Spinal epidural hematoma in a child with hemophilia A with high titer inhibitors and follow-up with prophylactic emicizumab: case report and literature review.

Szczegóły
Abstrakt

Tytuł:: Spinal epidural hematoma in a child with hemophilia A with high titer inhibitors and follow-up with prophylactic emicizumab: case report and literature review.
Autorzy:: Villarreal-Martínez L; Hematology Service.
Sepúlveda-Orozco MDC; Pediatric Department, Hospital Universitario 'Dr José Eleuterio González', Universidad Autónoma de Nuevo León, Monterrey, México.
García-Viera DA; Hematology Service.
Robles-Sáenz DA; Hematology Service.
Bautista-Gómez AJ; Hematology Service.
Ortiz-Castillo M; Hematology Service.
González-Martínez G; Hematology Service.
Mares-Gil JE; Hematology Service.
Źródło:: Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis [Blood Coagul Fibrinolysis] 2021 Sep 01; Vol. 32 (6), pp. 418-422.
Typ publikacji:: Case Reports; Journal Article; Review
Język:: English
Imprint Name(s):: Publication: London : Lippincott Williams And Wilkins
Original Publication: Oxford, UK : Rapid Communications of Oxford Ltd., c1990-
MeSH Terms:: Antibodies, Bispecific/*therapeutic use
Antibodies, Monoclonal, Humanized/*therapeutic use
Factor VIIa/*therapeutic use
Hematoma, Epidural, Spinal/*drug therapy
Hemophilia A/*drug therapy
Child, Preschool ; Hematoma, Epidural, Spinal/etiology ; Hemophilia A/complications ; Hemorrhage/prevention & control ; Humans ; Male ; Recombinant Proteins/therapeutic use
References:: Franchini M, Mannucci PM. Past, present and future of hemophilia: a narrative review. Orphanet J Rare Dis 2012; 7:24.
Kulkarni R, Soucie MJ. Pediatric hemophilia: a review. Semin Thromb Hemost 2011; 37:737–744.
Benson G, Auerswald G, Dolan G, Duffy A, Hermans C, Ljung R, et al. Diagnosis and care of patients with mild haemophilia: practical recommendations for clinical management. Blood Transfus 2018; 16:535–544.
Kreppel D, Antoniadis G, Seeling W. Spinal hematoma: a literature survey with meta-analysis of 613 patients. Neurosurg Rev 2003; 26:1–49.
Borkar SA, Prasad GL, Satyarthee GD, Mahapatra AK. Spontaneous spinal extradural hematoma in a child with hemophilia B, surgery or medical management – a dilemma? J Pediatr Neurosci 2011; 6:131–133.
Babayev R, Ekşi MŞ. Spontaneous thoracic epidural hematoma: a case report and literature review. Childs Nerv Syst 2016; 32:181–187.
Woods GM, Dunn MW, Dunn AL. Emergencies in hemophilia. Clin Pediatr Emerg Med 2018; 19:110–121.
Singh S, Philip CC, George U, John MJ. Spinal epidural bleed in hemophilia: a rare site of bleeding with complete resolution with nonsurgical management. J Pediatr Hematol Oncol 2019; 41:215–217.
Schoonjans AS, De Dooy J, Kenis S, Menousky T, Verhulst S, Hellinckx J, et al. Spontaneous spinal epidural hematoma in infancy: review of the literature and the ‘seventh’ case report. Eur J Paediatr Neurol 2013; 17:537–542.
Morsing IE, Brons P, Th Draaisma JM, van Lindert EJ, Erasmus CE. Hemophilia a and spinal epidural hematoma in children. Neuropediatrics 2009; 40:245–248.
Aras M, Oral S. Management of intracranial hemorrhage in hemophilia A patients. Childs Nerv Syst 2020; 36:2041–2046.
Salaj P, Brabec P, Penka M, Pohlreichova V, Smejkal P, Cetkovsky P, et al. Effect of rFVIIa dose and time to treatment on patients with haemophilia and inhibitors: analysis of HemoRec registry data from the Czech Republic. Haemophilia 2009; 15:752–759.
Pool JG, Shannon AE. Production of high-potency concentrates of antihemophilic globulin in a closed-bag system. N Engl J Med 1965; 273:1443–1447.
Franchini M, Mannucci PM. The history of hemophilia. Semin Thromb Hemost 2014; 40:571–576.
Sheikh AA, Abildgaard CF. Medical management of extensive spinal epidural hematoma in a child with factor IX deficiency. Pediatr Emerg Care 1994; 10:26–29.
Afshari FT, Parikh D, Petrik V. Spontaneous spinal epidural haematoma secondary to autoimmune acquired haemophilia. Case Rep Neurol 2018; 10:353–356.
