Acta Haematologica最新文献

Introduction: Early detection of fever is crucial in neutropenic patients. Lack of precision in body temperature measurement can occur with peripheral site measurements. Furthermore, intermittent temperature monitoring may result in a delay in fever detection.

Methods: We conducted a prospective study in hematologic intensive care unit (HICU) patients receiving autologous stem cell transplant or intensive chemotherapy in order to compare tympanic and enteral temperature measured by an ingested electronic pill (BodyCap®, France).

Results: Twenty-six patients ingested at least one capsule with a total of 218 days of tympanic and enteral simultaneous measurement. In 12% of these measurements, we identified a difference over 0.5°C between the left ear versus right ear measurements. Enteral temperature was usually higher than the tympanic one (p < 0.0001) with 14% of the measurements that were discordant with higher enteral temperature and 1.9% that were discordant with higher tympanic temperature. Febrile episodes were detected with the ingestible pill on average almost 24 h earlier than with the tympanic measurement. Early capsule evacuation occurred in 20.4% of the cases, mainly because of diarrhea, a frequent adverse event in these patients. Patients were satisfied with capsule ingestion according to a questionnaire.

Conclusion: In this prospective trial (TEMPET), we demonstrated that enteral temperature measured by an ingested pill is feasible in HICU and detects fever earlier than tympanic routine measurements. Digestive symptoms related to hematological disease and/or treatments are limiting factors for its generalized usage.

Introduction: Acute epiglottitis is a medical emergency characterized by inflammation of supraglottic structures of the larynx and epiglottis. The clinical characteristics and course of this complication in acute leukemia (AL) are largely unknown. We present a case series and a systematic review of the literature of adult patients with AL and acute epiglottitis.

Case presentation: Four patients with acute myeloid leukemia (age 38-61 years) were diagnosed with acute epiglottitis during their intensive treatment course. One patient presented with epiglottitis at the end of induction, two at the end of consolidation therapy, and 1 patient presented with a later event. Three patients were in grade 4 neutropenia during infection onset (median aplasia duration to event 9 [range 4-14] days). All patients presented with neck pain and dysphagia and 2 patients also had dyspnea at presentation. Urgent invasive airway protection was required in 3 patients. Two patients died due to this complication, one suffered anoxic brain injury. A systematic literature review identified 8 additional cases of epiglottitis.

Conclusion: Acute epiglottis is a rare but significant medical emergency with unique challenges in the setting of AL that is caused by atypical infectious pathogens. Early detection and a multidisciplinary therapeutic effort are crucial to improve patient outcome.

Introduction: We conducted a single-arm, open-label dose-exploration study to evaluate the safety and efficacy of the histone deacetylase inhibitor tucidinostat combined with bortezomib, liposomal doxorubicin, and dexamethasone (C-PDD) in treating relapsed and refractory multiple myeloma (RRMM) patients.

Methods: Eighteen patients were enrolled from August 2020 to May 2021, receiving 21-day cycles of C-PDD.

Results: Eighteen cases were analysed, with a median prior treatment line of 2 (range: 1-4). The median number of completed treatment cycles was 4 (range: 1-8). The overall response rate was 57%, including 14% complete response, 14% very good partial response, and 29% partial response. Both bortezomib-sensitive and refractory groups had a response rate of 57%. The response rate was 100% in patients with extramedullary extraosseous involvement. The median follow-up was 42 months (range: 3-44), with median progression-free survival of 7 months and median overall survival of 24.5 months. Grade 3-4 haematologic adverse events included thrombocytopaenia (50%), neutropenia (33%), and anaemia (33%). Non-haematologic adverse events were mostly grade 1-2, with 1 case of grade 3 peripheral sensory neuropathy.

Conclusion: The C-PDD regimen showed efficacy in RRMM, including bortezomib-refractory disease and EME patients. The optimal dose and combination need to be explored in the future.

