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We present the case of a 53-year-old woman with prefibrotic stage primary myelofibrosis (PMF) who underwent cord blood transplantation. Nine years after transplantation, she relapsed, which was confirmed by a bone marrow examination. We decided to treat her using azacitidine. After three courses of azacitidine, a partial cytogenetic response was confirmed. Azacitidine maintenance therapy successfully maintained a low level of recipient-origin peripheral blood cells with a stable hematological condition. Azacitidine may therefore be a promising therapeutic option for PMF patients who relapse after allogeneic stem cell transplantation.
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http://dx.doi.org/10.2169/internalmedicine.4863-20 | DOI Listing |
Transplant Cell Ther
September 2025
Department of Hematology, Kanagawa Cancer Center, Yokohama, Japan; Department of Hematology and Clinical Immunology, Yokohama City University School of Medicine, Yokohama, Japan.
The Dynamic International Prognostic Scoring System for primary myelofibrosis (DIPSS) has been reported to predict transplant outcomes in myelofibrosis (MF) patients. Recently, the pre-transplant use of JAK inhibitors has become common in clinical practice, but it is unclear whether DIPSS is also useful for predicting transplant outcomes for these patients. In this study, we compared the prognostic impact of DIPSS between MF patients with and without pre-transplant Ruxolitinib therapy.
View Article and Find Full Text PDFJ Hematol
August 2025
Department of Oncology, Edwards Comprehensive Cancer Institute, Marshall University, Huntington, WV 25701, USA.
Background: Myelofibrosis (MF) can be primary (PMF) or secondary (SMF), with PMF driven by Janus kinases-signal transducer and activator of transcription proteins (JAK-STAT) pathway activation due to Janus kinase 2 (), the thrombopoietin receptor gene (myeloproliferative leukemia virus oncogene ()), or calreticulin () mutations. Nearly 50% of PMF patients experience anemia (hemoglobin (Hb) < 10 g/dL), often worsened by JAK inhibitors like ruxolitinib and fedratinib. Momelotinib, an oral ACVR1, JAK1, and JAK2 inhibitor, improves anemia, symptoms, and splenomegaly, likely through hepcidin regulation.
View Article and Find Full Text PDFAm J Hematol
September 2025
CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, University of Florence, AOU Careggi, Florence, Italy.
Survival prediction models in essential thrombocythemia (ET) include the International Prognostic Scoring System (IPSET) and the more recently introduced triple-A (AAA) prognostic score. The latter enlists age and absolute neutrophil (ANC) and lymphocyte (ALC) counts as risk variables. In the current study, a Mayo Clinic discovery cohort of 658 patients with ET was used to identify AAA-independent risk variables.
View Article and Find Full Text PDFFuture Oncol
August 2025
Development Devision, Novartis Pharma K.K., Tokyo, Japan.
Aim: To evaluate the safety and effectiveness of ruxolitinib in patients with myelofibrosis (MF) in Japan.
Methods: A multicenter, observational study of patients who received ruxolitinib for MF from July 2014.
Results: Of 892 patients (mean age: 70 years, 45.
Tomography
July 2025
Department of Radiology, University of Michigan Medical School, Ann Arbor, MI 48109, USA.
Background/objectives: Myelofibrosis (MF) is a myeloproliferative neoplasm characterized by the replacement of healthy bone marrow (BM) with malignant and fibrotic tissue. In a healthy state, bone marrow is composed of approximately 60-70% fat cells, which are replaced as disease progresses. Proton density fat fraction (PDFF), a non-invasive and quantitative MRI metric, enables analysis of BM architecture by measuring the percentage of fat versus cells in the environment.
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