JSH practical guidelines for hematological malignancies, 2023: leukemia-2-acute promyelocytic leukemia (APL)
Int J Hematol. 2024 Apr 17. doi: 10.1007/s12185-024-03749-5. Online ahead of print.NO ABSTRACTPMID:38630356 | DOI:10.1007/s12185-024-03749-5 (Source: International Journal of Hematology)
Source: International Journal of Hematology - April 17, 2024 Category: Hematology Authors: Hiroyuki Fujita Source Type: research

Bernard-Soulier syndrome caused by a novel GP1BB variant and 22q11.2 deletion
Int J Hematol. 2024 Apr 16. doi: 10.1007/s12185-024-03768-2. Online ahead of print.ABSTRACTBernard-Soulier syndrome (BSS) is caused by defects in GP1BA, GP1BB, or GP9 genes. Patients with 22q11.2 deletion syndrome (22q11.2DS) are obligate carriers of BSS because GP1BB resides on chromosome 22q11.2. A 15-month-old girl without bleeding symptoms had giant platelets and thrombocytopenia. Physical findings and macrothrombocytopenia suggested 22q11.2DS, which was confirmed by fluorescence in situ hybridization. Flow cytometry showed decreased GPIbα on the platelets. Gene panel testing revealed a novel variant in GP1BB, p.(Val1...
Source: International Journal of Hematology - April 16, 2024 Category: Hematology Authors: Rintaro Nagoshi Atsushi Sakamoto Tsuyoshi Imai Toru Uchiyama Tadashi Kaname Shinji Kunishima Akira Ishiguro Source Type: research

Bernard-Soulier syndrome caused by a novel GP1BB variant and 22q11.2 deletion
Int J Hematol. 2024 Apr 16. doi: 10.1007/s12185-024-03768-2. Online ahead of print.ABSTRACTBernard-Soulier syndrome (BSS) is caused by defects in GP1BA, GP1BB, or GP9 genes. Patients with 22q11.2 deletion syndrome (22q11.2DS) are obligate carriers of BSS because GP1BB resides on chromosome 22q11.2. A 15-month-old girl without bleeding symptoms had giant platelets and thrombocytopenia. Physical findings and macrothrombocytopenia suggested 22q11.2DS, which was confirmed by fluorescence in situ hybridization. Flow cytometry showed decreased GPIbα on the platelets. Gene panel testing revealed a novel variant in GP1BB, p.(Val1...
Source: International Journal of Hematology - April 16, 2024 Category: Hematology Authors: Rintaro Nagoshi Atsushi Sakamoto Tsuyoshi Imai Toru Uchiyama Tadashi Kaname Shinji Kunishima Akira Ishiguro Source Type: research

Spontaneous regression of multiple solitary plasmacytoma harboring Epstein-Barr virus: a case report and literature review
We report a rare case of spontaneous regression (SR) in an elderly untreated patient with multiple solitary plasmacytoma (MSP). Diagnosis of MSP was confirmed through surgical resection of the left nasal cavity mass and subsequent biopsy of the right humerus. The patient was considered ineligible for chemotherapy due to poor performance status. At 3-month post-diagnosis, the patient's condition worsened with deteriorating bone lesions and emergence of a new serum monoclonal protein. However, these clinical findings completely disappeared at 6 months, and positron emission tomography-computed tomography at 1 year confirmed ...
Source: International Journal of Hematology - April 15, 2024 Category: Hematology Authors: Wataru Kitamura Hiroki Kobayashi Minori Noda Akiko Iseki Yumi Sato Yoshinobu Maeda Shoichi Kuyama Source Type: research

Honeycomb lung appearance accompanied by pediatric Langerhans cell histiocytosis: changes in imaging findings following chemotherapy
Int J Hematol. 2024 Apr 15. doi: 10.1007/s12185-024-03776-2. Online ahead of print.NO ABSTRACTPMID:38622431 | DOI:10.1007/s12185-024-03776-2 (Source: International Journal of Hematology)
Source: International Journal of Hematology - April 15, 2024 Category: Hematology Authors: Kenichi Sakamoto Osamu Miyazaki Yoko Shioda Source Type: research

Bispecific antibodies for multiple myeloma: past, present and future
Int J Hematol. 2024 Apr 13. doi: 10.1007/s12185-024-03766-4. Online ahead of print.ABSTRACTDespite the development of various therapeutic agents, multiple myeloma remains incurable. Recently, T-cell redirected immunotherapy has become a promising strategy for the treatment of refractory myeloma. Clinical trials using chimeric antigen receptor (CAR)-T cells and bispecific antibodies have demonstrated successful anti-myeloma responses in triple-class-refractory patients. However, unique and unwanted immune effects associated with on-target/off-target reactivity of activated immune cells need to be considered and properly man...
Source: International Journal of Hematology - April 13, 2024 Category: Hematology Authors: Toshiki Ochi Tatsuya Konishi Katsuto Takenaka Source Type: research