Introduction: Recent advances in genomic research have expanded the treatment landscape for acute myeloid leukemia (AML). This study examined treatment patterns and clinical outcomes among relapsed/refractory (R/R) FMS-like tyrosine kinase 3 (FLT3)-mutated AML patients.

Methods: This retrospective longitudinal study included patients with confirmed AML diagnosis, FLT3 mutation, and 1st R/R event from 1/1/2015 to 1/31/2023 in the ConcertAI Oncology Dataset. Treatment patterns, FLT3 testing rates, real-world overall survival (rwOS), and real-world time to next treatment (rwTTNT) were studied.

Results: Among the 336 treated patients, 50.6% received FLT3-tyrosine kinase inhibitors (FLT3-TKIs) as first treatment after R/R event, of which 51.8% received gilteritinib. High-intensity chemotherapy used as first treatment after R/R event decreased from 67.9% in 2015 to 20.0% in 2022, while FLT3-TKI utilization rose to 50% over the same period. Among the 246 patients tested for FLT3 at initial AML diagnosis, only 36% were retested at 1st R/R event. Median rwOS and rwTTNT among FLT3-TKI patients were 12.4 months and 2.9 months, respectively.

Conclusion: This study reveals a trend toward increasing FLT3-TKI use and highlights the need for repeated FLT3 testing among R/R AML patients. Real-world evidence is vital in understanding R/R AML patient care amidst emerging therapies.

Introduction: Leukemia is a group of diseases caused by malignant hematopoietic stem cell clones. Leukemia ranks 13th in incidence and 10th in mortality, according to the Global Cancer Statistics 2022.

Methods: We computed the prevalence, incidence, mortality, and disability-adjusted life years (DALYs) using the Global Burden of Disease (GBD) 2021 data. We rigorously tested the time trend from 1990 to 2021 using Joinpoint regression analysis. This method facilitates the calculation of annual percentage change (APC), average annual percentage change, and 95% confidence interval (CI).

Results: In 2021, the age-standardized prevalence rate (ASPR), age-standardized incidence rate (ASIR), age-standardized mortality rate (ASMR), and age-standardized DALYs rate of leukemia were 21.07 per 100,000 people (95% CI: 17.65-23.61), 5.63 per 100,000 people (95% CI: 4.83-6.17), 3.89 per 100,000 people (95% CI: 3.34-4.25), and 136.94 per 100,000 people (95% CI: 111.89-153.71), respectively. These figures have been obtained globally. Gender comparisons show that men have a higher burden of disease. The 90-94 age-group has the highest global prevalence of leukemia, with the prevalence increasing with age. Age-standardized DALYs rates, ASMR, and ASIR demonstrated a general declining trend from 1990 to 2021. The results of the Joinpoint analysis showed that between 2019 and 2021, there was a drop in the global ASPR (APC = -2.68%; 95% CI: -4.76% to -0.54%; p = 0.017), ASIR (APC = -2.46%; 95% CI: -3.33% to -1.59%; p < 0.001), and ASMR (APC = -1.87%; 95% CI: -2.75% to -0.99%; p < 0.001).

Conclusion: The results indicated an overall decrease in the burden of leukemia. These results provide important epidemiological data for the development of novel treatments.

Background: The advent of molecularly cloned hematopoietic growth factors (rhuG-CSF and rhuGM-CSF) enhanced the safety in treating acute myeloid leukemia (AML) by reducing the duration of neutropenia and thus decreasing the risks for infection. However, early in vitro studies demonstrated leukemia cell proliferation in response to these agents, raising reasonable concerns related to whether these factors can cause or contribute to relapse or progression of AML.

Summary: Clinical studies using recombinant myeloid hematopoietic growth factors have supported their safety, as there is little or no clear evidence for an association with an increased risk of relapse in AML in patients, regardless of the hematopoietic growth factor used, or whether the setting of AML is newly diagnosed or relapsed/refractory. One exception may be in the pediatric population, though this effect might reflect a different isoform of the G-CSF receptor expressed on the AML cells, as this truncated receptor is also seen in severe congenital neutropenia and aplastic anemia and underlies the increased myeloid malignancies that develop in these diseases after long-term exposure to growth factors.

