الفهرس 
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Abstract
Myelofibrosis (MF) is a myeloproliferative neoplasm that presents as primary (PMF) or secondary to polycythemia vera (PV) or essential thrombocythemia (ET), referred to as secondary myelofibrosis (SMF).
MF is a clonal stem-cell disorder that leads to bone marrow fibrosis, splenomegaly, anemia, constitutional symptoms, cachexia, and extramedullary hematopoiesis. The clinical course of MF is characterized by a leukemic progression and shortened overall survival.
Consequently, the number of allogeneic HSCTs performed for MF increased over the past years, despite the increasing use of JAK inhibitors, However, HSCT is associated with high treatment-related morbidity and mortality, particularly in older adults, where the risk of the procedure may outweigh the risk of disease. In addition, HSCT requires both good performance status and a suitable stem-cell donor.
Despite the availability of drugs that inhibit the JAK-STAT pathway, allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the only potentially curative option in this disease.
In this study, we aimed to discuss the clinical outcome of patients with primary myelofibrosis who underwent allo-HSCT conditioned with fludarabine, reduced-intensity busulfan, and antithymocyte globulin (FLU/BU/ATG) compared to patients received treatment by JAK2 inhibitors and other lines of treatment and their follow up for about 2 years.
This was a retrospective compartive Study performed at Ain Shams University Hospital (El-Demerdash) and Nasser’s Institute Hospital for six months to two years to collect data and follow up patients for about 2 years after transplant. Lab investigations and imaging were done.
This study conducted on 46 patients; 23 patients underwent Allo-SCT, and 23 patients received ruxolitinib, hydroxyurea (with or without supportive therapies including hematopoietic stimulating factors (HSFs, including erythropoietin and/or eltrombopag) or aspirin), or supportive therapies alone (collectively: “Non-Allo-SCT”).
Summary of results:
• Baseline Hgb ≤ 10 g/dl showed a trend towards an increased risk of mortality.
• Baseline PLT count ≤ 100×103/µl’ showed a significantly increased risk of mortality.
• Baseline TLC ≤ 25×103/µl did not show any significant association with the risk of mortality.
• JAKsV617F positive status, grade of BM fibrosis, and the DIPSS category were not associated with the risk of mortality.
• Patients who underwent Allo-SCT showed a trend toward higher mortality.
• Initial treatment or switching to ruxolitinib in non-Allo-SCT patients showed a trend towards lower mortality.
• For the remission phase, patients who underwent Allo-SCT had the shortest time in the “illness” state before achieving remission, death, or censorship, with an average time of 28 days, followed by ruxolitinib, with an average illness duration of 157 days.
• Patients who were treated with ruxolitinib had the longest time in the symptomatic free phase, with an average time of 573 days, followed by 495 days for patients who underwent Allo-SCT but the patient achieved disease remission.
• Patients who received hydroxyurea or HSFs only had the shortest Rmean durations of 132 and 115 days.
• Allo-SCT was found to be significantly associated with higher hemoglobin levels by more than 2 g/dl on averageand decrease of the splenic size throughout the study duration.