Spectrum of ASXL1 mutations in primary myelofibrosis: prognostic impact of the ASXL1 p.G646Wfs*12 mutation
(Source: Blood)
Source: Blood - June 26, 2019 Category: Hematology Authors: Rotunno, G., Mannarelli, C., Brogi, G., Pacilli, A., Gesullo, F., Mannelli, F., Fiaccabrino, S., Sordi, B., Paoli, C., Marone, I., Rumi, E., Manfredini, R., Barosi, G., Cazzola, M., Vannucchi, A. M., Guglielmelli, P. Tags: Myeloid Neoplasia Letter to Blood Source Type: research

Safety of CAR T-cell therapy in patients with B-cell lymphoma and chronic hepatitis B or C virus infection
(Source: Blood)
Source: Blood - June 26, 2019 Category: Hematology Authors: Strati, P., Nastoupil, L. J., Fayad, L. E., Samaniego, F., Adkins, S., Neelapu, S. S. Tags: Immunobiology and Immunotherapy, Lymphoid Neoplasia, Clinical Trials and Observations Letter to Blood Source Type: research

Total body irradiation dose and risk of subsequent neoplasms following allogeneic hematopoietic cell transplantation
We examined the impact of total body irradiation (TBI) dose and fractionation on risk of subsequent malignant neoplasms (SMNs) in the era of reduced-intensity and nonmyeloablative conditioning regimens for hematopoietic cell transplantation (HCT). Among 4905 1-year survivors of allogeneic HCT for hematologic malignancies (N = 4500) or nonmalignant disorders (N = 405) who received transplants between 1969 and 2014, we identified 581 SMNs (excluding squamous and basal cell of skin) in 499 individuals. With a median length of follow-up of 12.5 years, the cumulative incidence of SMNs by 30 years after HCT was 22.0%. Compared w...
Source: Blood - June 26, 2019 Category: Hematology Authors: Baker, K. S., Leisenring, W. M., Goodman, P. J., Ermoian, R. P., Flowers, M. E., Schoch, G., Storb, R., Sandmaier, B. M., Deeg, H. J. Tags: Transplantation, Clinical Trials and Observations Source Type: research

Recurrent MSCE116K mutations in ALK-negative anaplastic large cell lymphoma
Anaplastic large cell lymphomas (ALCLs) represent a relatively common group of T-cell non-Hodgkin lymphomas (T-NHLs) that are unified by similar pathologic features but demonstrate marked genetic heterogeneity. ALCLs are broadly classified as being anaplastic lymphoma kinase (ALK)+ or ALK–, based on the presence or absence of ALK rearrangements. Exome sequencing of 62 T-NHLs identified a previously unreported recurrent mutation in the musculin gene, MSCE116K, exclusively in ALK– ALCLs. Additional sequencing for a total of 238 T-NHLs confirmed the specificity of MSCE116K for ALK– ALCL and further demonstra...
Source: Blood - June 26, 2019 Category: Hematology Authors: Luchtel, R. A., Zimmermann, M. T., Hu, G., Dasari, S., Jiang, M., Oishi, N., Jacobs, H. K., Zeng, Y., Hundal, T., Rech, K. L., Ketterling, R. P., Lee, J.-H., Eckloff, B. W., Yan, H., Gaonkar, K. S., Tian, S., Ye, Z., Kadin, M. E., Sidhu, J., Jiang, L., Vo Tags: Lymphoid Neoplasia Source Type: research

Phase 1b study of venetoclax-obinutuzumab in previously untreated and relapsed/refractory chronic lymphocytic leukemia
This single-arm, open-label, phase 1b study evaluated the maximum tolerated dose (MTD) of venetoclax when given with obinutuzumab and its safety and tolerability in patients with relapsed/refractory (R/R) or previously untreated (first line [1L]) chronic lymphocytic leukemia (CLL). Venetoclax dose initially was escalated (100-400 mg) in a 3 + 3 design to define MTD combined with standard-dose obinutuzumab. Patients received venetoclax (schedule A) or obinutuzumab (schedule B) first to compare safety and determine dose/schedule for expansion. Venetoclax-obinutuzumab was administered for 6 cycles, followed by venetoclax mono...
Source: Blood - June 26, 2019 Category: Hematology Authors: Flinn, I. W., Gribben, J. G., Dyer, M. J. S., Wierda, W., Maris, M. B., Furman, R. R., Hillmen, P., Rogers, K. A., Iyer, S. P., Quillet-Mary, A., Ysebaert, L., Walter, H. S., Verdugo, M., Klein, C., Huang, H., Jiang, Y., Lozanski, G., Pignataro, D. S., Hu Tags: Lymphoid Neoplasia, Clinical Trials and Observations Source Type: research

