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Journal-Based CME: T- and B-cell abnormalities, pneumocystis pneumonia and chronic lymphocytic leukemia associated with an AIOLOS defect in patients


Journal-Based CME: T- and B-cell abnormalities, pneumocystis pneumonia and chronic lymphocytic leukemia associated with an AIOLOS defect in patients Banner

  • Overview
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Add to Calendar Journal-Based CME: T- and B-cell abnormalities, pneumocystis pneumonia and chronic lymphocytic leukemia associated with an AIOLOS defect in patients 10/25/2021 10:00:00 AM 10/25/2024 10:00:00 AM America/New_York For More Details: https://mskcc.cloud-cme.com/course/courseoverview?P=0&EID=35582 Description: This Journal-Based CME activity consists of a full-text article that is free to read, a multiple-choice question test, and an evaluation/self-assessment. Learners are tested on their comprehension of the key concepts, with the objective of enriching their clinical practice and patient care through better understanding of scientific advances and new techniques in the fields covered by Journal of Exp... Online false MM/DD/YYYY


Date & Location
Monday, October 25, 2021, 10:00 AM - Friday, October 25, 2024, 10:00 AM

Specialties
Specialties - Allergy and Immunology, Bone Marrow Transplant, Cellular Therapeutics, Epidemiology, General and Family Practice, Genetics, Hematology, Infectious Diseases, Integrative Medicine, Interventional Radiology, Laboratory Medicine, Leukemia, Lymphoma, Medical Oncology, Molecular Biology, Nurse Practitioner, Oncology and Hematology, Pathology, Pediatrics, Pulmonology, Transfusion Medicine, Urology

Overview

This Journal-Based CME activity consists of a full-text article that is free to read, a multiple-choice question test, and an evaluation/self-assessment. Learners are tested on their comprehension of the key concepts, with the objective of enriching their clinical practice and patient care through better understanding of scientific advances and new techniques in the fields covered by Journal of Experimental Medicine (JEM). 

Questions for this complimentary, online-only activity have been designed by JEM editors in collaboration with the article authors. Each CME activity should take approximately 1 hour to complete.

The article selected for this activity is “T- and B-cell abnormalities, pneumocystis pneumonia and chronic lymphocytic leukemia associated with an AIOLOS defect in patients,” by Sergio D. Rosenzweig, Chief of the Immunology Service at the Clinical Center in the National Institutes of Health, and colleagues.

Article Abstract
AIOLOS/IKZF3 is a member of the IKAROS family of transcription factors. IKAROS/IKZF1 mutations have been previously associated with different forms of primary immunodeficiency. Here we describe a novel combined immunodeficiency due to an IKZF3 mutation in a family presenting with T and B cell involvement, Pneumocystis jirovecii pneumonia, and/or chronic lymphocytic leukemia. Patients carrying the AIOLOS p.N160S heterozygous variant displayed impaired humoral responses, abnormal B cell development (high percentage of CD21low B cells and negative CD23 expression), and abrogated CD40 responses. Naive T cells were increased, T cell differentiation was abnormal, and CD40L expression was dysregulated. In vitro studies demonstrated that the mutant protein failed DNA binding and pericentromeric targeting. The mutant was fully penetrant and had a dominant-negative effect over WT AIOLOS but not WT IKAROS. The human immunophenotype was recapitulated in a murine model carrying the corresponding human mutation. As demonstrated here, AIOLOS plays a key role in T and B cell development in humans, and the particular gene variant described is strongly associated with immunodeficiency and likely malignancy.

 

Click the 'Course Content' tab at the top of this page to access the article and instructions on claiming CME credit

 

About Journal of Experimental Medicine (JEM) and CME Activities
JEM publishes peer-reviewed papers within the field of medical biology, providing novel conceptual insights into immunology, cancer biology, neuroscience, inflammation, vascular biology, microbial pathogenesis, and stem cell biology. These studies range from original findings on all aspects of disease pathogenesis to novel therapeutic approaches. Articles designated for Journal-Based CME have been selected by JEM editors for their clinical relevance and ability to address practice-based gaps.


Accreditation

This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of Memorial Sloan Kettering Cancer Center, the Journal of Experimental Medicine (JEM) and Rockefeller University Press. Memorial Sloan Kettering Cancer Center is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

AMA Credit Designation Statement

Memorial Sloan Kettering Cancer Center designates this enduring material for a maximum of 1.00 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Recorded/Review Date: October 25, 2021; Original Release Date: October 25, 2021; Termination Date: October 25, 2024



Keywords: Complimentary CourseOn DemandAMA PRA Category 1Journal-Based Activity



Faculty Member Information
Role in activity
Name of Ineligible Company(s) / Nature of Relationship(s)
Jean-Laurent Casanova, M.D., Ph.D.

Activity Director
Nothing to disclose
Xin Sun, Ph.D.

Planner
Nothing to disclose
Rory Williams, BA
Rockefeller University Press
Planner

T- and B-cell abnormalities, pneumocystis pneumonia and chronic lymphocytic leukemia associated with an AIOLOS defect in patients

 

Hye Sun Kuehn, Jingjie Chang, Motoi Yamashita, Julie E. Niemela, Chengcheng Zou, Kazuki Okuyama, Junji Harada, Jennifer Stoddard, Cristiane Nunes-Santos, Brigette Boast, Ryan Baxter, Elena Wen-Yuan Hsieh, Mary Garofalo, Thomas Fleisher, Tomohiro Morio, Ichiro Taniuchi, Cullen M. Dutmer, and Sergio D. Rosenzweig

National Institutes of Health Clinical Center

Activity Instructions

  • Click the blue 'Access Article' button below. The article will open in a new window.
  • When you have read the article in its entirety, return to this page and click the 'Post Test' button to take the test. You must be signed in to complete the test and claim CME credit. If this is your first time on the MSK CME site, please create an account. 

  • Select your answer to each of the three questions and click 'Check Answer', then click 'Next' in the upper right corner. After the last question of the test, click 'Show Results'. If you have passed, click 'CME Account' claim CME credit. 

  • On the window that opens, click 'Complete Evaluation' next to the activity name and attest to your participation.

  • A certificate will be generated and appear on the page in the same location. To navigate back to this page at any time, click 'MY CME' and 'Evaluations & Certificates'. 

     

Access Article Post-Test

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