Key messages: The current data support the use of rhuG-CSF and rhuGM-CSF in most patient populations with AML. Future studies should explore the factors influencing hematopoietic growth factor sensitivity in AML subpopulations to guide therapeutic decisions.

Introduction: Sarcopenia, defined by reduced muscle strength, mass, and performance, presents a significant challenge in cancer care due to its impact on treatment outcomes, quality of life, and survival. This study aimed to assess its prevalence in newly diagnosed lymphoma patients.

Methods: Adults planned for first-line anthracycline-based chemotherapy were enrolled and screened for sarcopenia before treatment. Sarcopenia was defined by the European guidelines (EWGSOP2) using low muscle strength (hand-grip), low muscle mass (DXA), and low physical performance (gait speed).

Results: Sixty-nine patients (mean age 57, 19 women) were included. Six patients (9%) had low hand-grip strength, 15 (22%) had low muscle mass, and 4 (6%) demonstrated low gait speed. Two patients met the criteria for sarcopenia, with one having severe sarcopenia.

Conclusion: Sarcopenia prevalence was 3%, but 22% had low muscle mass, suggesting muscle strength alone may not be an optimal screening tool for lymphoma patients.

Introduction: Acquired factor X (FX) deficiency is a rare coagulopathy. Occurrences in plasma cell dyscrasias (PCDs) independent of amyloid light-chain amyloidosis are exceedingly rare.

Case presentation: This case report presents a rare occurrence of acquired FX deficiency in a patient with multiple myeloma (MM) without concomitant amyloidosis. The patient, a 64-year-old male with prior diagnosis of smoldering MM, presented with abdominal pain and chronic bloody diarrheas and was diagnosed with absolute FX deficiency. Despite initial suspicion of amyloidosis, subsequent investigations ruled out its presence. Treatment with anti-myeloma therapy and supportive measures resulted in the normalization of coagulation parameters.

Conclusion: This case underscores the importance of considering acquired FX deficiency in PCD patients presenting with coagulopathy even in the absence of amyloidosis.

Introduction: Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia that has become highly curable with advances in targeted therapy. However, central nervous system (CNS) involvement is exceedingly rare in APL and presents significant therapeutic challenges due to the limited penetrance of standard therapies across the blood-brain barrier (BBB). While APL is traditionally managed without chemotherapy, cases with CNS involvement require a multimodal approach for effective disease control.

Case presentation: We present a unique case of a 31-year-old male with de novo APL and CNS involvement at the presentation, including a clival mass. The patient was successfully treated with a combination of systemic chemotherapy, intrathecal chemotherapy, and craniospinal irradiation, leading to durable remission.

Conclusion: This case highlights the rarity of CNS involvement in APL and underscores the importance of a multidisciplinary approach in its management. Additionally, it emphasizes the need to address logistical barriers to treatment to achieve optimal patient outcomes.

Introduction: Mind-body interventions (MBIs) are therapeutic practices that target the interactions between cognitive, emotional, and physiological systems to influence health outcomes. Previously, we demonstrated that MBI prolonged lymphocyte doubling time and treatment-free survival (TFS) in treatment-naïve chronic lymphocytic leukemia (CLL) patients during the watch-and-wait phase. In this follow-up study, we investigated the long-term effects of MBI on TFS after the intervention ceased.

Methods: Sixty participants from the initial study (34 who received intervention vs. 26 controls) were followed for an additional period of 20 months. TFS was assessed from the end of the intervention to the initiation of CLL therapy or death, using Kaplan-Meier analysis and the log-rank test.

Results: By the end of the follow-up, 9 participants who previously received MBI and 6 controls initiated CLL treatment. No significant difference in TFS was found between the groups (log-rank test, p = 0.65).

Conclusion: While MBI provided a clear advantage as long as it continued, our follow-up analysis suggests this effect diminishes after the intervention ends. Continuous or repeated MBI may be necessary for sustained improvements in TFS.