Previous studies investigating the role of TP53 mutations in chronic phase MPN have yielded inconsistent results. As such, the clinical relevance of these mutations remains to be elucidated. We report a case of a 67-year-old woman with a leukemic transformation of a post-polycythaemia vera myelofibrosis (post-PV MF) that culminated in the rare development of a myeloid sarcoma. During a 4-year follow-up, the patient had a stable JAK2 Val617Phe and NFE2 mutation (Variant Allele Frequencies (VAF's) 85%-87% and 43%-50%, respectively) and low-burden TP53 mutation (VAF 2%-7%) in blood and bone marrow. Despite having a low-risk post-PV MF, the patient soon presented with an aggressive lytic lesion in the humerus, which proved to be a myeloid sarcoma. The sarcoma was positive for JAK2 Val617Phe but, interestingly, was also highly enriched for the TP53 mutation (VAF 81%), implicating a role of the TP53 mutation in the leukemic progression. This case provides molecular evidence that a TP53 mutation, even at a low burden, may contribute to leukemic progression of a chronic phase myeloproliferative neoplasm (MPN). These observations increase our understanding of the pathophysiologic behavior of TP53 mutations and underscore the importance of further causal research towards the clinical implications of these mutations in chronic phase MPN.

Following treatment, relapse of Multiple Myeloma (MM) occurs due to measurable residual disease (MRD). As therapeutic options expand, advances in response assessment become more critical, necessitating more sensitive MRD detection methods.

Prognostic models in Waldenström's macroglobulinemia (WM) are typically static, baseline tools applied before treatment initiation and do not account for dynamic post-treatment factors. We evaluated time to next treatment within 24 months (TTNT24), as a prognostic marker in symptomatic patients, and time to lymphoma treatment within 24 months (TTLT24) in initially observed asymptomatic patients.

Central venous catheters (CVCs) are essential in pediatric hematology-oncology, for the administration of chemotherapy and supportive therapy. Thrombocytopenia increases the risk of bleeding and current guidelines recommend prophylactic platelet transfusions below 40-50 × 10/L, though evidence is limited and transfusions entail risks and costs. Advances in procedural bundles and simulation-based training may enhance safety, enabling lower thresholds.

To determine the diagnostic value of disclosing monotypic T-cell populations by expression analysis of the constant region 1 of T-cell receptor β (TRBC1) by flow cytometry, for the detection of T-cell neoplasms, in a routine hematopathology practice setting.

Relapsed/refractory (R/R) mantle cell lymphoma (MCL) remains a therapeutic challenge, particularly in patients with high-risk features or prior exposure to Bruton's tyrosine kinase inhibitors (BTKis). The advent of T-cell-redirecting immunotherapies, including chimeric antigen receptor T-cell (CAR-T) therapy and bispecific antibodies (BsAbs), has transformed the treatment landscape. CAR-T therapies, such as brexu-cel and liso-cel, induce high overall response rates and durable remissions, even in heavily pretreated or BTKi-refractory patients. However, CAR-T administration is limited by logistical constraints, the need for bridging therapy, and the risk of severe toxicities, including cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS). BsAbs, targeting CD20 and CD3, offer an off-the-shelf, repeatable immunotherapeutic option suitable for outpatient use, with generally manageable toxicities. Step-up dosing, corticosteroids, and anti-IL6 therapy mitigate CRS, while hematologic toxicity and infections require vigilant monitoring. Clinical data indicate that BsAbs are active in both CAR-T-naïve and post-CAR-T settings, providing disease control in patients ineligible for immediate CAR-T therapy. Emerging evidence supports rational sequencing and combinatorial strategies to optimize outcomes. BsAbs may be employed as a bridge to CAR-T, or CAR-T may be used to consolidate BsAb-induced remissions. Combination regimens, including CAR-T or BsAbs with BTK inhibitors or other targeted agents, are under investigation to enhance the depth and durability of response. In conclusion, CAR-T and BsAbs are complementary modalities in R/R MCL. Individualized therapeutic sequencing and rational combinations, tailored to disease biology and patient characteristics, represent the next frontier for improving long-term outcomes in this historically high-risk population.

Alpha thalassemia is characterized by the reduced or absent synthesis of alpha globin chains, most commonly due to deletional mutations. Hb Agrinio [α29(B10) Leu→Pro, CTG>CCG (α2)] is an infrequent nondeletional alpha thalassemia (α-Thal) that has been described in less than 20 individuals in the world. We presented the case of a girl with anemia since birth and high transfusion needs, with a recent diagnosis of homozygous Hb Agrinio.

