Thiol/Disulfide Balance in Older Patients with BCR-ABL Negative Myeloproliferative Neoplasms.

BACKGROUND: The trio Essential Thrombocytosis (ET), Polycythemia Vera (PV), and Primary Myelofibrosis (PM) are BCR-ABL negative myeloproliferative neoplasms. All three diseases have the risk of transforming into acute leukemia. Oxidative stress and some genetic mutations increase the risk of leukemic transformation. The median age in patients with ET, PV, and MF is around 64 years, and it is expected to exceed 65 in the coming years. Since oxidative stress increases with age, we aimed to evaluate the oxidative stress parameters in older patients with myeloproliferative neoplasms. METHODS: The study included a total of 160 patients (57 patients with Essential Thrombocytosis, 52 patients with Primary Myelofibrosis, and 51 patients with Polycythemia Vera) and 56 healthy controls, aged 65 and over. Ischemia Modified Albumin (IMA) and thiol parameters (native thiol, total thiol, and disulfide) were studied from serum samples taken at the time of diagnosis. RESULTS: The median age of the patients was 69 (65 - 85) years. Patients had higher levels of IMA and lower levels of thiol compared to the control group (p < 0.001). When evaluated according to disease subgroups, it was observed that the highest IMA levels and the lowest thiol levels were in patients with PM (p < 0.001). Higher IMA levels and lower native thiol levels were found in patients with the ASXL1 mutation (p < 0.001). CONCLUSIONS: Serum IMA and thiol levels are also significantly changed in older patients with BCR-ABL negative myeloproliferative neoplasia. Changes in these markers are independent of age. Disease-associated mutations such as ASXL1 can also affect the serum levels of these markers.

Which Markers Should the used for Diagnostic Chronic Lymphocytic Leukemia Immunophenotyping Scoring System by Flow Cytometry?

BACKGROUND: The scoring system used for chronic lymphocytic leukemia (CLL) cannot make an accurate diagnosis in some cases. Novel markers are available for the differential diagnosis of CLL, especially from MCL. However, these markers are still not incorporated into diagnostic algorithms. We investigated the role of CD43, CD81, CD200, and ROR1 in the differential diagnosis of CLL and their expression in non-CLL cases. METHODS: We investigated the role of CD43, CD81, CD20, and ROR1 in the differential diagnosis of CLL by incorporating them into the diagnostic panel after studying peripheral blood or bone marrow samples of 165 patients with 8-color flow cytometry. RESULTS: CD43 positivity was a sensitive marker but had a lower specificity for CLL. CD43 had high diagnostic value for CLL (sensitivity 100%, specificity 88.5%, AUC 98.0%). CD200 was a specific marker for CLL (sensitivity 98%, specificity 90%, AUC: 96%). CD81 expression was highest in the MCL cases, with a median expression rate of 68.5% (range: 54 - 82.5%). It was negative in all the CLL cases. For CLL, CD81 negativity had a sensitivity of 95%, a specificity of 82% and an AUC of 92%. ROR1 was positive in all CLL and MCL cases. CD79b, on the other hand, was a fairly sensitive and specific marker for MCL. CONCLUSIONS: CD43, CD81, CD200, and ROR1 should be incorporated into diagnostic algorithms for the differential diagnosis of CLL, especially from MCL.