Bispecific antibodies for multiple myeloma: past, present and future
Int J Hematol. 2024 Apr 13. doi: 10.1007/s12185-024-03766-4. Online ahead of print.ABSTRACTDespite the development of various therapeutic agents, multiple myeloma remains incurable. Recently, T-cell redirected immunotherapy has become a promising strategy for the treatment of refractory myeloma. Clinical trials using chimeric antigen receptor (CAR)-T cells and bispecific antibodies have demonstrated successful anti-myeloma responses in triple-class-refractory patients. However, unique and unwanted immune effects associated with on-target/off-target reactivity of activated immune cells need to be considered and properly man...
Source: International Journal of Hematology - April 13, 2024 Category: Hematology Authors: Toshiki Ochi Tatsuya Konishi Katsuto Takenaka Source Type: research

A phase 2, open-label study of ibrutinib plus rituximab in Japanese patients with Waldenstrom's macroglobulinemia
This study evaluated the efficacy and safety of ibrutinib-rituximab in Japanese patients with WM. Patients received ibrutinib 420 mg orally once daily plus weekly rituximab 375 mg/m2 IV (8 infusions total). The primary end point was major response rate (MRR; PR or better) by Independent Review Committee assessment. Secondary endpoints were progression-free survival (PFS), safety, pharmacokinetics, and biomarkers. Primary analysis was conducted in 16 patients [baseline, treatment naïve: 8 (50.0%); relapsed/refractory WM: 8 (50.0%)] who received ibrutinib-rituximab, after all patients completed Week 57 or end of treatment. ...
Source: International Journal of Hematology - April 10, 2024 Category: Hematology Authors: Koji Izutsu Hisashi Kato Naohiro Sekiguchi Tomoaki Fujisaki Toshiro Kawakita Naoshi Obara Kosei Matsue Mitsutaka Nishimoto Tomoyoshi Hatayama Mitsuo Inagaki Ei Fujikawa Source Type: research

A phase 2, open-label study of ibrutinib plus rituximab in Japanese patients with Waldenstrom's macroglobulinemia
This study evaluated the efficacy and safety of ibrutinib-rituximab in Japanese patients with WM. Patients received ibrutinib 420 mg orally once daily plus weekly rituximab 375 mg/m2 IV (8 infusions total). The primary end point was major response rate (MRR; PR or better) by Independent Review Committee assessment. Secondary endpoints were progression-free survival (PFS), safety, pharmacokinetics, and biomarkers. Primary analysis was conducted in 16 patients [baseline, treatment naïve: 8 (50.0%); relapsed/refractory WM: 8 (50.0%)] who received ibrutinib-rituximab, after all patients completed Week 57 or end of treatment. ...
Source: International Journal of Hematology - April 10, 2024 Category: Hematology Authors: Koji Izutsu Hisashi Kato Naohiro Sekiguchi Tomoaki Fujisaki Toshiro Kawakita Naoshi Obara Kosei Matsue Mitsutaka Nishimoto Tomoyoshi Hatayama Mitsuo Inagaki Ei Fujikawa Source Type: research

A phase 2, open-label study of ibrutinib plus rituximab in Japanese patients with Waldenstrom's macroglobulinemia
This study evaluated the efficacy and safety of ibrutinib-rituximab in Japanese patients with WM. Patients received ibrutinib 420 mg orally once daily plus weekly rituximab 375 mg/m2 IV (8 infusions total). The primary end point was major response rate (MRR; PR or better) by Independent Review Committee assessment. Secondary endpoints were progression-free survival (PFS), safety, pharmacokinetics, and biomarkers. Primary analysis was conducted in 16 patients [baseline, treatment naïve: 8 (50.0%); relapsed/refractory WM: 8 (50.0%)] who received ibrutinib-rituximab, after all patients completed Week 57 or end of treatment. ...
Source: International Journal of Hematology - April 10, 2024 Category: Hematology Authors: Koji Izutsu Hisashi Kato Naohiro Sekiguchi Tomoaki Fujisaki Toshiro Kawakita Naoshi Obara Kosei Matsue Mitsutaka Nishimoto Tomoyoshi Hatayama Mitsuo Inagaki Ei Fujikawa Source Type: research

Changes in chronic myeloid leukemia treatment modalities and outcomes after introduction of second-generation tyrosine kinase inhibitors as first-line therapy: a multi-institutional retrospective study by the CML Cooperative Study Group
This study investigated changes in treatment modalities and outcomes of chronic myeloid leukemia in the chronic phase (CP-CML) after the approval of second-generation tyrosine kinase inhibitors (2G-TKIs) for first-line therapy. Patients were grouped into those who underwent TKI therapy up to December 2010 (imatinib era group, n = 185) and after January 2011 (2G-TKI era group, n = 425). All patients in the imatinib era group were initially treated with imatinib, whereas patients in the 2G-TKI era group were mostly treated with dasatinib (55%) or nilotinib (36%). However, outcomes including progression-free survival, overall...
Source: International Journal of Hematology - April 8, 2024 Category: Hematology Authors: Noriyoshi Iriyama Eisaku Iwanaga Yuta Kimura Naoki Watanabe Maho Ishikawa Hitomi Nakayama Eriko Sato Takayuki Tabayashi Toru Mitsumori Tomoiku Takaku Tomonori Nakazato Michihide Tokuhira Hiroyuki Fujita Miki Ando Yoshihiro Hatta Tatsuya Kawaguchi Source Type: research

