Awni Alshurafa

Polycythemia vera is a chronic myeloproliferative neoplasm in which erythrocytosis, Janus kinase–signal transducer and activator of transcription activation, inflammation, endothelial dysfunction, and abnormal blood–cell activation converge to increase cardiovascular risk. This narrative review examines how electrocardiography, echocardiography, coronary angiography, cardiac magnetic resonance imaging, and right-heart catheterization contribute to recognition and characterization of cardiac involvement in polycythemia vera. Reported abnormalities include QT and P-wave changes, atrial arrhythmias, acute coronary syndromes, subclinical biventricular dysfunction, pulmonary hypertension, intracardiac thrombosis, and hypertrophic remodeling. Large observational datasets in myeloproliferative neoplasms also exhibit clinically important burdens of myocardial infarction, heart-failure hospitalization, bleeding, and echocardiographic pulmonary hypertension, although polycythemia vera–specific estimates remain limited. Baseline cardiovascular testing is increasingly relevant before novel therapies and trial enrollment; however, an abnormal test should prompt confirmation, reversible-cause assessment, and protocol-specific review rather than be treated as a universal contraindication. Hematocrit control and low-dose aspirin reduce thrombotic events, while evidence that cytoreductive therapy reverses electrocardiography or imaging abnormalities remains sparse. A symptom- and risk-directed diagnostic approach, integrated with hematology and cardiology assessment, is therefore more defensible than routine universal cardiac screening.

Sickle cell leg ulcers (SCLUs) are a chronic and debilitating complication of sickle cell disease (SCD), often associated with severe pain, impaired mobility, delayed healing, and recurrence. Their management remains challenging because ulcer formation and persistence are driven by multiple overlapping mechanisms, including hemolysis‐associated vasculopathy, microvascular dysfunction, chronic inflammation, venous disease, and local tissue hypoxia. Despite standard measures such as local wound care, compression therapy, infection control, analgesia, and optimization of SCD‐directed treatment, many ulcers remain refractory, and high‐quality evidence guiding treatment is limited. We report a 44‐year‐old man with homozygous SCD (HbSS) and chronic venous disease who developed a painful refractory medial malleolar ulcer that failed to improve with conventional management. Adjunctive hyperbaric oxygen therapy (HBOT) was administered alongside standard wound care and was well tolerated, with no treatment‐related adverse events. Two months after completion of therapy, the ulcer showed near‐complete healing, accompanied by complete pain resolution and restoration of normal ambulation. This case provides additional clinical evidence that HBOT may be feasible and potentially useful in selected patients with refractory SCLUs; however, causality cannot be established from a single case, and prospective evaluation is required.

Background: Myeloid neoplasms harboring both an isolated deletion of chromosome 5q (del(5q)) and myeloproliferative neoplasm (MPN) driver mutations ( JAK2, CALR, MPL ) constitute a rare and diagnostically challenging subset, often described with features overlapping those of myelodysplastic syndromes (MDS) and MPN. Methods: We systematically reviewed published case reports and case series of adult patients with myeloid neoplasms and concomitant isolated del(5q) and MPN driver mutations. A comprehensive search of PubMed and Google Scholar from inception through 31 December 2024 was performed using terms related to “del(5q)”, “ JAK2”, “CALR”, “MPL” , and “myeloid neoplasm”. Eligible reports required documentation of isolated del(5q) and at least one MPN driver mutation with extractable clinical, cytogenetic, and/or molecular data. Data were synthesised descriptively; no quantitative meta-analysis was feasible. The review followed PRISMA 2020 guidelines and was registered post-study at INPLASY2025120046. Results: Twenty publications reporting 24 patients (diagnosed between 2006 and 2021) met the inclusion criteria. The median age was 70.5 years with a female predominance. Most patients presented with macrocytic anemia, thrombocytosis, and megakaryocytic dysplasia, frequently accompanied by marrow fibrosis. JAK2 V617F was the predominant mutation, whereas CALR and MPL were rarely described. Lenalidomide achieved hematologic responses in 14 of 17 evaluable patients and cytogenetic responses in 8 of 16; JAK2 V617F clearance occurred in 4 of 13. During a median follow-up of approximately two years, 5 of 24 patients progressed to acute myeloid leukemia. Conclusion: Reported cases with isolated del(5q) and an MPN driver mutation suggest a rare overlap presentation with mixed dysplastic and proliferative features. Lenalidomide appears to provide hematologic and cytogenetic benefit in some patients, although molecular persistence and progression to AML have been observed. Given the limited number and heterogeneity of published cases, these observations should be interpreted with caution. Larger, systematically collected datasets are needed to better understand the clinical significance and optimal management of this combination.

