Hiramatsu Y, Urata S, Ishikawa T
… +1 more, Yamauchi T
Int J Hematol
· 2026 Apr · PMID 41774395
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Ivosidenib is a mutant IDH1 (mIDH1) inhibitor that demonstrated clinical benefit in combination with azacitidine for treatment of mIDH1 acute myeloid leukemia (AML) in the phase 3 AGILE trial. Results from AGILE led to t...Ivosidenib is a mutant IDH1 (mIDH1) inhibitor that demonstrated clinical benefit in combination with azacitidine for treatment of mIDH1 acute myeloid leukemia (AML) in the phase 3 AGILE trial. Results from AGILE led to the approval of ivosidenib plus azacitidine for patients with newly diagnosed mIDH1 AML unfit for intensive chemotherapy in Japan. However, data on the use of ivosidenib plus azacitidine in Japanese patients with mIDH1 AML are lacking. Here we present data from six Japanese patients enrolled in AGILE, of whom, three received ivosidenib plus azacitidine and three received placebo plus azacitidine. At data cut-off, Japanese patients in AGILE had an overall survival of 4.4-12.8 months and 24.8-36.5 months in the placebo plus azacitidine and ivosidenib plus azacitidine groups, respectively. Of the Japanese patients enrolled in AGILE, 2 of 3 (66.7%) patients receiving ivosidenib plus azacitidine and none receiving placebo plus azacitidine achieved complete remission within 24 weeks. Results from the Japanese population enrolled in AGILE are aligned with those of the overall study. The efficacy of ivosidenib plus azacitidine in Japanese mIDH1 AML patients enrolled in AGILE who were ineligible for intensive chemotherapy appears to be consistent with the overall study population.
Takahashi N, Kimura S, Matsuki E
… +22 more, Ono T, Doki N, Iino M, Sawa M, Saburi Y, Murai K, Fujimaki K, Kurahashi S, Iriyama N, Onaka T, Sakaida E, Yoshida C, Sato K, Miyamoto T, Takaku T, Shiratsuchi M, Kimura F, Katagiri S, Yamamoto M, Saito AM, Kiyoi H, Matsumura I
Int J Hematol
· 2026 Jul · PMID 41764681
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Treatment-free remission (TFR) is an emerging goal for patients with chronic myeloid leukemia (CML) treated with tyrosine kinase inhibitors (TKI). However, long-term TFR durability in real-world settings remains understu...Treatment-free remission (TFR) is an emerging goal for patients with chronic myeloid leukemia (CML) treated with tyrosine kinase inhibitors (TKI). However, long-term TFR durability in real-world settings remains understudied. The J-SKI study, a large observational study, was conducted to evaluate long-term TFR outcomes in Japanese patients with CML. This interim analysis included 795 eligible patients from the prospective (n = 283) and retrospective (n = 512) cohorts. With a median follow-up of 32 months (range 0.8-168) after TKI discontinuation, the 5-year TFR rate was 65.2% (95% confidence interval [CI]: 59.6-70.6%). Among patients who experienced molecular relapse, 99% (95% CI: 97-100%) regained a major molecular response after resuming TKI therapy, with no observed disease progression. Multivariate analysis identified the duration of deep molecular response (DMR) as the sole independent predictor of successful TFR, with each additional year of DMR reducing the relapse risk by 12.5% (HR: 0.875, p < 0.0001). A second TFR attempt was successful in 41% (95% CI: 22-59%) of the 32 patients. This study demonstrated that TKI discontinuation is a safe and feasible strategy in a real-world clinical setting. A sustained DMR is critical for TFR success, supporting its importance as a key therapeutic objective.
