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Case report
A case of Moyamoya disease complicated by aHUS in a patient with a history of marginal zone B-cell lymphoma: lightning strikes thrice?
  1. Ana Valle1,
  2. Vassiliki Papagermanos1,
  3. Michael Wotman1 and
  4. Dana Shani2
  1. 1Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, New York, USA
  2. 2Department of Hematology/Oncology, Lenox Hill Hospital, Northwell Health, New York City, New York, USA
  1. Correspondence to Dr Ana Valle; avalle1{at}


Atypical haemolytic uraemic syndrome (aHUS) is a rare, acquired thrombotic microangiopathy, mediated by complement activation, in very sick patients. Moyamoya is similarly a rare disease in which stenosis or occlusion of segment(s) of the anterior cerebral circulation leads to the formation of many thin collaterals. Other reports have described an association between HUS and Moyamoya disease in the paediatric population. However, this case study presents the exceptionally rare presentation of an adult with aHUS and Moyamoya disease in a patient who was treated with rituximab for marginal zone B-cell lymphoma.

  • haematology (incl blood transfusion)
  • Moyamoya
  • neurology
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  • Contributors The authors listed have made significant contributions to the case report, including drafting, revising, and approving the final version submitted. DS and AV: conceived of the presented idea. DS: encouraged VP to investigate MDD and write her findings and MW to investigate TTP and write his findings; revised the findings complied by VP, MW and AV. AV: compiled the patient’s clinical course, found patient’s cerebrovascular images (figure 1), and created visual graphic (figure 2); also searched literature for similar clinical presentations. All authors discussed the results and contributed to the final manuscript. They agree to be accountable for all aspects of the work, including accuracy of data and sources.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Next of kin consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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