Background Thrombotic thrombocytopenic purpura (TTP) is a hematologic disease that can be fatal if not treated early. We aimed to describe the clinical characteristics of Mexican patients with idiopathic TTP. Study design and methods We conducted a retrospective study, including all adult patients diagnosed with idiopathic TTP from 2011 to 2017 in two Mexican centers. We further compared our results with the published literature. Results Twenty patients were included; 70% were female, with a median age of 38.5 years at diagnosis (range 16‐63). The median time from onset of symptoms to hospital admission was 1.5 days (range 0‐16). Most patients (85%) presented with at least one systemic manifestation at admission (including fever) and 90% had neurological symptoms, most of them major (70%) including loss of consciousness, transient focal abnormalities, headache, and confusion. Only one patient (5%) had the classical pentad at the time of admission. Kidney failure was present in 25% of patients and hemorrhagic symptoms in 60%. Digestive and cardiorespiratory symptoms were less common (45% and 15%, respectively). Median platelet count and lactate dehydrogenase were 10 500/μL and 1319 IU/L, respectively. Eighty percent of patients achieved remission following treatment. Patients admitted within the first 48 hours (after the onset of symptoms) tended to have better overall survival. Conclusion Clinical presentation in Mexican TTP patients is similar to that in other countries. Early admission and a high suspicion for the disease will avoid delays in the initial work‐up and initiation of therapy, further improving prognosis.
Angiosarcomas are neoplasms of blood or lymphatic vessels with aggressive behavior. We report the coexistence of this malignancy within soft tissue of the breast in a 49-year-old woman who was diagnosed with Klippel-Trenaunay-Weber syndrome (KTW-S) during childhood. The patient has no previous history of radiation therapy on the chest and does not have any known risk factor for developing angiosarcoma, except for her congenital disease. To the best of our knowledge, the association between soft tissue angiosarcoma of the breast and KTW-S has never been previously reported.
Highlights d Doublets and multiplets in single-cell RNA-seq confound biological results d Single-cell VDJ-seq and CITE-seq can aid the identification of doublets/multiplets d Machine learning can identify further heterotypic and homotypic doublets/multiplets
PURPOSE: Establishing research capacity in low- and middle-income countries (LMICs) is key for improving the outcomes of patients with hematologic diseases globally. Few studies have analyzed the contributions of LMICs to global hematology. The American Society of Hematology Meeting (ASH) is the largest international academic event where peer-reviewed contributions in our field are presented. METHODS: In this cross-sectional analysis, all abstracts accepted to ASH 2018 selected for a poster or oral presentation were reviewed. Those that had a contributing author from an LMIC were identified. The proportion of LMIC abstracts across categories was analyzed. Country of origin, high-income country participation, the presence of a conflict of interest (COI), and sponsorship were determined. RESULTS: From 4,871 abstracts reviewed, 506 had a contributing author from an LMIC (10.4%), with 277 (54.7%) contributions in partnership with a high-income country. LMIC-independent contributions corresponded to 19 of 1,026 oral abstracts (1.9%) and 209 of 3,845 posters (5.4%). Most abstracts from LMICs were clinical (n = 311; 61.5%) and multicentric in nature (n = 353; 69.8%). COI statements with the pharmaceutical industry were common (n = 214; 42.3%). Collaboration between LMICs was infrequent (n = 33; 6.5%). Upper-middle–income countries had 466 participations (81.5%), in comparison with 96 (16.8%) in low-middle–income and 10 (1.7%) in low-income countries. CONCLUSION: LMICs were responsible for a small fraction of abstracts at ASH18; low-income countries were practically absent. Almost half of accepted works represented a form of international collaboration, with clinical, multicenter studies predominating and COI disclosures a frequent and unexpected feature, reflecting the instrumental nature of LMIC participation and a lack of independent, robust, locally developed hematology research.
Background. Establishing research capacity in low and middle-income countries (LMIC) is key for improving health systems and implementing actionable programs through evidence-based assessments. Few studies have analyzed hematology research capacity in LMICs. The American Society of Hematology (ASH) annual meeting is the largest hematology event where peer-reviewed contributions from researchers worldwide are selected for presentation based on scientific merit. Therefore, it can provide a useful snapshot of the current status of hematology research in a single point in time. For this reason, we analyzed abstracts presented at the 2018 ASH annual meeting (ASH18) with a focus on those from authors working in a LMIC. Objective. To describe the proportion of abstracts presented at ASH18 from an LMIC and analyze their characteristics as a surrogate for academic contributions to global hematology. Methods. We reviewed all abstracts presented at ASH18 in an oral presentation or poster form published online in the supplemental edition of Blood 2018;132 (Suppl 1). LMICs were selected according to the World Bank classification including countries or territories with a gross national income <$12,056 USD per capita. We described all abstracts that had a co-author with an affiliation from an institution in a LMIC, regardless of their position. We categorized studies as clinical vs. basic and single vs. multicenter nature. We also identified the presence of conflict-of interest statements (COI) and identifiable industry sponsors. We compared abstracts that had co-authors from high-income countries (HIC+LMIC) vs. those from LMIC countries alone. Comparison across groups was performed using chi-square and Fisher's exact test. Results. A total of 4,871 abstracts presented at ASH 2018, with 1,026 oral presentations and 3,845 posters were available online. Among them, 510 abstracts (10.5%) had a contributing author from an institution in an LMIC, corresponding to 92 (9%) of all oral presentations and 418 (10.9%) of all posters (Figure 1). LMIC-only contributions represented 4.7% of all abstracts (n=229). The most common LMIC of origin for LMIC-only contributions was China with 133 (58.1%) (Figure 1). Most abstracts were clinical and multicentric in nature (62 and 70.2%, respectively), and in 42.5% of them a COI was reported. Clinical trials reflected 19% of all LMIC contributions. In 31.9% of cases the first author was affiliated to an institution in a HIC. Mixed LMIC/HIC contributions had significantly more COIs and industry sponsors than those from LMIC-only institutions (Table 1). When comparing between oral vs poster LMIC presentations, works selected for an oral presentation were significantly more clinical and multicentric, had a higher proportion of clinical trials, more COIs and identified industry sponsors (Table 2). Conclusions. LMICs, where more than 80% of the world population resides, were responsible for only a small fraction of contributions to ASH18, half of them representing a form of international collaboration, with a high number of COI disclosures. Disclosures Gomez-Almaguer: Amgen: Consultancy, Speakers Bureau; Janssen: Consultancy, Speakers Bureau; Teva: Consultancy, Speakers Bureau; Takeda: Consultancy, Speakers Bureau; Celgene: Consultancy, Speakers Bureau.
Despite the anti-tumor effects of CD8+ T-cells, chronic exposure to tumor antigens causes T-cell exhaustion, dampening tumor growth control. A subset of exhausted T-cells with stem-like properties ( SL T-cells) has been identified in tumor samples, characterized by the expression of inhibitory molecules (PD-1, CTLA-4), the transcription factor TCF1, and reduced cytotoxic effects. After immune checkpoint inhibition (ICI), SL T-cells rapidly proliferate and differentiate into terminally differentiated/exhausted T-cells ( TD T-cells) with increased effector mechanisms, associating with favorable cancer outcomes patients. Moreover, various epigenetic mechanisms have been implicated in the development of exhausted T-cells, in which the transcription factor TOX has a crucial role. Getting a better understanding of T-cell exhaustion is essential to develop novel prognostic and therapeutic strategies that improve clinical responses to cancer immunotherapies. This review focuses on T-cell exhaustion: features, development, and therapeutic strategies to overcome this dysfunctional T-cell condition in cancer.
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