Expert Review of Hematology

Key features of the underlying pathophysiology of Transfusion-related acute lung injury
Kapur R
Transfusion-related acute lung injury (TRALI) remains a leading cause of blood transfusion associated mortality, particularly in the intensive care unit. TRALI is underrecognized, underreported and lacks specific biomarkers and clinical therapies.
Novel approaches for preventing COVID-19 infection in immunocompromised patients with hematologic malignancies
Shoham S, Focosi D, Franchini M and Atamna A
COVID-19 is a continuing challenge for immunocompromised patients with hematological malignancies. Such patients are at increased risk for complications, including hospitalization, respiratory failure, delayed anti-cancer therapies, and even death. In addition to non-pharmacologic interventions, the main strategies for prevention in such patients are vaccination and pre-exposure prophylaxis.
Quantitative MRI evaluation of iron deposition in patients with transfusion-dependent thalassemia: clinical management insights
Ning X, Peng F, Tan S, Tang C, Luo C, Xiao F, Zhao C and Peng P
In patients with thalassemia, different organs are affected differently by iron overload. Nevertheless, the reasons for this could be the same key transporters. This study investigated the iron deposition in different organs of transfusion-dependent thalassemia (TDT) patients and its correlation.
A review of the isocitrate dehydrogenase inhibitors in management of adult patients with AML and MDS
Norman M, Yamartino K, Gerstein R, Shallis R, Mendez L, Podoltsev N, Stahl M, Eighmy W and Zeidan AM
The development of oral therapies impacts the management of acute myeloid leukemia and myelodysplastic syndromes, especially for targetable mutations including .
What are the therapeutic options for previously treated myelofibrosis?
Petit C, de Lavallade H and Harrison C
The disruption of the JAK/STAT signaling pathway is a defining feature of myelofibrosis (MF). The introduction of JAK inhibitors (JAKi) has transformed the therapeutic approach to MF, becoming essential to treatment and reshaping the management landscape. While JAKi are now the preferred first-line treatment for most patients, various management options are available for those who do not respond to initial therapy.
Identification of miR-451 target genes as prognostic markers in diffuse large B-cell lymphoma
Zhang Y, Wei Z, Xu H, Wang X, Gu T, Dong H, Chang H and Pang L
B-cell lymphoma, a diverse malignancy, is intricately regulated by multiple factors. MicroRNAs (miRNAs) have been demonstrated to be important regulators of the initiation and progression of human B-cell lymphoma, but their functions need to be further explored.
Late effects of hemopoietic stem cell transplant for sickle cell disease: monitoring and management
Goldenberg M, Lanzkron S and Pecker LH
Allogeneic hemopoietic stem cell transplantation (HSCT) is a curative therapy for sickle cell disease (SCD). Exposure to both SCD and HSCT conditioning regimens is associated with late health effects.
Evolving strategies to overcome barriers in CAR-T cell therapy for acute myeloid leukemia
Colonne CK, Kimble EL and Turtle CJ
Acute myeloid leukemia (AML) is a complex and heterogeneous disease characterized by an aggressive clinical course and limited efficacious treatment options in the relapsed/refractory (R/R) setting. Chimeric antigen receptor (CAR)-modified T (CAR-T) cell immunotherapy is an investigational treatment strategy for R/R AML that has shown some promise. However, obstacles to successful CAR-T cell immunotherapy for AML remain.
Combination therapy of dexamethasone, rituximab, and cyclosporine for adults with primary immune thrombocytopenia: a prospective observational study
Liang Y, Tang C, Liu C, Zhang L, Fang Y, Zhao A and Chen N
To assess the effectiveness of the combination of dexamethasone, rituximab, and cyclosporine in treating adults with primary immune thrombocytopenia (ITP).
Global prophylaxis trends in hemophilia: a macroeconomic analysis and its association with world development indicators
John MJ, Stonebraker J, Jindal A, Tootoonchian E, Pierce GP, Gouider E, Jain A and Coffin D
Prophylaxis is the recommended management strategy for all persons with hemophilia (PwH), yet its adoption is uneven worldwide.
Crispr-based gene therapy for the induction of fetal hemoglobin in sickle cell disease
McManus M, Frangoul H and Steinberg MH
Sickle cell disease is ameliorated, and perhaps can be 'cured' if enough fetal hemoglobin is present in most erythrocytes. Hydroxyurea, which increases fetal hemoglobin levels, is widely available and effective, especially in children. Nevertheless, only cell-based gene therapy can achieve a 'curative' fetal hemoglobin threshold.