Optimized BEAC conditioning regimen improves clinical outcomes of autologous hematopoietic stem cell transplantation in non-Hodgkin lymphomas
Int J Hematol. 2024 Apr 8. doi: 10.1007/s12185-024-03755-7. Online ahead of print.ABSTRACTThe conditioning regimen is an important part of autologous hematopoietic stem cell transplantation (ASCT). We explored the efficacy and safety of an optimized BEAC (adjusted-dose, intermediate-dose cytarabine and reduced-dose cyclophosphamide, AD-BEAC) conditioning regimen for non-Hodgkin lymphoma (NHL). A total of 141 NHL patients received AD-BEAC or a standard-dose BEAC (SD-BEAC) conditioning regimen from January 2007 to December 2017, and 104 patients were included in the study after 1:1 propensity matching. The 5-year overall sur...
Source: International Journal of Hematology - April 8, 2024 Category: Hematology Authors: Sha Zhou Jun Rao Xiangyu Ma Yunjing Zeng Xixi Xiang Jiali Li Hongyun Liu Shijia Lin Song Dong Fu Li Xi Zhang Li Gao Source Type: research

Changes in chronic myeloid leukemia treatment modalities and outcomes after introduction of second-generation tyrosine kinase inhibitors as first-line therapy: a multi-institutional retrospective study by the CML Cooperative Study Group
This study investigated changes in treatment modalities and outcomes of chronic myeloid leukemia in the chronic phase (CP-CML) after the approval of second-generation tyrosine kinase inhibitors (2G-TKIs) for first-line therapy. Patients were grouped into those who underwent TKI therapy up to December 2010 (imatinib era group, n = 185) and after January 2011 (2G-TKI era group, n = 425). All patients in the imatinib era group were initially treated with imatinib, whereas patients in the 2G-TKI era group were mostly treated with dasatinib (55%) or nilotinib (36%). However, outcomes including progression-free survival, overall...
Source: International Journal of Hematology - April 8, 2024 Category: Hematology Authors: Noriyoshi Iriyama Eisaku Iwanaga Yuta Kimura Naoki Watanabe Maho Ishikawa Hitomi Nakayama Eriko Sato Takayuki Tabayashi Toru Mitsumori Tomoiku Takaku Tomonori Nakazato Michihide Tokuhira Hiroyuki Fujita Miki Ando Yoshihiro Hatta Tatsuya Kawaguchi Source Type: research

Optimized BEAC conditioning regimen improves clinical outcomes of autologous hematopoietic stem cell transplantation in non-Hodgkin lymphomas
Int J Hematol. 2024 Apr 8. doi: 10.1007/s12185-024-03755-7. Online ahead of print.ABSTRACTThe conditioning regimen is an important part of autologous hematopoietic stem cell transplantation (ASCT). We explored the efficacy and safety of an optimized BEAC (adjusted-dose, intermediate-dose cytarabine and reduced-dose cyclophosphamide, AD-BEAC) conditioning regimen for non-Hodgkin lymphoma (NHL). A total of 141 NHL patients received AD-BEAC or a standard-dose BEAC (SD-BEAC) conditioning regimen from January 2007 to December 2017, and 104 patients were included in the study after 1:1 propensity matching. The 5-year overall sur...
Source: International Journal of Hematology - April 8, 2024 Category: Hematology Authors: Sha Zhou Jun Rao Xiangyu Ma Yunjing Zeng Xixi Xiang Jiali Li Hongyun Liu Shijia Lin Song Dong Fu Li Xi Zhang Li Gao Source Type: research

Ex vivo expansion and activation of V γ9Vδ2 T cells by CELMoDs in combination with zoledronic acid
Int J Hematol. 2024 Apr 6. doi: 10.1007/s12185-024-03763-7. Online ahead of print.ABSTRACTAs multiple myeloma (MM) progresses, immune effector cells decrease in number and function and become exhausted. This remains an insurmountable clinical issue that must be addressed by development of novel modalities to revitalize anti-MM immunity. Human Vγ9Vδ2 T (Vδ2+ γδ T) cells serve as the first line of defense against pathogens as well as tumors and can be expanded ex vivo from peripheral blood mononuclear cells (PBMCs) upon treatment with amino-bisphosphonates in combination with IL-2. Here, we demonstrated that next-genera...
Source: International Journal of Hematology - April 6, 2024 Category: Hematology Authors: Yusuke Inoue Asuka Oda Yusaku Maeda Ryohei Sumitani Masahiro Oura Kimiko Sogabe Tomoko Maruhashi Mamiko Takahashi Shiro Fujii Shingen Nakamura Hirokazu Miki Masahiro Hiasa Jumpei Teramachi Takeshi Harada Masahiro Abe Source Type: research