Radhakrishnan N, Singh A, Pandharipande A
… +12 more, Tulsiyan A, Gaire H, Bhattacharya S, Shankar R, Singh S, Jain V, Shukla U, Ghosh U, Agrawal A, Arora S, Dua S, Tripathy S
BACKGROUND: Massive gastrointestinal (GI), intraperitoneal, and pelvic hemorrhage in inherited bleeding disorders (IBDs) is rare but potentially life-threatening. Pediatric data remain limited. METHODS: We retrospectivel...BACKGROUND: Massive gastrointestinal (GI), intraperitoneal, and pelvic hemorrhage in inherited bleeding disorders (IBDs) is rare but potentially life-threatening. Pediatric data remain limited. METHODS: We retrospectively reviewed patients ≤ 18 years with hemophilia A/B, von Willebrand disease (VWD), or rare bleeding disorders admitted with massive abdominal hemorrhage (September 2017-August 2025). Massive GI bleeding was defined as overt bleeding with estimated loss > 70 mL/kg/day or bleeding resulting in shock or transfusion. Intraperitoneal and pelvic hemorrhage required imaging confirmation. Demographics, interventions, and outcomes were analyzed and compared with the pediatric IBD cohort. RESULTS: Of 788 pediatric IBD patients, 10 (1.2%) developed massive abdominal hemorrhage: GI (n = 5), intraperitoneal (n = 3), and pelvic hematoma (n = 2). The GI bleeding cohort was older than the pediatric IBD cohort (median 16 vs. 8 years, p < 0.001). Diagnoses included hemophilia A (n = 4; 75% inhibitor-positive), hemophilia B (n = 1), VWD (n = 4), and Glanzmann thrombasthenia (n = 1). Seven required transfusions; four met massive transfusion criteria. Endoscopy identified a bleeding source in 80% of GI bleeds. Diagnostic delays were longer for intraperitoneal hemorrhage than for GI bleeds (p < 0.05). CONCLUSIONS: Massive abdominal haemorrhage causes significant morbidity. Early imaging, endoscopy, and aggressive hemostatic therapy resulted in 100% survival. Improved access to prophylaxis may prevent such events.
Miyazaki Y, Matsumura I, Arita T
… +2 more, Fukuda R, Yamanaka M
Int J Hematol
· 2026 Jul · PMID 41746494
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Quizartinib is an oral, once-daily, highly potent and selective second-generation, type II FMS-like tyrosine kinase 3 (FLT3) inhibitor, approved for the treatment of newly diagnosed and relapsed/refractory (R/R) FLT3-int...Quizartinib is an oral, once-daily, highly potent and selective second-generation, type II FMS-like tyrosine kinase 3 (FLT3) inhibitor, approved for the treatment of newly diagnosed and relapsed/refractory (R/R) FLT3-internal tandem duplication (ITD)-positive acute myeloid leukemia (AML). This descriptive, observational post-marketing surveillance (PMS) investigated the safety of quizartinib in patients with R/R FLT3-ITD-positive AML who initiated quizartinib between October 2019 and December 2021 in Japan. The safety analysis dataset included 126 patients (mean age 60.4 years) who consented to data publication. Eighty patients (63.5%) had previously received another FLT3 inhibitor. The median treatment duration was 71.0 days. Adverse drug reactions (ADRs) occurred in 68 patients (54.0%); grade ≥ 3 and serious ADRs occurred in 44 (34.9%) and 20 (15.9%) patients, respectively. QT interval prolongation occurred in 18/124 patients (14.5%), and the first event typically occurred < 30 days, but sometimes ≥ 150 days, after quizartinib initiation; none of these patients experienced clinical symptoms associated with the ADR. This PMS showed that the safety profile of quizartinib in routine clinical practice in Japanese patients with R/R FLT3-ITD-positive AML did not differ from the known safety profile of quizartinib. QT interval prolongation was manageable with current risk minimization measures.
