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Case Reports
. 2019 Feb 26;3(4):503-507.
doi: 10.1182/bloodadvances.2018027417.

Central nervous system-restricted familial hemophagocytic lymphohistiocytosis responds to hematopoietic cell transplantation

Hojun Li  1 , Leslie A Benson  2 , Lauren A Henderson  3 , Isaac H Solomon  4 , Alyssa L Kennedy  1 , Ariane Soldatos  5 , Bibiana Bielekova  6 , Jennifer Murphy  5 , Kimberly J Davies  1 , Leslie E Lehmann  1 , Michelle A Lee  7 , Sanda Alexandrescu  4 , Barbara A Degar  1 , Mark P Gorman  2 , Christine N Duncan  1
Affiliations
Case Reports

Central nervous system-restricted familial hemophagocytic lymphohistiocytosis responds to hematopoietic cell transplantation

Hojun Li et al. Blood Adv. .

Abstract

  1. Familial HLH can present as chronic isolated neuroinflammation.

  2. CNS-isolated HLH responds to hematopoietic cell transplantation.

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Conflict of interest statement

Conflict-of-interest disclosure: L.A.B. receives research support from Biogen Idec for clinical trial participation. B.B. has received patent royalty payments from the National Institutes of Health (NIH) for patents related to daclizumab therapy of multiple sclerosis. M.P.G. receives research funding from Biogen Idec and Novartis for clinical trial site participation and clinical research support from Pfizer, NIH, and the National Multiple Sclerosis Society. C.N.D. is a consultant for Bluebird Bio, Magenta Therapeutics, and AbGenomics. The remaining authors declare no competing financial interest.

Figures

Figure 1.
Figure 1.
Baseline and posttreatment CNS-HLH patient characteristics. Baseline characteristics for all patients in this study, including age at onset of symptoms, nature of clinical symptoms, age at eventual diagnosis, familial HLH mutation, therapy received before CNS-HLH diagnosis, CSF assessment, and hematoxylin and eosin–stained brain biopsy histopathology (20× magnification). Black arrows indicate perivascular lymphocytic infiltrates. Also displayed are T1 MRI scans and NK-cell immunologic tests at diagnosis and after HCT. White arrows indicate contrast-enhancing MRI lesions. -, NK-cell testing not indicated for the patient’s specific mutation. HCT, hematopoietic cell transplantation; IVIG, intravenous immune globulin; MMF, mycophenolate mofetil; NK, natural killer; WBC, white blood cell.
See this image and copyright information in PMC

References

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