Both hepatic sinusoidal occlusion syndrome (SOS) and transplant-associated thrombotic microangiopathy (TA-TMA) are acquired thrombotic disorders in which sinusoidal endothelial cell damage and vascular endothelial cell damage are thought to be involved in their pathogenesis. In Japan, defibrotide (DF), a mixture of oligonucleotides made from porcine small intestinal mucosal DNA, has been available for use against SOS since 2019. although the mechanism of action of DF has not yet been fully elucidated, it is thought to exert its effects on SOS via endothelial protection, anticoagulation, and fibrinolysis-promoting effects. No effective treatment for TA-TMA has been established yet. However, recent studies have shown that complement activation may be involved in the pathogenesis of the disease, and it is attracting attention as a diagnostic marker and therapeutic target molecule.

 The major limitations of cord blood transplantation (CBT) for adults have been higher rates of engraftment failure and early mortality. The amelioration of conditioning regimens could contribute to the recent improvement of early mortality in CBT. Although total body irradiation (TBI)-based regimens have remained the preferred regimens for CBT in younger patients, engraftment and survival after myeloablative intravenous busulfan-based regimens have also been improved by the addition of low-dose TBI, and/or other chemotherapeutic drugs, particularly for elderly patients. Intensified conditioning regimen could also improve engraftment and survival after CBT. To clarify whether conditioning treatment based on granulocyte-colony stimulating factor (G-CSF) priming effect improves results of CBT, a multicenter randomized phase III study is under way in Japan.

 Extracorporeal photopheresis (ECP) was approved in Japan for steroid-refractory, or-intolerant chronic graft-versus-host disease (cGVHD) in December 2020. The mechanism of action of ECP is still not completely understood, however, it has been hypothesized that the immunomodulation induced by ECP, including induction of lymphocyte apoptosis, shift in antigen-presenting cell populations, shift in cytokine secretion, induction of regulatory T cells, increase in myeloid-derived suppressor cells, and alteration of B-cell signaling and populations are responsible for the therapeutic effect of ECP on cGVHD. There is a wealth of clinical evidence for ECP from clinical studies conducted mainly in Europe and America, and this evidence generally supports the efficacy and safety of ECP in the treatment of cGVHD. The improved response rate by ECP therapy has been observed in various organs and, in particular, higher response rates have been reported for skin, oral mucosa and liver. The reduction in the dose of concomitantly administered steroids and prolongation of overall survival have also been observed in cGVHD patients treated with ECP. ECP therapy has been generally reported to be tolerable with an acceptable safety profile. It is important to accumulate additional clinical evidence for ECP therapy in Japanese cGVHD patients in real-world clinical practice.

 We conducted a single-center, prospective, observational study to evaluate longitudinal changes of physical function in 100 patients who underwent allogeneic hematopoietic cell transplantation (HCT) at our center between November 2016 and March 2019. We measured grip strength, knee extension strength, the Short Physical Performance Battery (SPPB), and muscle volume from before HCT until 56 days after HCT. Grip strength decreased with time after HCT. Knee extension strength and muscle volume increased until 14 days after HCT and showed a decrease at 42 days after HCT. SPPB showed minimal temporal changes after HCT. Multiple regression analysis indicated that the following factors were associated with decreased parameters at 42 days after HCT compared with baseline values before HCT: HLA mismatching (for grip strength), reduced-intensity conditioning (for SPPB), unrelated donor (for muscle volume), body weight decrease≥5% (for muscle volume), and mean cumulative prednisolone-equivalent steroid dose≥20 mg per day (for grip strength and SPPB). These results may contribute to the formulation of nursing plans to prevent decreased physical function in patients who undergo allogeneic HCT.

 We conducted a nationwide cross-sectional questionnaire study to assess the prevalence of organ symptoms in allogeneic hematopoietic cell transplantation (allo-HCT) survivors and its impact on work and daily life. We targeted allo-HCT survivors employed at diagnosis, aged 20-64 at survey, and who survived≥2 years without relapse. The questionnaire included the presence or absence of 34 organ symptoms and their impact on work and daily life, as well as patient-, HCT-, and work-related factors. A total of 841 participants who were working at the time of the survey were included in the analysis (response rate, 60%). Ninety-two percent of participants had at least one symptom; the skin (78%), joints/muscles (72%), eyes (61%), and psychological health (44%) were frequently affected. Multivariable logistic regression analysis of the impact of symptoms on work showed that different job types were independently associated with different symptoms: indoor/physical work (medical care staff, etc.) with itching, dry eyes, restricted joint mobility; outdoor/light duty (sales staff, etc.) with thinning of hair and depressive state; and outdoor/physical work (construction, etc.) with dazzling. Our findings indicated that it may be effective to provide individual intervention and education that take into consideration both survivors’ symptoms and their work/life conditions.

 Atypical chronic myeloid leukemia (aCML) is rare, with a poor prognosis. A 30-year-old man with aCML was referred to our hospital for allogeneic hematopoietic stem cell transplantation. Even after administering hydroxyurea, the neutrophil counts and level of lactate dehydrogenase (LDH) in the peripheral blood was high. On referral, a bone marrow aspirate smear revealed hypercellularity with granulocytic proliferation and granulocytic dysplasia. After administration of azacitidine, the neutrophil counts and LDH level in peripheral blood decreased. The patient underwent peripheral blood stem cell transplantation from a human leukocyte antigen (HLA) haplo-matched related donor, on day 24 of azacitidine administration. He achieved neutrophil engraftment on day 17 following transplantation. A chimerism test of bone marrow showed mixed chimera on day 21 and 49 following transplantation; however, complete donor type was observed on day 81, 179 and 326 following transplantation. In cases of aCML diagnosis, prompt preparation for transplantation is advised. Further studies in a larger sample of patients with aCML are necessary to determine the suitable donor source and the efficacy of azacitidine as a bridging therapy for transplantation.