Takenaka K, Ito T, Komatsu N
… +28 more, Yamaguchi H, Kirito K, Tomita A, Togano T, Tanaka T, Sugimoto Y, Murai K, Wada H, Kurokawa T, Koike M, Gotoh A, Maekawa T, Kubuki Y, Akagi T, Yamauchi T, Edahiro Y, Ikeda K, Kondo T, Tanaka H, Miyazaki Y, Saito TI, Shimoda K, Kada A, Saito AM, Kiyoi H, Akashi K, Matsumura I, Takaori A
Myeloproliferative neoplasms (MPNs) are clonal hematologic disorders characterized by proliferation of one or more myeloid lineages. Data from large-scale prospective studies in Japan remain limited. We conducted a multi...Myeloproliferative neoplasms (MPNs) are clonal hematologic disorders characterized by proliferation of one or more myeloid lineages. Data from large-scale prospective studies in Japan remain limited. We conducted a multicenter, prospective observational study (MPN-15) for the Japanese Society of Hematology to assess clinical characteristics, mutation profiles, risk stratification, and treatment patterns of patients diagnosed with polycythemia vera (PV), essential thrombocythemia (ET), prefibrotic primary myelofibrosis (pre-PMF), and fibrotic PMF after 2016. A total of 1252 patients were enrolled (PV: 323; ET: 726; MF: 203). JAK2V617F mutations were detected in 96.8% of PV patients and approximately 60% of patients with other subtypes; CALR and MPL mutations were more common in ET, pre-PMF, and fibrotic PMF. Chromosomal abnormalities and symptom burden were highest in fibrotic PMF. Thrombotic and survival risk stratification revealed that most patients with PV and ET were high risk. Use of ruxolitinib was reported in 14% of PV and 34% of fibrotic PMF patients, with no serious adverse events. This study represents the first large-scale prospective MPN registry in Japan. Ongoing follow-up will provide critical insights into long-term outcomes and therapeutic optimization in the Japanese population.
Ishida H, Suenobu S, Imamura T
… +22 more, Shiwaku T, Uemura S, Deguchi T, Miyamura T, Kato K, Hori H, Okada K, Tsujimoto H, Oda M, Endo M, Hasegawa D, Yumura-Yagi K, Takahashi Y, Usami I, Kosaka Y, Iguchi A, Kawasaki H, Sato A, Hara J, Moriya-Saito A, Horibe K, Hashii Y
Int J Hematol
· 2026 Jun · PMID 41701450
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Historically, patients with T-cell acute lymphoblastic leukemia (ALL) had poorer outcomes than those with B-cell ALL. However, with intensive chemotherapy regimens, outcomes for both groups have become similar. From 2002...Historically, patients with T-cell acute lymphoblastic leukemia (ALL) had poorer outcomes than those with B-cell ALL. However, with intensive chemotherapy regimens, outcomes for both groups have become similar. From 2002 to 2008, the Japan Association of Childhood Leukemia Study (JACLS) conducted the prospective ALL-02 study, using a unique protocol for T-ALL (T-02). All T-ALL patients received the same induction therapy, and those who had < 25% bone marrow blasts at day 15 and achieved complete remission (CR) after induction continued on the T-02 protocol. Of the 107 T-ALL patients enrolled, 98 (91.6%) achieved CR after induction. Among 79 patients continuing on the T-02 protocol, the 2-year event-free survival (EFS), the study's primary outcome, was 70.9%, with a cumulative incidence of relapse of 29.1% and no observed nonrelapse mortality. Univariate analysis identified female sex as a significant predictor of better EFS. This study suggests that excessive treatment reduction may lead to poorer outcomes, although female patients with a rapid early response could potentially benefit from less-intensive therapy, warranting further investigation in a larger study.
Int J Hematol
· 2026 Jun · PMID 41686349
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Acute and chronic graft-versus-host disease (GVHD) remains major obstacles to the success of allogeneic hematopoietic cell transplantation (allo-HCT). Recent advances in experimental models and clinical studies have refi...Acute and chronic graft-versus-host disease (GVHD) remains major obstacles to the success of allogeneic hematopoietic cell transplantation (allo-HCT). Recent advances in experimental models and clinical studies have refined our understanding of the cellular and molecular mechanisms that drive GVHD and revealed new therapeutic opportunities. Emerging evidence indicates tissue tolerance mediated by epithelial regeneration from tissue stem cells plays a protective role against GVHD. Furthermore, we recently found tissue stem cells persisting after acute GVHD have epigenetic changes that lead to GVHD exacerbation at GVHD flare. Chronic GVHD develops through a more complex immunopathology involving T and B cells, macrophages, and fibroblasts. Disrupted immune reconstitution, including impaired thymic tolerance, aberrant B-cell activation driven by BAFF, and defective regulatory T-cell recovery, contributes to sustained alloimmunity. T-cell exhaustion has recently been recognized as a central checkpoint: While terminal exhaustion promotes tolerance, early calcineurin inhibitor (CNI) administration suppresses terminal exhaustion and drives the accumulation of transitory exhausted T cells that mediate chronic GVHD while preserving graft-versus-leukemia (GVL) activity. Post-transplant cyclophosphamide (PTCy)-based platforms highlight how delayed CNI initiation reduces chronic GVHD by permitting donor T cells to undergo exhaustion.
