Mast Cell Leukemia (MCL) is the rarest form of systemic mastocytosis, a rare group of neoplastic disease that result from clonal proliferation of mast cells and their accumulation in one or more organ systems. The diagnosis of MCL is made by meeting the World Health Organization (WHO) 2017 criteria. MCL is further subclassified as leukemic or aleukemic based on presence or absence of circulating mast cells in the peripheral blood and acute versus chronic based on presence or absence of findings indicative of impaired organ function due to mast cell infiltration.
Background: Gastric per oral endoscopic myotomy (GPOEM) was developed as a therapeutic option for gastroparesis after the pylorus was identified as a key target for gastroparesis management. This study includes a systematic literature review of studies in which GPOEM was utilized as therapy for gastroparesis. Methods: A literature search was conducted in three databases (MEDLINE, Scopus and Embase) of articles that included the keywords “GPOEM”, “Gastric per oral endoscopy myotomy” or “per oral pyloromyotomy” and “Gastroparesis” in the abstract or title. The search covered articles published until 29 February 2020. Results: A total of 139 articles were identified. Only 15 articles met the final inclusion criteria and were retrieved for qualitative data synthesis. Conclusion: GPOEM for gastroparesis is shown to have a high success rate among the studies but data interpretations are limited because of small sample sizes and short follow-up. Subjective and objective data prior to and post-GPOEM will be mandatory to establish credibility. The procedure is technically feasible and safe based on the limited studies available.
Background: Gastric electrical stimulation (GES) therapy is indicated for the treatment of drug-refractory gastroparesis (GP). However, the long-term effects of GES therapy on GP symptom control and identification of the optimal parameters to activate this long-term efficacy have not been investigated. Methods: We conducted a retrospective cohort analysis of 57 GP patients who received GES and pyloroplasty (PP). The interrogation of the GES system and assessment of GP symptoms were conducted at the initiation of GES therapy and during follow-up visits. We determined the changes in GES parameters including voltage (V), impedance (I), and current (C). The outcome was total symptom score (TSS), which was measured by self-reported GP symptoms. Results: The mean age of patients was 44 (±14) years, and 72% were females. The etiology for GP was diabetes mellitus in 72% and idiopathic in 28%. The median duration of GES follow-up was 47 months (range 5–73) A significant decrease was found in individual symptom scores and the TSS (−10.8; 95%CI: −12.6, −9.08) compared to baseline scores (p < 0.0001). During follow-up, readings for I (515 vs. 598 Ω), V (3.3 vs. 4.8 V), and C (6.5 vs. 8.4 mA) significantly increased (p ≤ 0.0001 for all parameters). Higher GES settings were associated with lower TSS in the adjusted analysis (RC, −1.97; 95%CI: −3.81, −0.12, p = 0.037). Conclusions: these findings suggest that adjusting GES parameters over time based on optimizing symptom improvement should be incorporated into the long-term care of patients receiving gastric neurostimulation therapy.