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Background and aims Immunoglobulin 4 (IgG4)-related disease (IgG4-RD) is a lymphoproliferative disorder characterized by elevated serum IgG4 levels and tissue infiltration of IgG4-positive plasma cells. We analyzed the serum proteins, whose levels varied based on the disease state and treatment. Materials and methods Serum proteins from patients with IgG4-related disease and healthy subjects were resolved using two-dimensional electrophoresis, silver-stained, and scanned. Alternatively, the proteins were labeled with Cy2, Cy3, and Cy5 before electrophoresis. The proteins, whose expression differed significantly between patients and healthy individuals, and between before and after steroid treatment, were identified and validated using enzyme-linked immunosorbent assays. Results Pre-treatment sera from patients with IgG4-related disease was characterized by increased levels of immunoglobulins such as IgG1, IgG4; inflammatory factors such as α-1 antitrypsin (A1AT); and proteins associated with immune system regulation such as clusterin and leucine-rich α-2-glycoprotein (LRG-1). The serum levels of A1AT, LRG-1 and clusterin, during treatment with prednisolone for up to 12 months revealed that LRG-1 levels were halved after 1 month of treatment, comparable to those in healthy subjects; LRG-1 levels remained normal until the end of treatment. Conclusion LRG-1 could serve as a novel biomarker of IgG4-related diseases.

We conducted a retrospective analysis to compare outcomes in adult patients with acute lymphoblastic leukemia (ALL) who underwent allogeneic hematopoietic cell transplantation (allo-HCT) with conditioning regimens containing cyclophosphamide (CY) in combination with total body irradiation (TBI), oral busulfan (p.o. BU), or intravenous busulfan (i.v. BU). We used data for January 2000 to December 2012 from the Transplant Registry Unified Management Program of the Japan Society of Hematopoietic Cell Transplantation. We identified 2130 patients treated with TBI/CY (n = 2028), p.o. BU/CY (n = 60), or i.v. BU/CY (n = 42). Two-year overall survival (OS) and 2-year relapse-free survival rates were 69.0% and 62.1%, respectively, in the TBI/CY group, 55.9% and 54.2% in the p.o. BU/CY group, and 71.0% and 46.8% in the i.v. BU/CY group. In multivariate analysis, compared with TBI/CY, p.o. BU/CY, but not i.v. BU/CY, was associated with lower OS (hazard ratio [HR], 1.46; P = .047) and a higher incidence of sinusoidal obstruction syndrome (HR, 3.36; P = .030). No between-group differences were seen in the incidence of nonrelapse mortality, relapse, acute graft-versus-host disease (GVHD), or chronic GVHD. We suggest that i.v. BU/CY might be a possible alternative allo-HCT conditioning regimen for adults with ALL who are not suitable for TBI.

Introduction The addition of tyrosine-kinase inhibitor (TKI) to conventional chemotherapy followed by allogenic hematopoietic stem cell transplantation (HSCT) is a standard treatment for transplant-eligible patients with Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL). Although TKI administration after HSCT may carry a survival benefit, available data are scarce. Objectives We conducted a nationwide retrospective study to evaluate whether TKI prophylaxis after allogenic HSCT in patients with negative-MRD would reduce relapse and improve overall survival. Methods In this nationwide retrospective cohort study, we included patients with Ph+ ALL who underwent their first allogenic HSCT between 2001 and 2016, received TKI before HSCT, and achieved negative-MRD status within 180 days after HSCT. Administration of TKI within 180 days after HSCT in a patient who maintained negative-MRD was defined as prophylactic TKI. Cox proportional hazards regression models were used to evaluate the impact of post-transplant TKI prophylaxis as a time-dependent covariate. Results We identified 850 patients for inclusion in this study. Fifty patients received TKI prophylaxis, mostly imatinib or dasatinib. TKI was more frequently administered in late time periods of HSCT or in cases of positive-MRD at HSCT. In a multivariate analysis, disease status at HSCT was the sole risk factor for relapse (hazard ratio, 3.58; P Conclusions Routine post-transplant TKI prophylaxis especially with imatinib is not recommended because this strategy yielded no clinical benefits. Since the risk of relapse in patients who did not achieve negative-MRD at HSCT was higher, even though they did achieve negative-MRD after HSCT, close monitoring and different post-transplant strategies using new-generation TKIs might be preferable in such patients.

Varicella, characterized by a vesicular rash, occurs primarily in young children. Although older individuals can also be affected or vaccinated, outbreaks among adults are rare. We investigated a small outbreak of varicella in B-cell lymphoma patients for elucidation of risk factor of the disease. We experienced four cases of varicella after an index herpes zoster case. All varicella cases were confirmed varicella zoster virus (VZV) infection by PCR. All varicella cases occurred in diffuse large B-cell lymphoma patients receiving rituximab-containing chemotherapy. On the other hand, only three of the 18 non-varicella patients in the same room were receiving rituximab-containing chemotherapy (P = 0.005). All varicella patients had detectable serum anti-varicella zoster virus IgG antibodies before chemotherapy. Even in the presence of neutralizing antibodies to the virus, lymphoma patients treated with rituximab-containing chemotherapy can possibly become re-infected with varicella. These findings suggest that zoster patients should be strictly isolated in hematology and oncology ward, and prophylactic acyclovir should be considered for such patients when exposed to zoster/varicella.