Yasue S, Fujimori K, Gocho Y
… +9 more, Osumi T, Iguchi A, Deguchi T, Uchiyama T, Kawai T, Kato M, Tomizawa D, Matsumoto K, Sakaguchi H
Int J Hematol
· 2026 May · PMID 41678000
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Familial hemophagocytic lymphohistiocytosis type 2 (FHL2) is a rare congenital disorder caused by PRF1 mutations that leads to life-threatening hemophagocytic lymphohistiocytosis during infancy. Hematopoietic cell transp...Familial hemophagocytic lymphohistiocytosis type 2 (FHL2) is a rare congenital disorder caused by PRF1 mutations that leads to life-threatening hemophagocytic lymphohistiocytosis during infancy. Hematopoietic cell transplantation (HCT) is the only curative treatment, but optimal transplantation strategies remain unclear. We report two cases of infants with FHL2 successfully treated with cord blood transplantation (CBT) following reduced-intensity conditioning (RIC). Case 1: A 1 month-old boy presented with fever, pancytopenia, and multi-organ failure. Genetic testing identified compound heterozygous PRF1 mutations. After immunochemotherapy, he underwent RIC-CBT at 2 months of age. Engraftment occurred on day 16, complicated by grade II acute graft-versus-host disease (GVHD) and sinusoidal obstruction syndrome, both successfully managed. He remains alive and disease-free 10 years post-transplant with complete donor chimerism. Case 2: A 1 month-old girl presented with fever, respiratory failure, and pancytopenia. She was diagnosed with FHL2 due to a homozygous PRF1 mutation. Following immunochemotherapy, she underwent RIC-CBT at 3 months of age. Engraftment occurred on day 15, without GVHD. She developed remains alive and disease-free 2.5 years later, with stable donor-dominant mixed chimerism (approximately 90%). These cases highlight the feasibility and efficacy of immunochemotherapy followed by RIC-CBT in infants with FHL2.
Int J Hematol
· 2026 Jun · PMID 41670807
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Because primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (POAML) is rare, key clinical questions, including the best treatment strategy and the validity of watchful waiting (WW), remain unresolved. Altho...Because primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (POAML) is rare, key clinical questions, including the best treatment strategy and the validity of watchful waiting (WW), remain unresolved. Although radiotherapy (RT) is the standard treatment for localized POAML, it is commonly associated with acute and late toxicities. Overall, 128 patients with localized POAML diagnosed and managed at our institution between 1998 and 2015 were retrospectively analyzed. Forty-two patients were initially managed with WW, and 86 received immediate RT. During a median follow-up of 7.2 years (range 0.1-18.4), no patients died of lymphoma progression. Seven patients in the WW group experienced disease progression at the primary site. In the RT group, almost all patients (97.7%) achieved a complete response after RT; however, 12 patients relapsed. Histological transformation occurred in one patient in each group. The 10-year cumulative incidence of progression was 24.9% (95% CI, 12.6-45.7%) with WW and 13.1% (95% CI, 6.9-23.9%) with RT (p = 0.27). The rate of freedom from systemic therapy at 10 years was 89.7 and 94.8%, respectively (p = 0.67). This observational study suggests that WW is an acceptable treatment option for selected patients with localized POAML, with no significant differences in long-term outcomes compared with RT.
