Analysis of the Use of Anticoagulants and Antiplatelet Agents in Strokes Caused By the Deficiency of Adenosine Deaminase 2

Patrycja Hoffmann¹, Amanda K. Ombrello², Deborah L. Stone¹, Karyl Barron³, Gineth Pinto-Patarroyo¹, Anne Jones¹, Tina Romeo⁴, Dean Follmann⁵, Camilo Toro⁶, Ariane Soldatos⁷, Qing Zhou⁸, Ivona Aksentijevich¹ and Daniel L. Kastner¹, ¹Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ²Inflammatory Diseases Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ³National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ⁴National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ⁵NIAID, Bethesda, MD, ⁶NIH Undiagnosed Diseases Program, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ⁷National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, ⁸Inflammatory Disease Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Fever

Session Information

Date: Tuesday, November 15, 2016

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects - Poster III: Systemic JIA, Autoinflammatory Syndromes, Scleroderma, Vasculitis, Miscellaneous

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Deficiency of adenosine deaminase 2 (DADA2) is a recessive genetic condition in which children develop recurrent strokes, intermittent fevers, elevated acute-phase reactants, livedoid rash, hepatosplenomegaly, and hypglobulinemia caused by mutations in the CECR1 gene. Blood levels of ADA2 are very low or absent, compromising endothelial integrity while polarizing macrophage and monocyte subsets toward proinflammatory cells. In this abstract we aim to determine the safety of treating DADA2 patients with a history of stroke with anticoagulants and/or antiplatelet agents.

Methods: A single center study evaluated 22 patients who were positive for 2 mutations in CECR1. All patients underwent a clinical history and physical examination, neurologic evaluation, brain MRI/MRA, and testing for ESR/CRP, paying special attention to whether there was a history of stroke, the type of stroke (ischemic or hemorrhagic), and the history of the use of ASA either alone or in combination with anticoagulants and/or other antiplatelet agents. The primary outcome measure was to determine occurrence of hemorrhagic strokes while on anticoagulant and/or antiplatelet treatment.

Results: Out of 22 patients, 15 had strokes and 13 of those were subsequently started on anticoagulants and/or antiplatelet agents. Amongst the 13, 4 of the patients had a total of 6 hemorrhagic strokes. All 4 patients with hemorrhagic strokes were on ASA plus other anticoagulants and/or antiplatelet agents. Six of the patients with no hemorrhagic strokes were on ASA alone. Patients not on anticoagulants and/or antiplatelet agents did not have any hemorrhagic strokes. Nine out of the 13 patients who were put on anticoagulants and/or antiplatelet agents had an ischemic stroke only. Upon analyzing the statistical data, the probability of a hemorrhagic stroke following anticoagulants and/or antiplatelet agents was 4/13 while the probability of a hemorrhagic stroke following no anticoagulants and/or antiplatelet agents was 0/9. A Fisher’s exact test was be used to examine whether these 2 probabilities truly differ and the p-value was 0.115 which is short of statistical significance. The proportions of patients with hemorrhagic strokes on no antiplatelet or anticoagulant treatment, ASA alone, and ASA plus other anticoagulants and/or antiplatelet agents were 0/9, 0/6 and 4/7, respectively (p = 0.007, Fischer’s exact test).

Conclusion: In patients with DADA2, we oberseved an increased frequency of hemorrhagic strokes in those treated with ASA plus other anticoagulants and/or antiplatelet agents, although we observed no hemorrhagic strokes in patients treated with ASA alone. These observations suggest extreme caution in the use of ASA plus other anticoagulants and/or antiplatelet agents in DADA2.

Disclosure: P. Hoffmann, None; A. K. Ombrello, None; D. L. Stone, None; K. Barron, None; G. Pinto-Patarroyo, None; A. Jones, None; T. Romeo, None; D. Follmann, None; C. Toro, None; A. Soldatos, None; Q. Zhou, None; I. Aksentijevich, None; D. L. Kastner, None.

To cite this abstract in AMA style:

Hoffmann P, Ombrello AK, Stone DL, Barron K, Pinto-Patarroyo G, Jones A, Romeo T, Follmann D, Toro C, Soldatos A, Zhou Q, Aksentijevich I, Kastner DL. Analysis of the Use of Anticoagulants and Antiplatelet Agents in Strokes Caused By the Deficiency of Adenosine Deaminase 2 [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/analysis-of-the-use-of-anticoagulants-and-antiplatelet-agents-in-strokes-caused-by-the-deficiency-of-adenosine-deaminase-2/. Accessed .

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/analysis-of-the-use-of-anticoagulants-and-antiplatelet-agents-in-strokes-caused-by-the-deficiency-of-adenosine-deaminase-2/