; p=0. 001 was found. Reduced hematocrit levels compared to those who do not have PNH clone, increased lactic dehydrogenase rates, increased revised international prognostic score are identified as possible risk factors associated with mortality. Result: In the presence of a hypocellular bone marrow found in some low-risk MDS patients, PNH clone analysis is recommended for patients with non-blasted refractor anemia, PNH clone analysis is recommended if it accompanies high LDH to the cytopeni table.

Anahtar Kelimeler:

Özet:

Aim: Myelodysplastic syndrome (MDS) is a clonal stem cell disease of bone marrow characterized by dysplasia of hematopoietic precursor cells in bone marrow. Paroxysmal nocturnal hemoglobinuria (PNH) is a life-threatening, clonal, non-malignant hematopoietic stem cell disease of the bone marrow. This study was planned to evaluate both PNH clone level and frequency in patients with low-risk MDS. Methods: The normal distribution of the data was evaluated by Kolmogorov-Smirnov test. Normal distribution of numerical variables was expressed as mean ± standard deviation, and categorical variables were expressed as number and percentage.  Differences of numerical variables according to the presence of PNH were evaluated by Student T test and Mann Whitney U test. Myelodysplastic syndrome patients followed up in Mersin University Medical Faculty Hematology Department between 2010-2019 were included in the study. Results: The study consisted of 13 patients with PNH clones and 176 total 189 MDS patients without PNH clones.  The rate of patients with PNH clone was 7%. The mean age of the patients was 64.5 ± 13 years, female rate was 51.8%, and the rate of hypocellularity of bone marrow was higher (61.5% vs 16.5%; p = 0.001). Decreased hematocrit levels increased lactic dehydrogenase ratio, increased revised international prognostic score were identified as possible risk factors associated with mortality compared to those without PNH clones. Conclusion: In the presence of a hypocellular bone marrow in some of the low-risk MDS patients, PNH clone analysis is recommended for patients who go with refractory anemia without blast increase, and PNH clone analysis if cytopenia is accompanied by high LDH.