BCL-2 inhibition in acute myeloid leukemia: resistance and combinations
Tatarata QZ, Wang Z and Konopleva M
The introduction of venetoclax has revolutionized the treatment landscape of acute myeloid leukemia, offering new therapeutic opportunities. However, the clinical response to venetoclax varies significantly between patients, with many experiencing limited duration of response.
Rivaroxaban plus aspirin after lower-extremity revascularization
McClure GR and Eikelboom J
Patients undergoing revascularization of the lower extremities have unacceptably high rates of major adverse cardiac and limb events despite the routine use of antiplatelet therapy. Optimization of antithrombotic therapy provides an opportunity to reduce this risk. Recent large, randomized trials have demonstrated substantial benefit from the combination of low-dose rivaroxaban and aspirin compared with aspirin alone. Despite this new evidence, uptake remains limited.
The impact of oral health promotion on the quality of life of children with bleeding disorders: fighting misconceptions
El Sherif NHK, Wassel MOM, Mostafa S, Abd El Maged FRR, Nathan SE, Hamdy D and Ebeid FSE
Understanding the disease-specific risks and patient-related barriers of children with bleeding disorders is necessary for primary oral health promotion. Our goal was to assess the oral health status and the impact of oral health promotion among patients with bleeding disorders.
Safety and efficacy of danicopan in patients with paroxysmal nocturnal hemoglobinuria: a systematic review and meta-analysis
Muvaffak E, Mokresh ME, Varda A, Lakmoush M and Ilter MK
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease characterized by complement-mediated intravascular hemolysis (IVH). Current treatments like Eculizumab and Ravulizumab have limitations, with many patients still requiring transfusions. This study aimed to investigate the safety and efficacy of the new emergent treatment called Danicopan.
Effect of days of age at first blood transfusion on intraventricular hemorrhage in very low and extremely low birth weight infants
Qian Y, Huang J, Cheng H and Wang J
Preterm infants are a group cohort of transfusion recipients due to their low blood volume and underdeveloped hematopoietic system. The objective of this study was to probe the effect of days of age at first blood transfusion on intraventricular hemorrhage (IVH) in very low and extremely low birth weight VLBW and ELBW infants.
Hairy cell leukemia (HCL) and HCL-like disorders: present, emergent treatment options and future directions
Troussard X
Hairy cell leukemia accounts for less than 2% of leukemias. The hairy cells express CD11c, CD25, CD103, and CD123 markers. The mutation was detected in 95% of HCL cases. Patients achieve high complete response rate with purine analogues with or without rituximab, but relapses are inevitable. HCL-like disorders including HCL variant, splenic diffuse red pulp lymphoma, and splenic marginal zone lymphoma are BRAF negative. The CD25 expression is negative. The absence of BRAF mutation in HCL variant contrasts with the presence of mitogen-activated protein kinase kinase 1 () mutations in 50% of cases.
Etiological spectrum of pancytopenia in adults based on hematological parameters and bone marrow studies
Saleem U, Tariq H, Alam S, Zafar AF, Tanveer U and Khan MH
This study aimed to investigate the demographic, clinical, and diagnostic aspects of adult pancytopenia while exploring its etiological spectrum through hematological parameters and bone marrow studies.
Current and emerging therapies as potential treatment for people with von Willebrand disease
Favaloro EJ, Pasalic L and Curnow J
Von Willebrand disease (VWD) reflects the most common inherited bleeding disorder, arising from defects or deficiencies in the von Willebrand factor (VWF). VWD treatment mostly relies on the replacement of missing or defective VWF, but additional ('adjunct') therapies are useful in select patients/situations. Patients with VWD are often misdiagnosed and therefore non-optimally managed.
The role of interleukin-20 and vascular endothelial growth factor-A biomarkers in the detection of renal impairment in patients with multiple myeloma
Elsaid DS, Elbedewy TAE, Hamza MA and Haroun RA
Multiple myeloma (MM) is a malignant incurable disease characterized by monoclonal plasma cell increase associated with renal impairment. Evaluation of neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR), platelet-to-lymphocyte ratio (PLR), hemoglobin/red cell distribution width (HB/RDW), interleukin-20 (IL-20), and vascular endothelial growth factor-A (VEGFA) in patients with MM (with or without renal impairment) as prognostic and severity indicators.
Ibrutinib and venetoclax combination therapy for mantle cell lymphoma: are two better than one?
Li J and Tam CS
Mantle cell lymphoma (MCL) is a non-Hodgkin B-cell lymphoma typically regarded as incurable with standard chemotherapy. Ibrutinib has become an accepted second-line treatment for relapsed or refractory MCL. Although venetoclax has shown activity against the disease, these results have not been consistently seen in the post-ibrutinib era. Therefore, there is growing evidence that supports using ibrutinib and venetoclax together in patients with chronic lymphocytic leukemia.