Response assessment during treatment of multiple myeloma (MM) typically relies on immunofixation and serum electrophoresis. However, low levels of IgG and especially IgA paraprotein are difficult to quantify reliably. The Hevylite Assay quantifies the kappa and lambda fractions of IgG and IgA separately and is useful to determine response to therapy. Using serum samples of 360 evaluable patients from the prospective GMMG-MM5 trial (EudraCT-No. 2010-019173-16) we assessed the normalization of the kappa/lambda ratio with the Hevylite Assay (HLCr) at baseline, after induction, mobilization, autologous blood stem cell transplantation, consolidation and every three months during maintenance or follow-up within two years after the end of consolidation. We observed a steady increase in the proportion of patients with normalized HLCr over the course of therapyAchieving HLCr normalization any time until the end of consolidation was associated with a trend towards a prolonged progression-free survival (PFS; hazard ratio (HR) = 0.75, 95% confidence interval (95% CI) = 0.56-1.01, p = 0.06) but not overall survival (OS; HR = 0.94, 95% CI = 0.69-1.26, p = 0.66) in multivariable time-dependent Cox regression analyses. Using a landmark analysis from the end of consolidation there was again a marginally statistically significant effect of HLCr normalization by the end of consolidation on PFS using a multivariable Cox model on the subset of the two study arms with continuous lenalidomide maintenance (HR 0.61, 95% CI 0.37-1.02, p = 0.06). No such effect was observed in study arms in which maintenance was only applied to patients not in CR at the end of consolidation. In conclusion, our analysis of the Hevylite Assay in patients with IgG or IgA myeloma from the GMMG-MM5 study did not find evidence to support the general use of HLCr normalization as a response parameter for predicting PFS or OS. However, the differential effects of HLCr normalization depending on the way in which treatment was adapted to response may be of interest for future study designs on response-adapted therapy. Trial Registration: ISRCTN05622749.

Identifying patients with a higher risk of recidivate/refractory Hodgkin lymphoma (R/R HL) is a challenge that needs to be addressed. The International Prognostic System (IPS) identifies patients with a poor prognosis in advanced stages, although its relevance has decreased due to advancements in modern treatment. The interim PET-2 scan after two chemotherapy cycles is now considered the most crucial prognostic tool, but it is only available after treatment initiation.

Infections remain a leading cause of morbidity and mortality in patients with chronic lymphocytic leukemia (CLL), reflecting both intrinsic immune dysfunction and therapy-related immunosuppression. The pathogenesis of immunodeficiency in CLL is multifactorial: neoplastic B cells impair humoral immunity, T cells are functionally exhausted, and innate immune cells, particularly neutrophils and NK cells, display profound defects. Beyond impaired pathogen defense, these immune alterations actively support leukemic cell survival and promote a tolerogenic microenvironment. The advent of targeted therapies has reshaped the infectious risk profile. Bruton's tyrosine kinase inhibitors (BTKis) and venetoclax have largely replaced chemotherapy, reducing classic opportunistic infections but introducing new challenges. BTKis are associated with invasive fungal infections and increased pneumonia risk in combination regimens, while venetoclax frequently induces profound neutropenia. Anti-CD20 monoclonal antibodies cause long-lasting B-cell depletion and viral reactivation. These evolving risks demand nuanced approaches to prevention. Prophylactic strategies must be individualized. Antiviral prophylaxis is warranted with anti-CD20 antibodies and BTKis, and Pneumocystis jirovecii pneumonia (PJP) prophylaxis remains essential with fludarabine, cyclophosphamide, and rituximab (FCR) or prolonged corticosteroid therapy. Antifungal prophylaxis is not routinely indicated in CLL but may be considered in high-risk patients on BTKis or with refractory disease. Immunoglobulin replacement therapy (IgRT) reduces recurrent bacterial infections in patients with hypogammaglobulinemia, while vaccination-though often limited by suboptimal responses-remains the cornerstone of prevention. Timing before therapy or during treatment-free intervals is critical, and newer formulations, such as conjugate pneumococcal and recombinant zoster vaccines, are preferred. Future directions include developing predictive biomarkers of infection risk and vaccine responsiveness, integrating immune monitoring into clinical trials, and exploring strategies to modulate neutrophil plasticity and restore T-cell function. Until then, a pragmatic, risk-adapted approach combining vigilance, prophylaxis, immunoglobulin replacement, and optimized vaccination offers the best safeguard for patients with CLL.

To evaluate the safety and efficacy of the C5-inhibitor crovalimab in patients with paroxysmal nocturnal hemoglobinuria (PNH).

