Treatment-related acute myeloid leukaemia (t-AML) is a serious complication of topoisomerase 2 inhibitor therapy and is characterised by the presence of mixed lineage leukaemia (MLL) rearrangement. By molecular tracking, we were able to show that MLL cleavage preceded gene rearrangement by 3 months and before the clinical diagnosis of t-AML in a patient with haemophagocytic lymphohistiocytosis. This is the first report on the sequential detection of the two biomarkers in treatment-related leukaemogenesis.

Gene rearrangement

Hemophagocytic Lymphohistiocytosis

Cleavage (geology)

10.1038/sj.bjc.6602269

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Citations (4)

Supplementary Figures 1-3 from A Novel Kinase Inhibitor, INCB28060, Blocks c-MET–Dependent Signaling, Neoplastic Activities, and Cross-Talk with EGFR and HER-3

Xiangdong Liu Qian Wang Gengjie Yang Cindy Marando Holly K. Koblish

10.1158/1078-0432.22443834

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T-Replete Cord Blood Transplantation for High-Risk Acute Myeloid Leukemia in Children

Transplantation and Cellular Therapy (2022)

Ramya Nataraj Helen Campbell Denise Bonney Helena Lee Claire Donohue

Cord blood

10.1016/s2666-6367(22)00492-4

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Citations (1)

The effect of haemopoietic stem cell transplantation on the ocular phenotype in mucopolysaccharidosis type I (Hurler)

Acta Ophthalmologica (2017)

Ahmed Javed Tariq Aslam Simon Jones Jean Mercer Karen Tyler

Abstract Purpose To determine whether the ocular phenotype in patients with mucopolysaccharidosis type I (MPSI) Hurler is affected by the efficacy of previous haemopoietic stem cell transplantation (HSCT). Design A retrospective cohort study of patients with MPSI who had undergone treatment with HSCT. Methods Ocular phenotype was documented for each patient and compared to levels of biomarkers representing efficacy of previous transplantation. Main outcome measures: Assessment of visual acuity (VA), severity of corneal clouding and the presence of optic neuropathy or retinopathy. Biomarker assessment included dermatan sulphate/chondroitin sulphate (DS/CS) ratio and iduronidase enzyme level. Results Severe corneal clouding was significantly greater in patients with lower iduronidase levels (p = 0.023) and raised DS/CS ratio ( R 2 = 0.28 p = 0.043). Better VA was related to a higher iduronidase levels ( R 2 = 0.15, p = 0.004) and lower DS/CS ratio ( R 2 = 0.38, p = 0.001). Conclusion Improved ocular phenotypes in MPSI are associated with markers signifying efficacy of prior transplant. Early and effective HSCT may result in a better visual prognosis and reduction in ocular complications for patients with MPSI.

Mucopolysaccharidosis type I

Hurler syndrome

Mucopolysaccharidosis

Mucopolysaccharidosis I

10.1111/aos.13627

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Citations (5)

Dental Office Medical Emergencies: A Manual of Office Response Protocols

Ann Marie M.D. McMullin Timothy F. Meiller Robert Wynn

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Citations (1)

Improved Outcome From Invasive Adenovirus Infection in Pediatric Patients After Hemopoietic Stem Cell Transplantation Using Intensive Clinical Surveillance and Early Intervention

Journal of Pediatric Hematology/Oncology (2007)

Ponni Sivaprakasam Trevor F. Carr Mary Coussons Tasneem Khalid Andrew S. Bailey

Adenovirus is a common cause of morbidity and mortality after hemopoietic stem cell transplantation in children. Recently the incidence, risk factors, and outcome of such infections have been better defined using improved virologic detection methods, in particular polymerase chain reaction. We have introduced intensive virologic surveillance for adenovirus in our institution including at least weekly polymerase chain reaction testing of blood and stool samples. We report on 71 prospectively monitored transplants, including 40 from unrelated donors. In total, there were 8 cases of invasive adenovirus infection, 3 of whom died. Mortality was less than in previous studies as cases were managed with antiviral chemotherapy and reduction of immune suppression. In fatal cases, there was concurrent difficult graft versus host disease making withdrawal of immune suppression therapy impossible. We describe 2 cases of graft failure in association with adenovirus viremia and its treatment that were successfully managed with further donor cell infusion.

Viremia

Adenovirus infection

10.1097/mph.0b013e318030875e

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Citations (50)

Abnormal telomere shortening in leucocytes of children with Shwachman–Diamond syndrome

British Journal of Haematology (2002)

Ian Thornley Yigal Dror Lilian Sung Robert Wynn Melvin H. Freedman

Summary. Haemopoietic dysfunction, ranging from single‐lineage cytopenia to severe aplasia and/or myelodysplasia (MDS), is prominent in Shwachman–Diamond syndrome (SDS). To assess haemopoietic stem cell proliferation in SDS, we compared leucocyte telomere length in 12 patients with SDS to that of 41 controls, using an in‐gel hybridization technique. SDS patients had an age‐adjusted mean telomere length 1·4 kilobase pairs (kbp) shorter than controls ( P < 0·0001). Patients with'non‐severe′ SDS (one‐ or two‐lineage cytopenias; no MDS) had shortened telomeres (−1·4 kbp; P = 0·0004), as did those with ‘severe’ SDS. We conclude that stem cell hyperproliferation is a feature of SDS from its outset.

Cytopenia

Aplasia

Basophilia

10.1046/j.1365-2141.2002.03371.x

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Citations (51)

FDA new drug approvals in 2009.

General dentistry (2010)

Robert Wynn

Drug approval

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