Publications by authors named "Ahmad Al-Huniti"

5 Publications

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Inherited Platelet Disorders: Diagnosis and Management.

Transfus Med Rev 2020 10 19;34(4):277-285. Epub 2020 Sep 19.

Division of Haematology/Oncology, Hospital for Sick Children, Toronto, ON, Canada; Cell Biology Program, Research Institute, Hospital for Sick Children, Toronto, ON, Canada; Departments of Paediatrics and Biochemistry, University of Toronto, Toronto, ON, Canada. Electronic address:

Inherited platelet disorders are rare but they can have considerable clinical impacts, and studies of their causes have advanced understanding of platelet formation and function. Effective hemostasis requires adequate circulating numbers of functional platelets. Quantitative, qualitative and combined platelet disorders with a bleeding phenotype have been linked to defects in platelet cytoskeletal elements, cell surface receptors, signal transduction pathways, secretory granules and other aspects. Inherited platelet disorders have variable clinical presentations, and diagnosis and management is often challenging. Evaluation begins with detailed patient and family histories, including a bleeding score. The physical exam identifies potential syndromic features of inherited platelet disorders and rules out other causes. Laboratory investigations include a complete blood count, blood film, coagulation testing and Von Willebrand factor assessment. A suspected platelet function disorder is further assessed by platelet aggregation, flow cytometry, platelet dense granule release and/or content, and genetic testing. The management of platelet function disorders aims to minimize the risk of bleeding and achieve adequate hemostasis when needed. Although not universal, platelet transfusion remains a crucial component in the management of many inherited platelet disorders.
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http://dx.doi.org/10.1016/j.tmrv.2020.09.006DOI Listing
October 2020

Up-front Treatment With Romiplostim in Children With Acquired Bone Marrow Failure: A Single Institutional Pediatric Case Series.

J Pediatr Hematol Oncol 2021 04;43(3):e431-e435

Stead Family Department of Pediatrics.

Background: Thrombopoietin receptor agonists are emerging as a therapeutic option for patients with aplastic anemia (AA) and myelodysplastic syndrome (MDS). We report our experience of treating children with AA/MDS with romiplostim, thrombopoietin receptor agonist.

Observations: Three children (AA, 2; MDS, 1) received romiplostim treatment at a median dose of 10 μg/kg/week (starting dose: 5 μg/kg/wk; 2.5 μg/kg/wk increment). Trilineage hematopoietic recovery occurred at a median of 13 weeks (range: 13 to 16 wk) without adverse events. Hematopoiesis continued to improve after therapy discontinuation (median follow-up: 2.8 y; range: 0.5 to 3.0).

Conclusion: Our experience supports the short-term safety and efficacy of romiplostim in children with AA/MDS.
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http://dx.doi.org/10.1097/MPH.0000000000001810DOI Listing
April 2021

Mental health disorders in haemophilia: Systematic literature review and meta-analysis.

Haemophilia 2020 May 19;26(3):431-442. Epub 2020 Apr 19.

Stead Family Department of Pediatrics, Carver College of Medicine, University of Iowa, Iowa City, IA, USA.

Aim: Despite significant advances in morbidity and mortality outcomes, quality of life for people with haemophilia (PWH) remains compromised. Underrecognized and undertreated mental health disorders decrease quality of life; however, reports are inconsistent regarding the true prevalence of mental health disorders in PWH.

Methods: We conducted a systematic literature search of Ovid MEDLINE, EMBASE, Psychinfo and the Cochrane Library, and hand searched the journal Haemophilia to identify records and subsequently conducted a meta-analysis to determine the prevalence of depression, anxiety and attention deficit hyperactivity disorder (ADHD) in patients with congenital haemophilia.

Results: Our search strategy identified 2315 records, and 28 studies met eligibility criteria. Meta-analysis demonstrated that PWH are at increased risk of depression (odds ratio (OR) 2.45; 95% confidence interval (CI) 1.64-3.68), anxiety (OR 1.74, 95% CI 1.01-3.00), anxiety/depression (OR 2.60, 95% CI 2.35-2.87) and ADHD (OR 3.48, 95% CI 1.74-6.96). We found considerable heterogeneity among the studies likely due to differences in assessment tools, populations studied and year of publication. This suggests that standardized tools to diagnose mental health disorders in PWH are needed. Additionally, high-quality studies investigating mental health disorders in PWH are necessary to adequately document the prevalence of these disorders.