Allogeneic hematopoietic stem cell transplantation (allo‐HSCT) remains the only potentially curative therapy for many patients with high‐risk myeloid neoplasms, yet relapse and transplant‐related toxicity continue to limit durable benefit. Venetoclax (VEN), a selective BCL‐2 inhibitor, provides a biologically compelling strategy for conditioning augmentation through chemotherapy sensitization and potential lowering of host immune resistance to engraftment. We performed a hybrid scoping review and evidence‐mapping analysis to summarize published clinical experience and the active investigational landscape of VEN‐containing conditioning or sequential regimens prior to allo‐HSCT. Eighteen studies (20 reports) met the inclusion criteria, including nine published cohorts comprising 238 patients and 11 ongoing clinical trials. Reported platforms were heterogeneous, spanning reduced‐intensity, myeloablative, and sequential approaches. Engraftment and graft‐versus‐host disease outcomes were generally comparable to expected benchmarks, although available data remain preliminary and non‐randomized, underscoring that the primary aim of this scoping review is to map existing evidence and ongoing trials rather than to inform clinical practice or guideline recommendations.

Introduction: Tyrosine kinase inhibitors (TKIs) are the cornerstone of therapy for chronic myeloid leukemia (CML). Although nephrotoxicity has been reported, existing evidence remains inconsistent, with limited data from the Gulf region. This study aimed to evaluate the impact of TKIs on renal function among patients with CML treated at Hamad Medical Corporation (HMC), Qatar. Methods: This retrospective, single-center study included adults with CML who received a TKI between 2016 and 2023. The primary outcome was the change in estimated glomerular filtration rate (eGFR) over time. Secondary outcomes included acute kidney injury (AKI), chronic kidney disease (CKD) and the need for dialysis. Repeated-measures analysis of variance was used to assess unadjusted eGFR trends. Linear mixed-effects modeling was used to assess longitudinal eGFR trends while adjusting for baseline comorbidities. Results: A total of 209 patients were followed for a median of 69.3 months (IQR, 39.4–117.8). Imatinib was the most frequently prescribed TKI (n = 136, 65.1%), followed by dasatinib (n = 39, 18.7%) and nilotinib (n = 34, 16.3%). The mean baseline eGFR was 93.8 mL/min/1.73 m2 (SD 26.3) and showed no significant decline at 3 months (p = 0.498), 3 years (p = 0.112), or 8 years (p = 0.297). However, among dasatinib-treated patients, eGFR significantly decreased from 87.5 (SD 16.7) to 76.8 (SD 11.4) over 3 years (p = 0.042). Multivariate mixed-effects modeling revealed that this reduction was confounded by hypertension (p < 0.001). After adjusting for comorbidities, no significant difference in eGFR decline was observed between the TKI groups. AKI occurred in 17 (8.1%) patients, CKD in 4 (1.9%), and none required dialysis. No significant differences were observed across TKI types. Conclusion: Renal function remained largely stable during long-term TKI therapy. Initial declines to dasatinib were largely comorbidity-driven, pointing to the need for cardiovascular risk assessment in this population.

Assessing a patient with a suspected bleeding disorder is a complex aspect of hematology. Prolonged activated partial thromboplastin time often indicates a coagulation factor deficiency or inhibitor and warrants detailed evaluation. Factor XI deficiency, a rare autosomal recessive disorder, shows variable bleeding tendencies with poor correlation between factor levels and bleeding risk. We report a 24-year-old asymptomatic male with an incidentally prolonged activated partial thromboplastin time during preoperative assessment for septoplasty. He had no bleeding or family history suggestive of a bleeding disorder. Mixing studies showed full correction, and factor assays confirmed severe Factor XI deficiency. Given the uncertain bleeding phenotype, a tailored perioperative plan was implemented, including fresh frozen plasma and prophylactic tranexamic acid. Recombinant activated Factor VII was available as rescue therapy, but not required. The surgery and postoperative course were uneventful, highlighting the importance of individualized perioperative management in Factor XI deficiency.

