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Access through your institution Buy or subscribe Acquired hemophilia A (AHA), a rare disease predominantly described in adults, has a reported incidence of 0.2–1.48 per million individuals
[1, 2]. Pathophysiology is incompletely understood but involves autoantibodies to coagulation factor VIII. AHA has been associated with pregnancy, autoimmunity, malignancy, infection,
post-autologous stem cell transplantation [2], and following penicillin exposure [3]. AHA is unlike congenital hemophilia A in bleeding patterns. Patients with AHA may develop spontaneous,
potentially life-threatening, soft tissue, muscle, and mucocutaneous bleeding, rather than hemarthroses; or they may exhibit mild or no bleeding symptoms. The autoantibodies exhibit
nonlinear second-order kinetics, such that low but measurable factor VIII activity levels may coexist with major hemorrhage. Management options for bleeding patients include factor
replacement to overcome low-titer inhibitors (<5 Bethesda units/mL, BU/mL), bypassing agents for high-titer inhibitor, and immunosuppression for inhibitor eradication [2]. Lupus
anticoagulants (LACs) are pro-thrombotic autoantibodies that interfere with phospholipid and protein interactions [4]. Anti-prothrombin antiphospholipid antibodies are rare, but are
associated with bleeding [4]. LACs are common in autoimmune disorders [4]. However, development of both AHA and LAC after allogeneic hematopoietic stem cell transplant (HSCT) in a pediatric
setting has not been reported. A ten-year-old Hispanic female with high-risk Philadelphia chromosome-like acute lymphoblastic leukemia in first complete remission (CR1) received conditioning
with total body irradiation and cyclophosphamide followed by allogeneic HSCT utilizing 9/10 HLA mismatched (at the A allele) bone marrow. Transplant was preceded by multiple, aggressive
cycles of chemotherapy, though remission was not achieved until blinatumomab was added to the treatment regimen. Post-HSCT course was complicated by acute and chronic gut, skin, and lung
GVHD treated using local steroids (topical and buccal), systemic steroids, topical calcipotriene, and photopheresis, with a variable but overall poor response. Ruxolitinib was added to her
GVHD treatment, with significant hepatotoxicity. She developed recurrent opportunistic bacteremias from her central line and chronic steroid use. This is a preview of subscription content,
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OPTIONS: * Log in * Learn about institutional subscriptions * Read our FAQs * Contact customer support REFERENCES * Giangrande P. Acquired hemophilia: revised edition. Treat Hemoph.
2012;38:1–7. Google Scholar * Webert KE. Acquired hemophilia A. Semin Thromb Hemost. 2012;38:735–41. Article CAS Google Scholar * Collins PW. Management of acquired haemophilia A. J
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USA. 1992;89:3795–9. Article CAS Google Scholar Download references ACKNOWLEDGEMENTS LJ performed data extraction and wrote the paper. JE-B oversaw data extraction, co-wrote and edited
the paper providing management for the overall project. LL-J and EM provided additional editing and valuable conceptual perspective. CD, KCM, SJ, and CTQ provided insights for manuscript
design and editing. AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children’s Hospital Medical Center, 3333 Burnet
Ave, MLC 11027, Cincinnati, OH, 45229, USA LaQuita Jones, Christopher Dandoy, Sonata Jodele, Kasiani C. Myers & Javier El-Bietar * Division of Hematology, Cincinnati Children’s Hospital
Medical Center, 3333 Burnet Ave, MLC 11027, Cincinnati, OH, 45229, USA Lori Luchtman-Jones, Charles T. Quinn & Eric Mullins Authors * LaQuita Jones View author publications You can also
search for this author inPubMed Google Scholar * Christopher Dandoy View author publications You can also search for this author inPubMed Google Scholar * Sonata Jodele View author
publications You can also search for this author inPubMed Google Scholar * Kasiani C. Myers View author publications You can also search for this author inPubMed Google Scholar * Lori
Luchtman-Jones View author publications You can also search for this author inPubMed Google Scholar * Charles T. Quinn View author publications You can also search for this author inPubMed
Google Scholar * Eric Mullins View author publications You can also search for this author inPubMed Google Scholar * Javier El-Bietar View author publications You can also search for this
author inPubMed Google Scholar CORRESPONDING AUTHOR Correspondence to Javier El-Bietar. ETHICS DECLARATIONS CONFLICT OF INTEREST EM has served on advisory boards for Shire and Bayer for
matters unrelated to this report. The remaining authors declare that they have no conflict of interest. RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE
Jones, L., Dandoy, C., Jodele, S. _et al._ Successful management of concurrent acquired hemophilia A and a lupus anticoagulant in a pediatric hematopoietic stem cell transplant patient.
_Bone Marrow Transplant_ 53, 487–489 (2018). https://doi.org/10.1038/s41409-017-0041-0 Download citation * Received: 20 June 2017 * Revised: 09 November 2017 * Accepted: 16 November 2017 *
Published: 12 January 2018 * Issue Date: April 2018 * DOI: https://doi.org/10.1038/s41409-017-0041-0 SHARE THIS ARTICLE Anyone you share the following link with will be able to read this
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