Noth I, Hutter JJ, Meltzer PS, Damiano ML, Carter LP. Spinal epidural hematoma in a hemophilic infant. Am J Pediatr Hematol Oncol 1993; 15:131–134.
Morichika S, Shima M, Imanaka Y, Nakajima M, Iwasaki S, Yoshioka A. Spinal canal bleeding in hemophilia A. Rinsho Ketsueki 1995; 36:687–693.
Leach M, Makris M, Hampton KK, Preston FE. Spinal epidural haematoma in haemophilia A with inhibitors—efficacy of recombinant factor VIIa concentrate. Haemophilia 1999; 5:209–212.
Irwin SL, Attia MW. A traumatic spinal epidural hematoma in an infant with hemophilia A. Pediatr Emerg Care 2001; 17:40–41.
Kalina P, Morris J, Raffel C. Spinal epidural hematoma in an infant as the initial presentation of severe hemophilia. Emerg Radiol 2008; 15:281–284.
Rois PV, López MR, de Vergara BC, de la Lama Zaragoza A, García JG, Uxo JM. Spinal epidural hematoma in hemophilic children: controversies in management. Childs Nerv Syst 2009; 25:987–995.
Oymak Y, Muminoglu N, Ay Y, Karapinar TH, Köker SA, Töret E, et al. Torticollis in a haemophilic infant with inhibitor: a case of spinal epidural haematoma. Blood Coagul Fibrinolysis 2016; 27:576–579.
Erkutlu I, Bay A, Aktekin E. Conservative management of an acute spinal epidural hemorrhage in a child with hemophilia a with inhibitor. Blood Coagul Fibrinolysis 2016; 27:930–932.
Tamura K, Kanazawa T, Suzuki M, Shioya A, Morikawa A. Successful induction of immune tolerance by continuous infusion of recombinant factor VIII in a haemophilia A patient with high-inhibitor titres. Haemophilia 2006; 12:100–102.
Bilir ÖA H, Yarali N, Kac¸ar D, Kara A, C¸ulha V, Özbek NY. Spinal epidural hematoma in hemophilia. Poster presented at: WFH 2018 World Congress; 20–24 May 2018; Glasgow, Scotland.
Stanley P, McComb JG. Chronic spinal epidural hematoma in hemophilia A in a child. Pediatr Radiol 1983; 13:241–243.
Abdelaal MA, McGuinness FE, Sagar G. Case report: spinal extradural haematoma in haemophilia-A – a diagnosis not to be missed. Clin Radiol 1994; 49:573–575.
Travis SF, Greenbaum BH, Mintz M, White RM, Stein S, Steckler RE, et al. Epidural hematoma of a cauda equina in a child with hemophilia A. J Pediatr Hematol Oncol 1996; 18:308–310.
Chretiennot-Bara C, Guet A, Balzamo E, Noseda G, Torchet MF, Rothshild C, et al. Epidural hematoma in a child with hemophilia: diagnostic difficulties. Arch Pediatr 2001; 8:828–833.
Cuvelier GD, Davis JH, Purves EC, Wu JK. Torticollis as a sign of cervicothoracic epidural haematoma in an infant with severe haemophilia A. Haemophilia 2006; 12:683–686.
Iizuka Y, Iizuka H, Nagano K, Tsutsumi S, Nakajima T, Takagishi K, et al. Laminoplasty preserving the deep extensor musculature for a pediatric cervicothoracic epidural hematoma case of hemophilia A. J Pediatr Orthop 2014; 23:584–588.
Cromwell LD, Kerber C, Ferry PC. Spinal cord compression and hematoma: an unusual complication in a hemophiliac infant. AJR Am J Roentgenol 1977; 128:847–849.
Faillace WJ, Warrier I, Canady AI. Paraplegia after lumbar puncture. In an infant with previously undiagnosed hemophilia A. Treatment and peri-operative considerations. Clin Pediatr (Phila) 1989; 28:136–138.
Heer JS, Enriquez EG, Carter AJ. Spinal epidural hematoma as first presentation of hemophilia A. J Emerg Med 2008; 34:159–162.
Lee HJ. A case report spontaneous spinal epidural hematoma diagnosed in a hemophilic child with neck pain. J Korean Soc Emerg Med 2018; 29:231–235.
Zhong W, Chen H, You C, Huang S. Spinal epidural hematoma in a child with hemophilia B. Neurol India 2011; 59:294–296.
Nirupam N, Pemde H, Chandra J. Spinal epidural hematoma in a patient with hemophilia B presenting as acute abdomen. Indian J Hematol Blood Transfus 2014; 30: (Suppl 1): 54–56.
Fessey BM, Meynell MJ. Haemorrhage involving the central nervous system in haemophilia: account of the management of five cases. Br Med J 1966; 2:211–212.