Myeloproliferative neoplasms (MPNs) are chronic hematologic disorders characterized by clonal proliferation of myeloid lineage cells, commonly driven by JAK2 mutations, and associated with significant thrombotic and hemorrhagic complications. Women of reproductive age with MPNs face unique challenges in selecting safe and effective contraception, as these disorders inherently increase thrombotic risk and may interact with hormonal contraceptive therapies. Estrogen‐containing contraceptives are contraindicated due to their association with venous and arterial thrombosis, while progestin‐only and non‐hormonal methods are generally safer but must be tailored to individual risks such as thrombocytopenia, prior thrombotic events, and disease‐modifying therapies. This review provides a comprehensive analysis of contraceptive options for women with MPNs, exploring the thrombotic and bleeding risks associated with different methods and the potential impact of MPN treatments, including JAK2 inhibitors, on contraceptive efficacy. By synthesizing current evidence, the review aims to guide clinicians in optimizing reproductive health for women with MPNs while minimizing the risks of their underlying hematologic condition.

Background: Renal adverse drug reactions (ADRs) associated with tyrosine kinase inhibitors (TKIs) in the treatment of chronic myeloid leukemia (CML) are relatively rare, and there is currently no standardized protocol for their management. Therefore, this study aimed to summarize renal ADRs related to TKIs use in CML and propose an evidence-based approach to monitor and manage these ADRs. Methods: A systematic literature review was performed to identify renal ADRs associated with TKIs in CML. Two authors screened the search results and extracted data from 37 eligible studies. These findings were then used to develop a scheme for clinicians to monitor and manage these ADRs. Results: Overall, imatinib seemed to be significantly linked to renal adverse events compared to other TKIs, and switching to dasatinib or nilotinib significantly improved renal function. Similar events were reported with bosutinib, although they were not statistically significant. However, most of the renal events reported on dasatinib were described as nephrotic syndrome that resolved with switching to imatinib. Few cases were reported with nilotinib that described tumor lysis syndrome (TLS)-related kidney injury. Conclusions: Recommendations include monitoring for progressive decline in the estimated glomerular filtration rate with imatinib, nephrotic syndrome with dasatinib, and TLS with nilotinib. Additionally, holding the offending TKI and managing renal ADRs according to local guidelines were adopted more frequently than reducing the TKI dose.

Introduction: Adolescents and young adults (AYAs) diagnosed with chronic myeloid leukemia (CML) constitute a significant demographic group, particularly in regions with youthful populations like Qatar. Despite the global median age of CML diagnosis being 65 years, Qatar’s age distribution reflects a younger cohort. This study investigates whether AYAs with CML exhibit distinct clinicopathological characteristics or outcomes compared to older age groups. Methods: A total of 224 CML patients were enrolled, including 114 AYAs (defined as ages 15 through 39). Demographic and clinical parameters, including gender, BMI, BCR-ABL1 transcript type, white blood cell (WBC) count, hemoglobin level, platelet count, and spleen size, were compared between AYAs and older patients. Prognostic scoring systems (Sokal, Hasford, EUTOS, and ELTS) and molecular response rates (MMR and DMR) were also evaluated. Results: AYAs demonstrated higher WBC counts at diagnosis (median 142.3 vs. 120; p = 0.037) and lower hemoglobin levels (10.5 vs. 11.40; p = 0.004) compared to older patients. Spleen size was significantly larger in AYAs (18.8 vs. 15.5; p = 0.001). While AYAs showed better prognostic scores by Sokal and Hasford criteria, EUTOS and ELTS scores indicated comparable risk stratification. However, AYAs exhibited lower rates of MMR (56.7 vs. 73.4%; p = 0.016) and achieved MMR at a slower pace (median time 130 vs. 103 months; p = 0.064). Similarly, the percentage of DMR was lower in AYAs (37.1 vs. 46.8%; p = 0.175). Conclusion: Despite their younger age, AYAs with CML displayed poorer prognoses compared to older patients. These findings underscore the importance of tailored management strategies for AYAs with CML to optimize outcomes in this distinct patient population. Key Point: AYAs are underrepresented in CML studies and risk scores, so this is the focus of this study.

Invasive Fusariosis in a Patient with Refractory Acute Myeloid Leukemia on Posaconazole ProphylaxisDr. Loai Abdullah1, Dr. Nada Soliman1, Dr. Ehab Adam1, Dr. Awni Alshurafa2, Dr. AbdulRahman Almashdali2,Dr. Mahir Petkar3, Dr. Deena Sideeg Mudawi2, Dr. Mohamed A Yassin2.Internal medicine department, Hamad medical corporation, Doha, QatarHematology department, Hamad medical corporation, Doha, QatarPathology department, Hamad medical corporation, Doha, Qatar