Conclusion: Overall, our meta-analysis suggests that the prevalence of depression, anxiety and ADHD across decades is significantly increased in PWH compared to the general population.
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http://dx.doi.org/10.1111/hae.13960DOI Listing
May 2020

Discrepant Hemophilia A: An Underdiagnosed Disease Entity.

Am J Clin Pathol 2020 06;154(1):78-87

Department of Pathology and Laboratory Medicine, University of Iowa Carver College of Medicine, Iowa City.

Objectives: The term discrepant hemophilia A (DHA) denotes the discrepancy between factor VIII activity (FVIII:C) measured by different assay methodologies in patients with nonsevere hemophilia A (HA). The objective was to review the characteristics and the current understanding of mechanisms contributing to assay discrepancy in DHA.

Methods: Characteristics of the DHA patients treated were examined by retrospective chart review. In addition, a literature review was performed to determine the current understanding of DHA.

Results: Three cases of DHA were diagnosed based on bleeding phenotype: 2 cases represented missed diagnoses of HA, and 1 represented misclassification of hemophilia severity. The revised diagnosis and classification of hemophilia directly affected clinical management. Review of the literature identified 18 articles with an estimated pooled prevalence of 36% (95% CI, 23%-56%; I2 = 85%; P < .01) among nonsevere HA. Furthermore, literature indicated that DHA is a feature of how different FVIII gene mutations affect FVIII:C activity within different assay methodologies.

Conclusions: Our experience and literature review suggested that DHA is not only a laboratory phenomenon-it can affect clinical management in a subset of patients. A high index of suspicion for DHA is necessary while evaluating bleeding patients and/or classifying nonsevere HA.
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http://dx.doi.org/10.1093/ajcp/aqaa024DOI Listing
June 2020

Impact of the Survey of Inhibitors in Plasma-Product Exposed Toddlers (SIPPET) study and its post hoc analyses on clinical practice in the United States: A survey of Haemophilia and Thrombosis Research Society members.

Haemophilia 2019 Sep 2;25(5):764-772. Epub 2019 Jul 2.

University of Iowa Carver College of Medicine, Iowa City, Iowa.

Introduction: A recent randomized trial, the Survey of Inhibitors in Plasma-Product Exposed Toddlers (SIPPET), confirmed that exposure to recombinant FVIII (rFVIII) products doubled the risk of inhibitor development compared to plasma-derived FVIII (pdFVIII) in previously untreated (or minimally treated) patients (PUPs) with severe haemophilia A. SIPPET post hoc analyses showed that early exposure to rFVIII was more immunogenic and that rFVIII could harm low-risk PUPs with non-null mutations. Clinical implications of SIPPET findings for the haemophilia community were unclear.

Aim: Study the impact of the SIPPET study and its post hoc analyses on clinical practice for PUPs with severe haemophilia A in the United States.

Methods: Members of the North American Hemophilia and Thrombosis Research Society (HTRS) completed two online questionnaires related to SIPPET publications and PUP management (study period: 12/2016-8/2018).

Results: Over 50% participated the study. Sixty per cent expressed methodological concerns about the SIPPET study, yet 55% shared the study with new families. During the study period, rFVIII selection fell from 43/61 (70%) to 15/54 (28%) while use of pdFVIII and shared decision-making increased from 5/61 (8%) to 9/54 (17%) and from 4/61 (7%) to 10/54 (19%), respectively. Based on post hoc analyses, 44/54 (82%) would change their clinical practice with 31/44 (70%) using pdFVIII for PUPs. Barriers to translation of SIPPET analyses included study design concerns, non-inclusion of novel therapies, inability to perform genetic testing at diagnosis and risk of plasma-derived infections.

Conclusion: Despite the methodological concerns about the SIPPET study, this Grade I evidence appears to have influenced the clinical practice of haemophilia providers in the United States.
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http://dx.doi.org/10.1111/hae.13806DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7056002PMC
September 2019