Harvie A, Lowe GD, Forbes CD, Prentice CR, Turner J. Intraspinal bleeding in haemophilia: successful treatment with factor VIII concentrate. J Neurol Neurosurg Psychiatry 1977; 40:1220–1223.
Narawong D, Gibbons VP, McLaughlin JR, Bouhasin JD, Kotagal S. Conservative management of spinal epidural hematoma in hemophilia. Pediatr Neurol 1988; 4:169–171.
Hamre MR, Haller JS. Intraspinal hematomas in hemophilia. Am J Pediatr Hematol Oncol 1992; 14:166–169.
Hutt PJ, Herold ED, Koenig BM, Gilchrist GS. Spinal extradural hematoma in an infant with hemophilia A: an unusual presentation of a rare complication. J Pediatr 1996; 128 (5 Pt 1):704–706.
Schmitz A, Wallny T, Sommer T, Brackmann H, Schulze-Bertelsbeck D, Effenberger W, et al. Spinal epidural haematoma in hemophilia A. Hemophilia 1998; 4:51–55.
Iwamuro H, Morita A, Kawaguchi H, Kirino T. Resolution of spinal epidural haematoma without surgery in a haemophilic infant. Acta Neurochir (Wien) 2004; 146:1263–1265.
Balkan C, Kavakli K, Karapinar D. Spinal epidural haematoma in a patient with haemophilia B. Haemophilia 2006; 12:437–440.
Bisson EF, Dumont T, Tranmer B. Spontaneous spinal epidural hematoma in a child with hemophilia B. Can J Neurol Sci 2007; 34:488–490.
Kubota T, Miyajima Y. Spinal extradural haematoma due to haemophilia A. Arch Dis Child 2007; 92:498.
Tagaya A, Miyajima Y, Sakamoto M, Ito M, Nakamura L, Ohe H, et al. Spontaneous spinal epidural hematoma in an infant with undiagnosed hemophilia A. Pediatr Int 2010; 52:296–298.
Kiehna EN, Waldron PE, Jane JA. Conservative management of an acute spontaneous holocord epidural hemorrhage in a hemophiliac infant. J Neurosurg Pediatr 2010; 6:43–48.
Rathi N, Rathi A. Haemophilia A presenting as spontaneous spinal epidural haematoma in a 3-month-old infant. BMJ Case Rep 2010; 2010:.
Fruchtman Y, Dardik R, Barg AA, Livnat T, Feldman Z, Rubinstein M, et al. Spinal epidural hematoma following cupping glass treatment in an infant with hemophilia A. Pediatr Blood Cancer 2016; 63:1120–1122.
Keely ML, Taylor N, Chard RL Jr. Spinal cord compression as a complication of haemophilia. Arch Dis Child 1972; 47:826–828.
Young G, Liesner R, Chang T, Sidonio R, Oldenburg J, Jiménez-Yuste V, et al. A multicenter, open-label phase 3 study of emicizumab prophylaxis in children with hemophilia A with inhibitors. Blood 2019; 134:2127–2138.
Young G, Callaghan M, Dunn A, Kruse-Jarres R, Pipe S. Emicizumab for hemophilia A with factor VIII inhibitors. Expert Rev Hematol 2018; 11:835–846.
Sakamoto A, Nakadate H, Watanabe N, Ishiguro A. Successful treatment by switching from activated prothrombin complex concentrate to emicizumab therapy in a hemophilia A patient with inhibitors. Rinsho Ketsueki 2020; 61:617–620.
Substance Nomenclature:: 0 (Antibodies, Bispecific)
0 (Antibodies, Monoclonal, Humanized)
0 (Recombinant Proteins)
7NL2E3F6K3 (emicizumab)
AC71R787OV (recombinant FVIIa)
EC 3.4.21.21 (Factor VIIa)
Entry Date(s):: Date Created: 20210416 Date Completed: 20211126 Latest Revision: 20230924
Update Code:: 20240104
DOI:: 10.1097/MBC.0000000000001038
PMID:: 33859115
: Czasopismo naukowe

Hemorrhage in the central nervous system is the most severe and debilitating manifestation affecting patients with hemophilia A. The spinal epidural space is the most unusual and clinically challenging site of central nervous system hemorrhage in hemophilia A. These patients often show insidious neurological signs and symptoms that delay diagnosis and treatment. We share our experience treating a 4-year-old male patient with severe hemophilia A and high titer inhibitors with a spontaneous spinal epidural hematoma. The patient presented initially with intense headache and neck pain. After blood tests and imaging studies, bypassing agent therapy with recombinant-activated factor VII was used until discharge; this was later replaced with emicizumab. After 18 months, the patient is without neurological sequelae and has not experienced subsequent bleeding episodes. We review the available literature and discuss the relevance of emicizumab compared with standard therapies in the context of spontaneous spinal epidural hematoma.
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