BV + AVD is increasingly used for frontline treatment of stage III/IV cHL. Young adults and adults (Ya&A) were the most common patients treated with BV + AVD in clinical trials but have not been studied in non-trial settings. We conducted a real-life study in secondary and tertiary cancer centers to evaluate the PFS in 18-59 years aged patients who were scheduled to receive six BV + AVD for newly diagnosed advanced stage cHL. This is the largest cohort of Ya&A reported to date including 150 patients from four clinical institutions in Southern Italy, all of which employed structured supportive care programs for HL. Fifty patients (30%) had at least one ECHELON-1 ineligibility criterion, including comorbidities and/or adverse performance status. All 150 patients underwent BV + AVD with a median relative dose intensity of 100% (dose reduction and/or discontinuation ≥ 15%, in 11% of them). At end-of-treatment (EoT) FDG-PET, 93% (140/147) of patients (three did not undergo EoT FDG-PET due to early grade 5 toxicity) achieved a complete response (95% CI, 88.1-96.8). Altogether, four patients (2.7%) received consolidation radiotherapy of residual nodal masses with a Deauville score of 4. Grade ≥ 2 peripheral neuropathy, cardiotoxicity, and febrile neutropenia were reported by 13%, 7%, and 3% of patients, respectively. With a 24 month median follow-up, PFS in the entire analyzed population was 91% (95% CI, 0.864-0.958). In Ya&A with high-risk cHL, our data suggest that a BV-driven strategy (without bleomycin and consolidation radiotherapy) is an effective up-front option in oncologic centers specialized in HL care, improving the rate of durable complete remission in routine clinical practice. Trial Registration: ClinicalTrials.gov identifier: NCT06857500.

Perceptions and experiences of shared decision-making between patients and physicians in multiple myeloma (MM) care are not fully understood. A survey study was conducted to explore these perspectives in Spain.

Totally implantable venous access devices (TIVADs) are essential in pediatric oncology but can cause mechanical complications at removal. Identifying risk factors helps guide management and timing of elective removal. Our objective is to describe the incidence and determinants of mechanical complications that occur during the removal of TIVADs in a Chilean tertiary pediatric oncology center.

Hematologic malignancies represent the most common cancers in children. While the mainstays of treatment are chemotherapy and potentially hematopoietic stem cell transplant, minimally invasive surgery (MIS) has a role in the diagnosis and management of complications related both to disease and therapy as well as common pediatric surgical conditions. MIS can be considered in the management of adverse events of disease and therapy such as pneumatosis, bowel perforation, or pneumoperitoneum in stable patients as a first-look alternative to open surgery. Additionally, given the extent of therapy for many children, common surgical problems such as the need for enteral access, appendicitis, and cholecystitis can occur, and laparoscopic options should be considered for management to enable a faster return to chemotherapy. Finally, MIS can assist with diagnosis through lymph node or tissue biopsy. The utilization of MIS in pediatric patients with hematologic malignancies is a safe and feasible approach for a variety of surgical problems. Nevertheless, current evidence is largely limited to retrospective series and case reports, and prospective multicenter studies will be required to validate these approaches and emerging technologies such as augmented reality and robotics.

Primary cutaneous B-cell lymphomas (CBCL) represent a clinically and biologically heterogeneous group of extranodal non-Hodgkin lymphomas confined to the skin at the time of diagnosis. They account for approximately 25% of all primary cutaneous lymphomas and are subclassified into distinct entities according to the World Health Organization-European Organization of Research and Treatment of Cancer (WHO-EORTC) classification and International Consensus Classification (ICC). including primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), intravascular large B-cell lymphoma (IVLBCL) and Epstein-Barr virus-positive mucocutaneous ulcer (EBVMCU). These subtypes differ significantly in clinical behavior, histopathological features, molecular alterations, and prognosis. Indolent forms such as PCFCL and PCMZL are typically managed with local therapies and are associated with an excellent prognosis. In contrast, aggressive variants such as PCDLBCL-LT require systemic treatment and are linked to poorer outcomes. EBVMCU, despite its alarming histological appearance, generally follows a benign and self-limiting course. This review provides an updated overview of the current diagnostic criteria, clinical management strategies, and emerging molecular insights for each CBCL subtype. It also emphasizes the importance of a multidisciplinary approach and discusses the challenges of prognostication, along with the evolving but still limited role of innovative therapies.

