Successful management of concurrent acquired hemophilia a and a lupus anticoagulant in a pediatric hematopoietic stem cell transplant patient

Successful management of concurrent acquired hemophilia a and a lupus anticoagulant in a pediatric hematopoietic stem cell transplant patient

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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.


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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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