Fujiwara SI, Kawamura S, Kimura SI
… +22 more, Takeshita J, Tominaga R, Yokoyama D, Noguchi A, Furuki S, Koyama S, Murahashi R, Nakashima H, Hyodo K, Toda Y, Umino K, Minakata D, Gomyo A, Kusuda M, Ashizawa M, Yamamoto C, Hatano K, Sato K, Ohmine K, Nakasone H, Kako S, Kanda Y
Int J Hematol
· 2026 Jun · PMID 41667916
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This study evaluated cytomegalovirus (CMV) reactivation after allogeneic stem cell transplantation (allo-SCT) using both CMV-PCR and antigenemia assays in 109 adult recipients. CMV-PCR and antigenemia values had a modera...This study evaluated cytomegalovirus (CMV) reactivation after allogeneic stem cell transplantation (allo-SCT) using both CMV-PCR and antigenemia assays in 109 adult recipients. CMV-PCR and antigenemia values had a moderate linear correlation. In total, 58 patients exhibited CMV-PCR positivity before starting antigenemia-based preemptive treatment. Excluding the two patients with persistent PCR positivity, the antigenemia value subsequently reached the threshold in 31 of 54 patients. On the other hand, 25 patients had spontaneous clearance of viremia without preemptive treatment. Spontaneous clearance was associated with letermovir use and the absence of graft-versus-host disease. The clinical course of CMV infection was simulated using various CMV-PCR thresholds (range 50-1000 IU/mL). In high-risk patients not treated with letermovir, a threshold of 50 IU/mL enables preemptive treatment initiation without increasing overtreatment risk in patients with spontaneous clearance. However, in high-risk patients treated with letermovir, thresholds > 150 IU/mL delayed the start of preemptive treatment. In low-risk patients, a threshold of 500-750 IU/mL balances avoiding spontaneous resolution and increasing delayed treatment. PCR thresholds of 50 and 150 IU/mL may be appropriate for initiating preemptive therapy in high-risk patients treated and not treated with letermovir, respectively, while 500-750 IU/mL may be optimal for low-risk patients.
Sakurai M, Ueda Y, Obara N
… +8 more, Kroes M, Dochi T, Wiyani A, Balp MM, Somenzi O, Taylor Y, Kawaguchi T, Nishimura JI
Int J Hematol
· 2026 Jun · PMID 41667915
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This study aimed to determine the clinical profile and disease burden of patients with paroxysmal nocturnal hemoglobinuria (PNH) in Japan using real-world data from the Adelphi Real World PNH Disease Specific Programme (...This study aimed to determine the clinical profile and disease burden of patients with paroxysmal nocturnal hemoglobinuria (PNH) in Japan using real-world data from the Adelphi Real World PNH Disease Specific Programme (DSP)™, a cross-sectional survey conducted between January and December 2022. Data included demographics, treatment, and clinical values (hemoglobin [Hb] and lactate dehydrogenase [LDH]). Patient-reported outcome measures included the EQ-5D-5L and the FACIT-Fatigue. Seventeen physicians provided information on 45 patients, among whom 86.7% were receiving treatment with complement 5 inhibitors (C5i). Median (IQR) age was 65.0 (54.5, 73.0) years; 55.6% were male. Among C5i-treated patients (n = 39), 51.3% received ravulizumab and 48.7% eculizumab, for a median (IQR) duration of 1.6 (1.0, 2.7) years. Median (IQR) Hb level was 7.2 (7.0, 8.1) g/dL at diagnosis and 10.0 (8.8, 10.6) g/dL at survey; 91.4% had LDH levels exceeding 1.5 times the upper limit of normal at diagnosis, 11.4% at survey. Twelve patients returned a self-completed questionnaire. Patient-reported symptoms included tiredness (83.3%), shortness of breath (75.0%), and anemia (58.3%). Mean (SD) EQ-5D-5L and FACIT-Fatigue scores were 0.73 (0.16) and 32.3 (7.1). Even C5i-treated patients continued to experience substantial disease burden, highlighting the need for more effective treatments to improve quality of life.
Int J Hematol
· 2026 Feb · PMID 41656394
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Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by complement-dependent intravascular hemolysis and thrombosis as well as bone marrow failure. Complement dysregulation occurs as a result of defective cell surf...Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by complement-dependent intravascular hemolysis and thrombosis as well as bone marrow failure. Complement dysregulation occurs as a result of defective cell surface expression of two glycosylphosphatidylinositol (GPI)-anchored complement regulators, decay-accelerating factor/CD55 and CD59, caused by somatic mutation of the phosphatidylinositol glycan anchor biosynthesis class A gene (PIGA). Somatic loss-of-function mutation of PIGA generates GPI-anchor-defective hematopoietic stem cell clones, the expansion of which results in large numbers of abnormal erythrocytes, platelets, and other blood cells. The clonal expansion of PIGA mutant hematopoietic stem cells is thought to be mediated by an autoimmune mechanism that suppresses or eliminates normal hematopoietic stem cells while sparing GPI-defective stem cell clones under bone marrow failure environments, the acquisition of a growth phenotype, or both of these mechanisms. This review examines current knowledge and views about the clonal expansion mechanism.
