Citations

Survival outcomes for older adults with acute lymphoblastic leukemia (ALL) are poor and optimal management is challenging due to higher-risk leukemia genetics, comorbidities, and lower tolerance to intensive therapy. A critical understanding of these factors guides the selection of frontline therapies and subsequent treatment strategies. In addition, there have been recent developments in minimal/measurable residual disease (MRD) testing and blinatumomab use in the context of MRD-positive disease after therapy. These NCCN Guidelines Insights discuss recent updates to the NCCN Guidelines for ALL regarding upfront therapy in older adults and MRD monitoring/testing in response to ALL treatment.

Massage therapy is utilized in hospitals for patients experiencing pain, anxiety, sleeplessness, among other symptoms. Postsurgical pain is one of the most common reasons massage therapy is ordered. We present a case of a 45-year-old woman who underwent ventral hernia repair. Postoperative day 2, she began to experience multiple symptoms, including double-vision, left-sided facial numbness, tremors, pain, and weakness in her left fingers, arm, and leg. A static magnetic resonance image of the brain and cervical spine were obtained, which revealed disc protrusions at C3/C4, C5/C6, C6/C7, and mild deformity of the spinal cord. The patient’s pain was difficult to control and she was unable to be weaned from intravenous pain medication. Massage therapy was ordered on postoperative days 6 and 7. During both massage sessions, as upper neck muscle tension was reduced, the therapist and patient observed several audible “pops” of the cervical spine with immediate relief of symptoms. In this particular case, massage therapy, though requested to address pain, had a secondary benefit in relieving her diplopia, left-sided facial numbness, tremors, and weakness. Although the mechanism of action is not clear, this case highlights the significant secondary beneficial effects that often occur with massage therapy.

Background and objectives: Loeys–Dietz syndrome 3, also known as aneurysms-–osteoarthritis syndrome, is an autosomal dominant genetic connective tissue disease caused by pathogenic variants in SMAD3, a transcription factor involved in TGF-β signaling. This disorder is characterized by early-onset osteoarthritis and arterial aneurysms. Common features include scoliosis, uvula abnormalities, striae, and velvety skin. Materials and Methods: The pathogenicity of a variant of uncertain significance in the SMAD3 gene was evaluated (variant c.220C > T) through personalized protein informatics and molecular studies. Results: The case of a 44-year-old male, who was originally presumed to have Marfan syndrome, is presented. An expanded gene panel determined the probable cause to be a variant in SMAD3, c.220C > T (p.R74W). His case was complicated by a history of stroke, but his phenotype was otherwise characteristic for Loeys–Dietz syndrome 3. Conclusion: This case emphasizes the importance of comprehensive genetic testing to evaluate patients for connective tissue disorders, as well as the potential benefit of utilizing a protein informatics platform for the assessment of variant pathogenicity.

Abstract Purpose: Myelofibrosis is characterized by bone marrow fibrosis, atypical megakaryocytes, splenomegaly, constitutional symptoms, thrombotic and hemorrhagic complications, and a risk of evolution to acute leukemia. The JAK kinase inhibitor ruxolitinib provides therapeutic benefit, but the effects are limited. The purpose of this study was to determine whether targeting AURKA, which has been shown to increase maturation of atypical megakaryocytes, has potential benefit for patients with myelofibrosis. Patients and Methods: Twenty-four patients with myelofibrosis were enrolled in a phase I study at three centers. The objective of the study was to evaluate the safety and preliminary efficacy of alisertib. Correlative studies involved assessment of the effect of alisertib on the megakaryocyte lineage, allele burden, and fibrosis. Results: In addition to being well tolerated, alisertib reduced splenomegaly and symptom burden in 29% and 32% of patients, respectively, despite not consistently reducing the degree of inflammatory cytokines. Moreover, alisertib normalized megakaryocytes and reduced fibrosis in 5 of 7 patients for whom sequential marrows were available. Alisertib also decreased the mutant allele burden in a subset of patients. Conclusions: Given the limitations of ruxolitinib, novel therapies are needed for myelofibrosis. In this study, alisertib provided clinical benefit and exhibited the expected on-target effect on the megakaryocyte lineage, resulting in normalization of these cells and reduced fibrosis in the majority of patients for which sequential marrows were available. Thus, AURKA inhibition should be further developed as a therapeutic option in myelofibrosis. See related commentary by Piszczatowski and Steidl, p. 4868

Objective To assess whether the Berlin Sleep Questionnaire and the Snoring, Tired, Observed, Pressure, Body mass, Age, Neck, and Gender questionnaire (STOP-BANG) might be suitable replacements for an overnight sleep pulse oximetry as screening tools for sleep disordered breathing in patients with fibromyalgia. Participants: From June 8, 2018 through July 25, 2018, adult patients with a confirmed diagnosis of fibromyalgia (via the 1990 and/or 2010 American College of Rheumatology Fibromyalgia Classification Criteria) who attended Mayo Clinic’s Fibromyalgia Treatment Program were invited to participate in the study. Methods: The design was a prospective comparative study with a retrospective chart review component. Participants completed 2 validated surveys: the Berlin Sleep Questionnaire and the STOP-BANG. Medical records were reviewed for demographic information and overnight pulse oximetry test results. Results: Results from both questionnaires indicate that there is an association between sleep apnea risk category (defined by questionnaire) and oximetry results. Fisher’s exact test for STOP-BANG and Berlin Sleep Questionnaire are statistically significant ( P < .001), indicating that participants at high risk for sleep apnea (based on the questionnaires) had a greater prevalence of abnormal oximetry results than those at low risk for sleep apnea. Participants who were classified as high risk (85.7%) or intermediate risk (61.5%) on the STOP-BANG questionnaire for sleep apnea had abnormal oximetry results. Participants who scored as high risk (85.7%) for sleep apnea on the Berlin Sleep Questionnaire had abnormal oximetry results. Conclusions: In patients with fibromyalgia, the Berlin Sleep Questionnaire and the STOP-BANG questionnaires could be beneficial in determining the probability of obstructive sleep apnea and the subsequent need for pulse oximetry testing, in higher risk patients.