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Introduction: Severe pulmonary GvHD after allo-HCT is a significant cause of morbidity and mortality with limited therapeutic options. Selected patients can be lung transplanted, however there are no consensus guidelines on whom to select nor on when to refer. Method: Retrospective review of patients who underwent LT for lung GvHD after allo-HCT. Results: Ten patients were identified between January 2002 and December 2020. The median age at LT was 31 years. Seven patients developed aGvHD after allo-HCT and all patients developed cGvHD. The median immunosuppressive lines used before lung transplant was four. The median time between allo-HCT and bronchiolitis obliterans syndrome was 20.5 months. The median time from allo-HCT to LT was 71 months. The median time from first lung transplant evaluation to actual LT was 61 months. The median time from listing for LT to actual LT was 4.8 months. The median FEV1 upon referral to LT clinic was 31% and the median FEV1 upon LT was 25%. All patients received cadaveric bilateral lung transplants. After a median follow up of 4.5 years post-LT, the estimated 5-years OS after LT was 85.7%. The cumulative incidence of CLAD was 50% with a median time from LT to CLAD of 47 months. The CLAD-free survival at 5 years was 50%. Two patients died after developing CLAD complications. No hematologic relapse nor secondary malignancies reported. Conclusion: LT is an effective intervention for selected lung GvHD patients. Multidisciplinary management and consensus guidelines are needed to better serve these patients.

Background: primary graft failure is a devastating complication after allogeneic transplant. Donor specific antibodies has been reported as a major risk factor contributing to graft failure. We retrospectively analyzed our haploidentical transplant registry to report the incidence and impact of DSA and anti-HLA on engraftment in a cohort of malignant hematologic disease conditioned with myeloablative regimens. Methods: retrospective analysis of consecutive cases with malignant hematologic disorders who received a myeloablative haplo-HSCT at a single center. Results: 107 patients were identified with a median recipient age of 22 and a median donor age of 31. Sixty-two patients had AML (58%), 29 had ALL (27%), and 16 (15%) had other malignancies. Sixty-one recipients (57%) had positive anti-HLA, 56 of them had the DSA results available, of these 17 patients had DSAs (15% of the total number of patients, or 28% of patients who have anti-HLA antibodies). The median cumulative MFI was 2062 (IQR: 1038 - 6500). Sixty three percent of the DSA were against class II HLA antigens, while 37% were against class I HLA antigens. The OS, CIR, aGvHD, and cGvHD did not differ between patients with and without anti-HLA antibodies, nor between patients with and without DSA. The gender of the recipient and donor, as well as the gender mismatch between recipient and donor, were statistically associated with the incidence of anti-HLA antibodies, but not with DSA. Three patients only developed GF (2.8%), one was primary (0.9%) and the other two had secondary GF (1.9%). None of the GF cases was in patients with anti-HLA antibodies or DSA. Conclusion: In our cohort, the presence of anti-HLA or DSAs did not affect the outcomes including the incidence of PGF following myeloablative haplo-HSCT for malignant hematologic disorders.

This is a retrospective analysis of the clinical and laboratory features of 17 cases of factor XIII deficiency that were followed in tertiary care hospitals in Riyadh, Kingdom of Saudi Arabia, over 20 years. Cases were referred to these hospitals from other health care centers in the country.We performed a retrospective analysis of 17 cases of factor XIII deficiency comprising 11 males and 6 females, who were seen over a period of 20 years (1978-1998) in Riyadh, Kingdom of Saudi Arabia. Data variables including age, sex, origin, clinical presentation, bleeding time, prothrombin time, partial thromboplastin time, factor XIII screening and assay, hemoglobin, and platelet count were collected and analyzed. The diagnosis of factor XIII deficiency was made by urea clot lysis test alone in one patient and urea clot lysis test in combination with factor XIII quantitative assay in 16 patients.Eleven patients were males and 6 were females. Median age at the time of diagnosis was 9 years (3-29 years). Ten patients (59%) had a family history of excessive bleeding. Presenting symptoms included ecchymosis and recurrent hematomas in 12 patients (71%), bleeding after circumcision in 6 male patients (55%), umbilical stump bleeding in 7 (41%), poor wound healing and keloids in 3 patients (18%), and intracranial bleeding in 3 patients (18%). Other manifestations included cephalohematoma, abortion, abruptio placenta, and intraperitoneal bleeding (one patient each). Laboratory evaluation revealed a normal prothrombin time, partial thromboplastin time, bleeding time and platelet count in all patients. Factor XIII screening test was positive in all 17 patients tested and assay for factors XIII was0.06 U/ml in 16 patients tested.Our data confirms that factor XIII deficiency is a rare bleeding disorder characterized by variable bleeding manifestations but consistent laboratory findings. The occurrence of keloid in our patient group may reflect the poor quality of the clotting, associated with loss of tensile strength of fibrin polymers, caused by factor XIII deficiency and leading to abnormally large scar formation.

The poor prognosis for patients with diffuse large B cell lymphoma (DLBCL) who relapse within 1 year of initial diagnosis after first-line rituximab-based chemo-immunotherapy has created controversy about the role of autologous transplantation (HCT) in this setting. We compared autologous HCT outcomes for chemosensitive DLBCL patients between 2000 and 2011 in 2 cohorts based on time to relapse from diagnosis. The early rituximab failure (ERF) cohort consisted of patients with primary refractory disease or those with first relapse within 1 year of initial diagnosis. The ERF cohort was compared with those relapsing >1 year after initial diagnosis (late rituximab failure [LRF] cohort). ERF and LRF cohorts included 300 and 216 patients, respectively. Nonrelapse mortality (NRM), progression/relapse, progression-free survival (PFS), and overall survival (OS) of ERF versus LRF cohorts at 3 years were 9% (95% confidence interval [CI], 6% to 13%) versus 9% (95% CI, 5% to 13%), 47% (95% CI, 41% to 52%) versus 39% (95% CI, 33% to 46%), 44% (95% CI, 38% to 50%) versus 52% (95% CI, 45% to 59%), and 50% (95% CI, 44% to 56%) versus 67% (95% CI, 60% to 74%), respectively. On multivariate analysis, ERF was not associated with higher NRM (relative risk [RR], 1.31; P = .34). The ERF cohort had a higher risk of treatment failure (progression/relapse or death) (RR, 2.08; P