Int J Hematol
· 2026 Jun · PMID 41655162
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Graft-versus-host disease (GVHD) remains a major barrier to successful allogeneic hematopoietic stem cell transplantation, and its prevention requires not only suppression of early alloimmune responses but also the ratio...Graft-versus-host disease (GVHD) remains a major barrier to successful allogeneic hematopoietic stem cell transplantation, and its prevention requires not only suppression of early alloimmune responses but also the rational design of post-transplant immune reconstitution. The pathophysiology of GVHD has traditionally been understood based on Billingham's classical three criteria and a subsequent cytokine storm-driven, multistep model that emphasizes early tissue injury. In recent years, however, emerging concepts-including disruption of tissue tolerance in target organs and the layered reconstitution of donor T cells after transplantation, characterized by dynamic changes in fitness and exhaustion-have led to a refinement of these classical frameworks. This review summarizes recent advances, focusing on two key aspects: (1) updated local pathophysiological mechanisms, including injury to tissue stem cells and impaired regenerative capacity in target organs, disruption of the gut microbiota-metabolic network, and damage to the bone marrow hematopoietic niche; and (2) the mechanistic links between immune reconstitution and the development of acute and chronic GVHD, based on recent studies of donor T cell clonal dynamics. These insights support a shift from a unidimensional, immunosuppression-centered approach toward a novel, multidimensional therapeutic strategy that integrates organ protection, hematopoietic niche repair, and precise control of immune reconstitution.
Nakamura T, Suzuki K, Kawashima M
… +13 more, Saito T, Yokoyama H, Katsube A, Fukushima R, Ishii H, Oshima S, Tanoue S, Sakayori Y, Kamitani I, Okuda K, Numata T, Araya J, Yano S
Int J Hematol
· 2026 May · PMID 41632423
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Tumor-associated bronchiolitis obliterans (BO) is a major cause of death and is frequently complicated by paraneoplastic pemphigoid (PNP). In this study, three cases of follicular lymphoma (FL) with BO were retrospective...Tumor-associated bronchiolitis obliterans (BO) is a major cause of death and is frequently complicated by paraneoplastic pemphigoid (PNP). In this study, three cases of follicular lymphoma (FL) with BO were retrospectively analyzed. All cases were complicated by PNP. All patients achieved a tumor response with anti-CD20 monoclonal antibody-containing treatment, and two patients showed clinical improvement in PNP. However, BO continued to progress, and two of the three patients developed pulmonary infections.
Sahara N, Matsunaga T, Chie G
… +5 more, Mizusawa M, Miura Y, Kobayashi S, Fujii T, Ohno N
Int J Hematol
· 2026 May · PMID 41622392
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Relapsed/refractory multiple myeloma (RRMM) with extramedullary disease (EMD) is associated with poor prognosis and limited response to standard therapies. Elranatamab, a bispecific antibody targeting BCMA and CD3, has d...Relapsed/refractory multiple myeloma (RRMM) with extramedullary disease (EMD) is associated with poor prognosis and limited response to standard therapies. Elranatamab, a bispecific antibody targeting BCMA and CD3, has demonstrated clinical activity in triple-class-exposed (TCE) RRMM but remains less effective in patients with EMD. We report a case of a 67-year-old woman with TCE RRMM and aggressive EMD that progressed after two cycles of elranatamab. Following the addition of Juzentaihoto (JTT), a traditional Japanese herbal medicine, rapid symptom improvement, tumor regression, and disappearance of M-protein on immunofixation were observed. Moreover, after JTT initiation, lymphocyte and CD4-CD8bright cell counts increased rapidly and robustly, suggesting that JTT may enhance the elranatamab-induced antitumor immune response via these cells. To our knowledge, this is the first in vivo report indicating that JTT increases CD4-CD8bright lymphocyte counts in humans. JTT may enhance the efficacy of T cell-redirecting immunotherapies in RRMM by promoting cytotoxic T cell expansion. These findings warrant further investigation into the use of JTT as an adjunct to improve outcomes in patients with EMD.
