Myelodysplastic Syndromes: From Inflammatory to Therapeutic Approaches
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Editor
Prof. Dr. Thorsten Braun
Prof. Dr. Thorsten Braun
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Department of Hematology, Avicenne Hospital, Assistance Publique-Hôpitaux de Paris, Bobigny, France
Interests: MDS; CMML; Treg; ruxolitinib; AML progression
Topical Collection Information
Dear Colleagues,
Impaired function of immune cells and aberrant inflammatory response of the immune system is frequently observed for patients with myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML). Increased secretion of inflammatory cytokines, including tumor necrosis factor-(TNF)-α and interferon (IF)-γ, contributes to cell death of myeloid precursor cells and triggers transformation to acute myeloid leukemia (AML). For CMML patients, a phospho-STAT5 hypersensitive response to GM-CSF has been identified that is positively correlated with high-risk disease. Altered function of cytotoxic, regulatory (Treg), helper (Th17) T cells, and natural killer (NK) cells contribute to cytopenias and disease progression. Autoimmune disorders (AD) observed for MDS and CMML patients include vasculitis, seronegative polyarthritis, relapsing polychondritis, or neutrophilic dermatosis. In a subset of patients, cytopenias are the consequence of autoreactive cytotoxic T cells and respond to immunosuppressive treatment (IST). Interestingly, despite IST, other drugs are of interest by treating MDS and CMML-associated AD and inflammatory response as the hypomethylating agent azacytidine improves cytopenias, delays disease progression, and controls AD by sparing corticosteroids and other immunosuppressive drugs. The immunomodulatory drug lenalidomide improves anemia in specific subtypes of MDS and inhibitors of the JAK-STAT pathway should be evaluated for the treatment of selected MDS and CMML patients.
Prof. Dr. Thorsten Braun
Guest Editor
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Keywords
- MDS
- CMML
- AML
- Autoimmune disorders
- Azacytidine
- Ruxolitinib
- Imids
- Cytokines
- T cells
Published Papers (1 paper)
2023
Open AccessArticle
MDS-Related Anemia Is Associated with Impaired Quality of Life but Improvement Is Not Always Achieved by Increased Hemoglobin Level
by
Yael Haring, Noa Goldschmidt, Shaimaa Taha, Galia Stemer, Kalman Filanovsky, Ilana Hellman, Doaa Okasha, Baher Krayem, Itai Levi, Hanna Rosenbaum, Maya Koren-Michowitz, Shai Yagna, Anatoly Nemets, Sharon Gino-Moor, Revital Saban, Joseph Cohen, Erez Halperin, Ofir Wolach, Najib Dally, Drorit Merkel, Howard S. Oster and Moshe Mittelmanadd
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Abstract
Quality of life is impaired in MDS, but the role of hemoglobin level is unclear. To study the Hb–QoL correlation at diagnosis and 1 year later, patients filled out the EQ-5D questionnaire, assessing their mobility, self care, daily activities, pain/discomfort, and anxiety/depression, using
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Quality of life is impaired in MDS, but the role of hemoglobin level is unclear. To study the Hb–QoL correlation at diagnosis and 1 year later, patients filled out the EQ-5D questionnaire, assessing their mobility, self care, daily activities, pain/discomfort, and anxiety/depression, using scores of 0 (normal), 1 (mild/moderate), or 2 (poor). They also evaluated their health using a visual analogue scale, scoring from 0 (poor) to 100 (excellent). The anemia subgroups were: none/normal (Hb ≥ 12.5 g/dL), mild (10 ≤ Hb < 12.5), moderate (9 ≤ Hb < 10), severe (8 ≤ Hb < 9), or very severe (Hb < 8). LR-MDS patients (n = 127) and inpatient controls (n = 141) participated. The anemic patients had a poor QoL and the MDS patients had a lower QoL with a lower Hb. The controls had no QoL difference among the various anemia subgroups. In addition, the MDS QoL sharply decreased with an Hb of < 9. The MDS patients showed a wide QoL variability, i.e., different QoL scores in the same Hb subgroup, suggesting that other factors affect QoL (e.g., age and comorbidities). After 1 year (n = 61), the QoL was still poor for most MDS patients (including 27 patients with an increased Hb). In summary: (1) a poor QoL in MDS-anemia is non-linear, suggesting other influencing factors on QoL. (2) The sharp QoL drop with Hb < 9 g/dL challenges the transfusion Hb threshold. (3) The QoL in anemic MDS patients might differ from that in non-MDS patients. (4) Raising Hb, while recommended, does not guarantee an improved QoL.
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