Patients experience physical, psychological, and psychosocial distress, as well as a deteriorating quality of life (QOL) due to complications at different stages of hematopoietic stem cell transplantation (HSCT). Pre-transplant status is often compromised by inactivity due to side effects of chemotherapy or the disease itself. Regimen-related toxicity (RRT), complications such as infections, graft versus host disease, and treatment processes during and after HSCT often result in patients being immobilized for long periods by bed rest that leads to fatigue and loss of muscle strength and endurance. Previous studies have shown that exercise might not only improve muscle strength and aerobic fitness, but also decrease cancer-related fatigue and enhance QOL. Many studies recommend flexibility training, as well as aerobic exercise and muscle strengthening using resistance. Aerobic exercise should be gradually increased to a total of at least 150 minutes spread over three to five days per week, resistance training should be implemented two to three days per week, and flexibility training should occur every day. Consistent evidence has shown that exercise is safe for patients undergoing HSCT.
Early-onset hemophagocytic lymphohistiocytosis (HLH) can arise as a rare, but important, complication of hematopoietic cell transplantation (HCT) and is difficult to diagnose and treat. It can be considered to be a type of post-transplant cytokine storm syndrome, similar to pre-engraftment immune reaction, engraftment syndrome, other forms of early-onset HLH, and idiopathic pneumonia syndrome. HLH is assumed to be caused by overwhelming activation of monocytes/macrophages. Combined treatment with low-dose etoposide and dexamethasone palmitate has been reported to an effective treatment for early-onset HLH after HCT, as it suppresses abnormally activated monocytes/macrophages. The elucidation of the immunological profile of this condition and the establishment of methods for achieving optimal control of engraftment-related post-transplant cytokine storm syndrome are expected to be achieved in future.
Sinusoidal obstruction syndrome (SOS), also called veno-occlusive disease (VOD) of the liver, is one of the most relevant complications of hepatic sinusoidal endothelial origin that appears early after haematopoietic cell transplantation (HCT). Despite its relatively low incidence and the fact that most cases of SOS/VOD resolve spontaneously, in the severe SOS/VOD that evolve to multi-organ failure have a mortality rate higher than 80% and represent one of the major clinical problems after HCT. In these patients, the sinusoidal endothelial cells and hepatocytes are damaged by toxic metabolites generated by the conditioning regimen. Several risk factors have been identified for the development of SOS/VOD. Despite defibrotide is recommended for both prevention and treatment, there is no satisfactory therapy for managing severe SOS/VOD. In the present review we report the new definition for late onset SOS/VOD diagnosis and severity grading by the European Society for Blood and Marrow Transplantation, results of nationwide survey in Japan and current treatment.
In a secondary immunodeficient state following hematopoietic cell transplantation, a variety of infectious episodes may take place. Infections may be difficult to treat in some instances. Furthermore, specific immunity against pathogens, either infection-induced or vaccine-induced, may eventually disappear after transplantation. Therefore, it is desirable to vaccinate post-transplant patients if it is suitable for them. Before vaccination for vaccine-preventable diseases, it is important to know when and how immune reconstitution may begin after transplantation. In clinical practice, specific antibody responses against vaccines may be evaluated before and after vaccination. However, “Correlates of Protection”, as indicators of immunological protection against pathogens, include humoral, cellular, and mucosal immunity. Thus, evaluating antibody alone may be limited to predict the clinical outcome. In this article, I have reviewed the current status of post-transplant immune recovery and vaccination.
Iron overload is a common complication of allogeneic hematopoietic cell transplantation (HCT); however, its management remains to be studied. We retrospectively analyzed the efficacy and safety of low-dose deferasirox treatment in four HCT survivors with iron overload and measured serum ferritin levels, liver iron concentrations (LIC), and non-transferrin-bound iron (NTBI) levels. Patients who had become transfusion-independent after HCT were treated using 10 mg/kg/day deferasirox. Their median age was 36.5 years (range, 27-39), and they had survived a median of 66 months (range, 27-101) after HCT. After a median of 23.5 months (range, 16-34) of deferasirox treatment, serum ferritin levels and LIC decreased in all patients, and NTBI decreased in three patients. The median ferritin levels, LIC, and NTBI levels decreased from 6135 (range, 3720-10,500) to 1782 ng/mL (range, 775-6840), 24.6 (range, 9.6-43.0) to 7.8 mg/g (range, 2.8-42.3), and 1.26 (range, 0.89-2.09) to 0.82 μmol/L (range, 0.64-1.54), respectively. Abnormal liver function tests improved in all patients after deferasirox treatment. On the other hand, all patients experienced an increase in serum creatinine levels. In conclusion, treatment with low-dose deferasirox might be an effective alternative for allogeneic HCT survivors with iron overload.
18F-fluorodeoxyglucose-positron emission tomography (PET) is a useful asset for evaluating the disease stage, residual disease, and activity of malignant lymphoma, including adult T-cell leukemia/lymphoma (ATLL), and its use to treat infectious diseases has also been reported. After a 51-year-old female with ATLL received chemotherapy, all of her lesions became undetectable by computed tomography (CT). However, intrathoracic lymph nodes were found to be PET-avid without significant swelling, and were considered recurrent ATLL lesions. She subsequently underwent cord blood transplantation (CBT). Two months later, she presented with high-grade fever. CT revealed significant left hilar and mediastinal lymph node swelling with central low density, suggesting necrosis. After Mycobacterium tuberculosis (M. tuberculosis) was repeatedly detected in her blood, urine, feces, and gastric juice, disseminated tuberculosis was diagnosed. Physicians should always be aware of infectious diseases, particularly tuberculosis, as a possible cause of PET-avid nodal lesions in immunocompromised patients.