Multiple myeloma is an incurable plasma cell malignancy. Its prognosis improved with the introduction of proteasome inhibitors, immunomodulatory drugs, and monoclonal antibodies, but outcomes for triple-class-exposed (TC...Multiple myeloma is an incurable plasma cell malignancy. Its prognosis improved with the introduction of proteasome inhibitors, immunomodulatory drugs, and monoclonal antibodies, but outcomes for triple-class-exposed (TCE) myeloma remained poor. New therapeutic options including CAR-T cell therapy and bispecific antibodies have now further improved prognosis, even in TCE myeloma. While true cure remains challenging, functional cure, defined as long-term disease control through a favorable immune environment suppressing minimal residual disease (MRD), is currently considered a realistic therapeutic goal.
Int J Hematol
· 2026 Jun · PMID 41604064
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The diagnosis-to-treatment interval (DTI) has been identified as a prognostic factor in newly diagnosed diffuse large B-cell lymphoma (DLBCL). However, its significance in relapsed or refractory DLBCL (R/R DLBCL) remains...The diagnosis-to-treatment interval (DTI) has been identified as a prognostic factor in newly diagnosed diffuse large B-cell lymphoma (DLBCL). However, its significance in relapsed or refractory DLBCL (R/R DLBCL) remains unclear. This study aimed to investigate the impact of DTI on survival outcomes in patients with R/R DLBCL. We reviewed the medical records of patients with R/R DLBCL who received second-line therapy. DTI at relapse or refractory disease (r/r DTI) was defined as the time from radiological diagnosis-to-treatment initiation, with a cut-off of 28 days. The primary endpoint was overall survival (OS). A total of 184 patients with R/R DLBCL were included. Patients with short r/r DTI had significantly worse OS than those with long r/r DTI (1-year OS: 50.4% vs. 79.5%; P < 0.001). Short r/r DTI was associated with adverse clinical features, including poor performance status, elevated lactate dehydrogenase levels, and high International Prognostic Index at relapse. Multivariate analysis demonstrated that short r/r DTI was independently associated with significantly inferior OS (HR 1.77; P = 0.043). In conclusion, DTI is an independent prognostic factor in R/R DLBCL and can be considered in patient selection for clinical trials.
Kaya H, Hamada M, Murayama Y
… +3 more, Mochizuki K, Uchiyama A, Okumura H
Int J Hematol
· 2026 Feb · PMID 41604063
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Micafungin and other echinocandins are commonly used for the prophylaxis and empirical treatment of invasive fungal infections in neutropenic patients due to their broad-spectrum activity and favorable safety profile. Dr...Micafungin and other echinocandins are commonly used for the prophylaxis and empirical treatment of invasive fungal infections in neutropenic patients due to their broad-spectrum activity and favorable safety profile. Drug-induced hemolytic anemia (DIHA) is a rare condition, and micafungin-related cases are exceptionally uncommon. We describe a fatal case of intravascular hemolysis triggered by re-administration of micafungin in a patient receiving chemotherapy for peripheral T cell lymphoma. The patient experienced convulsions and shock within minutes of infusion of the drug, followed by severe hemolysis and disseminated intravascular coagulation. Subsequently, hemolytic mechanisms were evaluated using drug-adsorption and immune complex models. Additionally, we had sera from eight patients treated with micafungin between January 2019 and December 2022, including our index case, tested for anti-micafungin antibodies. Immune complex-mediated hemolysis was confirmed in the index case, supported by positive indirect antiglobulin tests and in vitro hemolysis. Anti-micafungin antibodies were only detected in this single patient, indicating an extremely rare immunologic reaction. Although rare, micafungin-induced DIHA can be life-threatening. Clinicians should remain vigilant, particularly when resuming or continuing administration of micafungin.