Hematopoietic stem cell transplantation (HSCT) involves the intravenous infusion of stem cells to restore bone marrow function in patients with bone marrow disorders. This study aimed to evaluate the complications of HSCT, mainly gastrointestinal, hepatobiliary, urinary, and pulmonary complications, and their incidence, severity, impacts on survival, and the importance of the surgical role in managing these complications. We retrospectively studied 433 patients treated at the IOP-GRAACC/Unifesp for a period of 10 years, with an average age of 8.8 years. The most frequent primary diseases were leukemia (33.5%) and lymphoma (13.2%). Most patients had complications (96.3%), of which half had only one complication (51.3%). Approximately 298 patients had gastrointestinal complications, with mucositis being the most common one (95.3%). Approximately 6.7% of the patients had some hepatobiliary complications; sinusoidal obstruction syndrome (SOS) occurred in 72.4% of these. Only 9.2% of patients had hemorrhagic cystitis, and most cases were classified as acute, grade I, or mild. Pulmonary complications including viral pneumonia (35.9%), bacterial pneumonia (23.1%), and fungal pneumonia (15.4%) were also recorded. Only 2.1% of patients had aspergillosis. Approximately 37.6% of the patients died, 55.2% were alive, and 7.2% were alive with the disease. The most frequent cause of death among patients was disease progression (72.4%). Only two patients underwent surgery. We concluded that the incidence of HSCT complications was 96.3%; however, it was not possible to determine the risk factors for the need for surgical treatment in these patients, although surgical evaluation in the decision-making process remains essential.

Acute myeloid leukemia (AML) in elderly patients presents a major therapeutic challenge, as many are deemed unfit for intensive chemotherapy due to age, comorbidities, or frailty. Venetoclax in combination with hypomethylating agents (HMA) has emerged as a standard-of-care for this population, yet outcomes remain heterogeneous and predictive tools are limited. In this retrospective single-center study, we analyzed 52 treatment-naïve AML patients receiving venetoclax combined with either azacitidine (n = 33) or decitabine (n = 19) at the Hematology Department of Cosenza Hospital between August 2021 and June 2025. Frailty was assessed using the Clinical Frailty Scale (CSHA CFS), with 19 patients classified as low frailty (score ≤ 3) and 33 as high frailty (score > 3). The median age was 75.3 years (range 58.2-89.2), and the cohort included 33 de novo and 19 secondary AML cases. After a median follow-up of 18 months, 34 patients (65.4%) had died: 14 due to disease progression, 12 due to treatment-related toxicity-predominantly severe infections-and 5 from unrelated causes. ROC curve analysis showed that a CFS score > 3 was associated with worse survival (AUC 0.75, 95% CI 0.61-0.89, p < 0.004), with median overall survival of 7.6 months for low-frailty patients versus 2.5 months for high-frailty patients (1-year OS 44.1% vs. 18.7%, p = 0.031). Multivariate analysis confirmed that lower frailty (p = 0.031) and azacitidine-based therapy (p = 0.025) were independently associated with improved survival. Overall response rate was 48%, including 21 complete responses (CR/CRi) and 4 partial responses. Frailty was the only significant predictor of response (p = 0.005), whereas age, sex, type of AML, ELN risk score, renal function, BMI, or type of HMA did not significantly influence outcomes. Grade 3-4 treatment-related adverse events occurred in all patients, predominantly hematological; non-hematological events included infections (61.9%), cardiotoxicity (11.9%), and liver toxicity (9.5%). High-frailty patients experienced a higher incidence of infections (72.7% vs. 36.8%, p = 0.02) and hospitalizations (57.6% vs. 21.1%, p = 0.011). These results suggest that the CSHA CFS is a simple and clinically meaningful tool to stratify elderly AML patients for venetoclax-HMA therapy, identifying those at higher risk of treatment-related complications and poor survival. Incorporating frailty assessment into routine practice may enhance patient selection, optimize supportive care, and guide individualized therapeutic decisions. Prospective, multicenter studies are warranted to validate these findings and refine the use of frailty-guided treatment strategies in this vulnerable population.

Spontaneous remission (SR) in pediatric acute myeloid leukemia (AML-M5) is extremely rare and is typically characterized by short duration and high relapse risk. We report a case of AML-M5 in a child at Yunnan Children's Medical Center who achieved SR following blood transfusions and anti-infective therapy, without leukemia-specific treatment, but relapsed one month later. A comprehensive literature review was also conducted to explore factors contributing to SR in pediatric leukemia, with the aim of informing treatment protocols and improving outcomes for AML patients.

Determine self-administered bleeding assessment tool (self-BAT) scores in individuals with verified bleeding disorders, and determine sensitivity and specificity of different cut-off values.