Successful Treatment of Henoch-Schönlein Purpura-associated Hematochezia in a Child with Hemophilia A: a Case Report

Overview

Journal BMC Pediatr

Publisher Biomed Central

Specialty Pediatrics

Date 2023 Mar 1

PMID 36859289

Authors

Kai Feng

Chang Liu

Keqing Zhang

Jing Hao

Affiliations

Soon will be listed here.

Abstract

Background: Henoch-Schönlein purpura (HSP) is a common form of immunological vasculitis in children. Hemophilia A is a genetic disorder and characterized by spontaneous hemorrhage or prolonged bleeding due to factor VIII deficiency. Both diseases increase the risk of bleeding, but they have different mechanisms. How should we treat patients with both diseases?

Case Presentation: An 8-year-old male with hemophilia A was diagnosed with HSP while receiving coagulation factor VIII replacement therapy in our hospital. Hematochezia occurred 6 days after the diagnosis of HSP. And he treated with coagulation FVIII, methylprednisolone and hemostatic drugs.

Conclusions: There is no causal relationship between hemophilia A and HSP, but both diseases can cause bleeding. This child's hematochezia was caused by HSP, but hemophilia could not be ignored during the treatment. Our case report adds to the present body of knowledge about the treatment of HSP associated hematochezia in a child with hemophilia A.

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References

Srivastava A, Brewer A, Mauser-Bunschoten E, Key N, Kitchen S, Llinas A . Guidelines for the management of hemophilia. Haemophilia. 2012; 19(1):e1-47. DOI: 10.1111/j.1365-2516.2012.02909.x. View

Fisher J, Stamm D, Modi B, Duggan C, Jaksic T . Gastrointestinal bleeding as a complication of serial transverse enteroplasty. J Pediatr Surg. 2014; 49(5):745-9. PMC: 4468065. DOI: 10.1016/j.jpedsurg.2014.02.060. View

Melero M, Radaelli E, GENNARO O, KAMINKER A . [Schönlein-Henoch purpura in a patient with hemophilia A]. Rev Clin Esp. 1991; 189(7):348-9. View

Joly B, dOiron R, Desconclois C, Bendelac L, Rafowicz A, Meyzer C . [Mild hemophilia A fortuitously discovered during Henoch-Schönlein purpura]. Arch Pediatr. 2015; 22(11):1167-70. DOI: 10.1016/j.arcped.2015.07.014. View

Kang H, Chung H, Kang K, Han K . High-dose methylprednisolone pulse therapy for treatment of refractory intestinal involvement caused by Henoch-Schönlein purpura: a case report. J Med Case Rep. 2015; 9:65. PMC: 4378549. DOI: 10.1186/s13256-015-0545-4. View

Rigante D, Castellazzi L, Bosco A, Esposito S . Is there a crossroad between infections, genetics, and Henoch-Schönlein purpura?. Autoimmun Rev. 2013; 12(10):1016-21. DOI: 10.1016/j.autrev.2013.04.003. View

Arlt A, Schuldt D, Nikolaus S, Schafmayer C, von Schoenfels W, Schreiber S . Abdominal malrotation overlooked at extensive cross-sectional imaging and leading to life-threatening gastrointestinal bleeding. Endoscopy. 2015; 47 Suppl 1:E639-40. DOI: 10.1055/s-0034-1393651. View

Hamzaoui A, Melki W, Harzallah O, Njim L, Klii R, Mahjoub S . Gastrointestinal involvement revealing Henoch Schonlein purpura in adults: Report of three cases and review of the literature. Int Arch Med. 2011; 4(1):31. PMC: 3213065. DOI: 10.1186/1755-7682-4-31. View

Xu H, Jiang G, Shen H, Li W, Mao J, Pan Y . Association of TLR4 gene polymorphisms with childhood Henoch-Schönlein purpura in a Chinese population. Rheumatol Int. 2017; 37(11):1909-1915. DOI: 10.1007/s00296-017-3815-1. View

10.

Carron D, BOON T, Walker F . Peptic ulcer in the haemophiliac and its relation to gastrointestinal bleeding. Lancet. 1965; 2(7421):1036-9. DOI: 10.1016/s0140-6736(65)90569-6. View

11.

Giordano P, Lassandro G, Valente M, Molinari A, Ierano P, Coppola A . Current management of the hemophilic child: a demanding interlocutor. Quality of life and adequate cost-efficacy analysis. Pediatr Hematol Oncol. 2014; 31(8):687-702. DOI: 10.3109/08880018.2014.930768. View

12.

Lin Q, Li X . Children with Henoch-Schönlein purpura with low complement levels: follow-up for >6 years. Pediatr Nephrol. 2017; 32(7):1279. DOI: 10.1007/s00467-017-3630-7. View

13.

Mannucci P, Tuddenham E . The hemophilias--from royal genes to gene therapy. N Engl J Med. 2001; 344(23):1773-9. DOI: 10.1056/NEJM200106073442307. View

14.

Fischer K, Steen Carlsson K, Petrini P, Holmstrom M, Ljung R, van den Berg H . Intermediate-dose versus high-dose prophylaxis for severe hemophilia: comparing outcome and costs since the 1970s. Blood. 2013; 122(7):1129-36. PMC: 3744988. DOI: 10.1182/blood-2012-12-470898. View

15.

Fischer K, Astermark J, van der Bom J, Ljung R, Berntorp E, Grobbee D . Prophylactic treatment for severe haemophilia: comparison of an intermediate-dose to a high-dose regimen. Haemophilia. 2002; 8(6):753-60. DOI: 10.1046/j.1365-2516.2002.00694.x. View

16.

de Almeida J, Campos L, Paim L, Leone C, Koch V, Silva C . Renal involvement in Henoch-Schönlein purpura: a multivariate analysis of initial prognostic factors. J Pediatr (Rio J). 2007; 83(3):259-66. DOI: 10.2223/JPED.1638. View

17.

Sano H, Izumida M, Shimizu H, Ogawa Y . Risk factors of renal involvement and significant proteinuria in Henoch-Schönlein purpura. Eur J Pediatr. 2002; 161(4):196-201. DOI: 10.1007/s00431-002-0922-z. View

18.

Janic D, Smoljanic Z, Krstovski N, Dokmanovic L, Rodic P . Ruptured intramural intestinal hematoma in an adolescent patient with severe hemophilia A. Int J Hematol. 2009; 89(2):201-203. DOI: 10.1007/s12185-008-0236-7. View

19.

Weiss P, Feinstein J, Luan X, Burnham J, Feudtner C . Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review. Pediatrics. 2007; 120(5):1079-87. PMC: 3525094. DOI: 10.1542/peds.2007-0667. View

20.

Valentino L . Blood-induced joint disease: the pathophysiology of hemophilic arthropathy. J Thromb Haemost. 2010; 8(9):1895-902. DOI: 10.1111/j.1538-7836.2010.03962.x. View