BackgroundThe number of adolescents with chronic health conditions (CHCs) continues to increase. Medication nonadherence is a global challenge among adolescents across chronic conditions and is associated with poor health outcomes. While there has been growing interest in the use of mHealth technology to improve medication adherence among adolescents with CHCs, particularly text messaging and mobile phone apps, there has been no prior systematic review of their efficacy.ObjectiveThe purpose of this review was to systematically evaluate the most recent evidence for the efficacy of text messaging and mobile phone apps as interventions to promote medication adherence among adolescents with CHCs.MethodsPubMed, Embase, CENTRAL, PsycINFO, Web of Science, Google Scholar, and additional databases were searched from 1995 until November 2015. An additional hand search of related themes in the Journal of Medical Internet Research was also conducted. The Preferred Reporting Results of Systematic Reviews and Meta-Analyses guidelines were followed. Two reviewers independently screened titles/abstracts, assessed full-text articles, extracted data from included articles, and assessed their quality using Grades of Recommendation, Assessment, Development, and Evaluation criteria. Included studies were described in original research articles that targeted adherence in adolescents with CHCs (12-24 years-old).ResultsOf the 1423 records examined, 15 met predefined criteria: text messaging (n=12) and mobile phone apps (n=3). Most studies were performed in the United States (11/15, 73%), were randomized-controlled trials (8/15, 53%), had a sample size <50 (11/15, 73%), and included adherence self-report and/or biomarkers (9/15, 60%). Only four studies were designed based on a theoretical framework. Approaches for text messaging and mobile phone app interventions varied across studies. Seven articles (7/15, 47%) reported significant improvement in adherence with moderate to large standardized mean differences. Most of the included studies were of low or moderate quality. Studies varied in sample size, methods of adherence assessment, and definition of adherence, which prohibited performing a meta-analysis.ConclusionsThe use of text messaging and mobile phone app interventions to improve medication adherence among adolescents with CHCs has shown promising feasibility and acceptability, and there is modest evidence to support the efficacy of these interventions. Further evaluation of short- and long-term efficacy and cost-effectiveness of these interventions is warranted given the early and evolving state of the science.

We describe a 4-year-old female with pre-B-cell acute lymphoblastic leukemia on maintenance chemotherapy, who developed hemophagocytic lymphohistiocytosis (HLH) secondary to Epstein-Barr virus (EBV) infection, complicated by an aggressive lymphoproliferative disorder. Although there was no history of bone marrow transplant or underlying immunodeficiency, EBV triggered a post-transplant lymphoproliferative disease (PTLD)-like lymphoma. Multiple regimens of chemotherapy failed to induce remission and patient developed multiorgan failure. The association of HLH with EBV-related PTLD-like lymphoproliferative disorder is rare. We present this case to highlight this unusual association so that this highly fatal disease can be recognized and promptly addressed.

Gene rearrangements involving the histone lysine methyltransferase 2A gene (KMT2A) are identified in 4-7% of childhood acute lymphoblastic leukaemias (ALL). 1 The rearrangement is associated with a poor prognosis, presentation in infancy and lymphoblasts lacking CD10 with expression of myeloid markers. 2-4 KMT2A fusions are promiscuous with 135 different transfusion partner genes (TPGs) reported. 1 The vast majority fuse the 5 0 end of KMT2A in-frame to a TPG at the 3 0 end. 1 However, reciprocal translocations (TPG-KMT2A) are also found either in association with KMT2A-TPG fusions or rarely, in some instances, by themselves without a corresponding KMT2A-TPG fusion. 1 Here, we describe an isolated ATP5L-KMT2A reciprocal fusion in a 15-month-old male with B lymphoblastic leukaemia/lymphoma (B-ALL). The University Hospitals Cleveland Medical Center/Case Western Reserve University's Institutional Review Board granted approval for the study under number #02-14-36. The patient presented at 14 months in Kuwait with refusal to walk, malaise and fevers. A complete blood count demonstrated: white blood cells 22Á4 9 10 9 /l, haemoglobin 75 g/l, platelets 24 9 10 9 /l. A bone marrow work-up revealed 91Á5% blasts. Phenotypically, the cells were CD10 À , CD19 + , CD20 À , CD34 + , CD45 dim , TdT + (partial) and MPOconsistent with BALL. Genetic studies showed a normal karyotype and fluorescent in situ hybridization (FISH) was negative for t(12;21) (p13;q22), t(9;22)(q34;q11), t(1;19)(q23;p13Á3) and KMT2A (11q23) rearrangements. The patient was classified as standard risk BALL and received three-drug induction therapy per UK-MRC-ALL 2011. On day 15 of induction, persistent blasts (66%) were present and treatment was intensified to include anthracyclines and an additional dose of PEG asparaginase. He was transferred to our institution after completing induction therapy. Persistent disease was noted upon arrival at our institution with 48% marrow blasts with a phenotype as previously reported (Fig 1A, B). Repeat FISH for KMT2A rearrangement was negative with two yellow signals suggesting two intact copes of the KMT2A gene (Fig 1C). To further evaluate for a molecular aberration, cells were assessed for novel gene fusions using a NeoGenomics (Fort Myers, FL, USA) ALL fusion profile which identified an ATP5L-KMT2A fusion (Fig 2), discussed below. He received consolidation therapy for very high-risk BALL patients per COG AALL1131 followed by two cycles of blinatumomab. Subsequently he underwent a matched, unrelated umbilical cord transplant and presently is seven months post-transplant and disease-free. ized to exons 9-11 of the KMT2A gene 1. Only 6Á5% of KMT2A fusions occur with breakpoints outside of this Correspondence