Hydroa vacciniforme-like lymphoproliferative disorder: an EBV disease with a low risk of systemic illness in whites
We report our 11-year experience with 16 HVLPD patients from the United States and England and found that whites were less likely to develop systemic EBV disease (1/10) than nonwhites (5/6). All (10/10) of the white patients were generally in good health at last follow-up, while two-thirds (4/6) of the nonwhite patients required hematopoietic stem cell transplantation. Nonwhite patients had later age of onset of HVLPD than white patients (median age, 8 vs 5 years) and higher levels of EBV DNA (median, 1 515 000 vs 250 000 copies/ml) and more often had low numbers of NK cells (83% vs 50% of patients) and T-cell clones in th...
Source: Blood - June 26, 2019 Category: Hematology Authors: Cohen, J. I., Manoli, I., Dowdell, K., Krogmann, T. A., Tamura, D., Radecki, P., Bu, W., Turk, S.-P., Liepshutz, K., Hornung, R. L., Fassihi, H., Sarkany, R. P., Bonnycastle, L. L., Chines, P. S., Swift, A. J., Myers, T. G., Levoska, M. A., DiGiovanna, J. Tags: Lymphoid Neoplasia, Clinical Trials and Observations Source Type: research

CRISPR/Cas9-mediated in vivo gene targeting corrects hemostasis in newborn and adult factor IX-knockout mice
Many genetic diseases, including hemophilia, require long-term therapeutic effects. Despite the initial success of liver-directed adeno-associated virus (AAV) gene therapy for hemophilia in clinical trials, long-term sustained therapeutic effects have yet to be seen. One explanation for the gradual decline of efficacy over time is that the nonintegrating AAV vector genome could be lost during cell division during hepatocyte turnover, albeit at a slow pace in adults. Readministering the same vector is challenging as a result of the AAV-neutralizing antibodies elicited by the initial treatment. Here, we investigated the use ...
Source: Blood - June 26, 2019 Category: Hematology Authors: Wang, L., Yang, Y., Breton, C. A., White, J., Zhang, J., Che, Y., Saveliev, A., McMenamin, D., He, Z., Latshaw, C., Li, M., Wilson, J. M. Tags: Thrombosis and Hemostasis, Gene Therapy Source Type: research

Proteomic evidence of specific IGKV1-8 association with cystic lung light chain deposition disease
In conclusion, the high frequency of IGKV1-8 usage in cystic pLCDD constitutes an additional feature arguing for a specific entity distinct from the systemic form that preferentially uses IGKV4-1. (Source: Blood)
Source: Blood - June 26, 2019 Category: Hematology Authors: Camus, M., Hirschi, S., Prevot, G., Chenard, M.-P., Mal, H., Stern, M., Reynaud-Gaubert, M., Gilhodes, J., Burlet-Schiltz, O., Brousset, P., Colombat, M. Tags: Brief Reports, Clinical Trials and Observations Source Type: research

Nuke less for less cancer
(Source: Blood)
Source: Blood - June 26, 2019 Category: Hematology Authors: Mohty, M. Tags: Free Research Articles COMMENTS Source Type: research

A one-two punch with VO KOs CLL
(Source: Blood)
Source: Blood - June 26, 2019 Category: Hematology Authors: Coutre, S. Tags: Free Research Articles COMMENTS Source Type: research

Deciphering hydroa vacciniforme
(Source: Blood)
Source: Blood - June 26, 2019 Category: Hematology Authors: Quintanilla-Martinez, L., Fend, F. Tags: Free Research Articles COMMENTS Source Type: research

Gene editing in hemophilia: a "CRISPR" choice?
(Source: Blood)
Source: Blood - June 26, 2019 Category: Hematology Authors: Pipe, S. W., Selvaraj, S. R. Tags: Free Research Articles COMMENTS Source Type: research

Role of urine immunofixation in defining CR in MM
(Source: Blood)
Source: Blood - June 19, 2019 Category: Hematology Tags: Free Research Articles, CME article CONTINUING MEDICAL EDUCATION (CME) QUESTIONS Source Type: research

Ring-shaped granulomas on bone marrow found in a returning traveler with fever of unknown origin
(Source: Blood)
Source: Blood - June 19, 2019 Category: Hematology Authors: Ng, T. F., Chuah, H. S. Tags: Free Research Articles, BloodWork, Phagocytes, Granulocytes, and Myelopoiesis BLOOD WORK Source Type: research

Mantle cell lymphoma with unusual Burkitt-like morphologic features
(Source: Blood)
Source: Blood - June 19, 2019 Category: Hematology Authors: Chan, A. C., Vali Betts, E. Tags: Free Research Articles, BloodWork, Lymphoid Neoplasia BLOOD WORK Source Type: research