Immunoglobulin G4‑related acquired hemophilia: A case report

Li,Xiaoyan; Duan,Wei; Zhu,Xiang; Xu,Jianying

doi:10.3892/etm.2016.3898

Immunoglobulin G4‑related acquired hemophilia: A case report

Authors:
- Xiaoyan Li
- Wei Duan
- Xiang Zhu
- Jianying Xu
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Affiliations: Department of Respiratory Medicine, Shanxi DAYI Hospital of Shanxi Medical University, Taiyuan, Shanxi 030032, P.R. China, Department of Pathology, Peking University Third Hospital, Beijing 100191, P.R. China
Published online on: November 14, 2016 https://doi.org/10.3892/etm.2016.3898
Pages: 3988-3992
Copyright: © Li et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

Acquired hemophilia A (AHA) is a relatively rare and life‑threatening bleeding disorder whose pathogenesis is not completely understood. The present study reports a rare case of immunogubulin (IgG)4‑related AHA with multisystemic involvement. A 55‑year old male patient presented with symptoms of bronchial asthma and multiple subdermal hematomas. Chest computed tomography showed multiple diffuse nodular lesions with thickening of bronchovascular bundles, and scattered high‑density spots in both lung lobes. Laboratory investigations showed increased activated partial prothrombin time (120.0 sec), a markedly decreased factor VIII (FVIII) activity (0.5%), a high‑titer of FVIII inhibitor (27.2 Bethesda units/ml) and a marked increase in serum IgG4 (>4.03 g/l) level. Left inguinal lymph node biopsy revealed capsular thickening with marked lymphoplasmacytic infiltration, occlusive phlebitis and irregular fibrosis. Immunostaining revealed numerous IgG4‑positive plasma cells (>100 cells/human plasma fibronectin) in the nodular lesions, with an IgG4/IgG ratio of >40%. The symptoms were markedly alleviated following corticosteroid therapy. The current study presents the first reported case of a rare IgG4‑related AHA that presented with unusual clinical features and multisystemic involvement. The patient responded well to corticosteroid therapy. Documentation of such rare cases will help in characterizing the pathogenesis, and prompt recognition and timely treatment of this rare disorder.

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1	Collins P, Macartney N, Davies R, Lees S, Giddings J and Majer R: A population based, unselected, consecutive cohort of patients with acquired haemophilia A. Br J Haematol. 124:86–90. 2004. View Article : Google Scholar : PubMed/NCBI
2	Aljasser MI, Sladden C, Crawford RI and Au S: Bullous pemphigoid associated with acquired hemophilia a: A rare association of autoimmune disease. J Cutan Med Surg. 18:123–126. 2014. View Article : Google Scholar : PubMed/NCBI
3	Suzuki S, Kida S, Ohira Y, Ohba T, Miyata M, Nishimaki T, Morito T, Kasukawa R, Hojyo H and Wakasa H: A case of Sjögren's syndrome accompanied by lymphadenopathy and IgG4 hypergammaglobulinemia. Ryumachi. 33:249–254. 1993.PubMed/NCBI
4	Zen Y, Inoue D, Kitao A, Onodera M, Abo H, Miyayama S, Gabata T, Matsui O and Nakanuma Y: IgG4-related lung and pleural disease: A clinicopathologic study of 21 cases. Am J Surg Pathol. 33:1886–1893. 2009. View Article : Google Scholar : PubMed/NCBI
5	Ryu JH, Sekiguchi H and Yi ES: Pulmonary manifestations of immunoglobulin G4-related sclerosing disease. Eur Respir J. 39:180–186. 2012. View Article : Google Scholar : PubMed/NCBI
6	Khosroshahi A and Stone JH: A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. 23:57–66. 2011. View Article : Google Scholar : PubMed/NCBI
7	Raissian Y, Nasr SH, Larsen CP, Colvin RB, Smyrk TC, Takahashi N, Bhalodia A, Sohani AR, Zhang L, Chari S, et al: Diagnosis of IgG4-related tubulointerstitial nephritis. J Am Soc Nephrol. 22:1343–1352. 2011. View Article : Google Scholar : PubMed/NCBI
8	Khosroshahi A, Deshpande V and Stone JH: The clinical and pathological features of IgG (4)-related disease. Curr Rheumatol Rep. 13:473–481. 2011. View Article : Google Scholar : PubMed/NCBI
9	Zen Y and Nakanuma Y: IgG4-related disease: A cross-sectional study of 114 cases. Am J Surg Pathol. 34:1812–1819. 2010. View Article : Google Scholar : PubMed/NCBI
10	Kamisawa T and Okamoto A: Autoimmune pancreatitis: Proposal of IgG4-related sclerosing disease. J Gastroenterol. 41:613–625. 2006. View Article : Google Scholar : PubMed/NCBI
11	Hamano H, Kawa S, Ochi Y, Unno H, Shiba N, Wajiki M, Nakazawa K, Shimojo H and Kiyosawa K: Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 359:1403–1404. 2002. View Article : Google Scholar : PubMed/NCBI
12	Takeda S, Haratake J, Kasai T, Takaeda C and Takazakura E: IgG4-associated idiopathic tubulointerstitial nephritis complicating autoimmune pancreatitis. Nephrol Dial Transplant. 19:474–476. 2004. View Article : Google Scholar : PubMed/NCBI
13	Nagao Y, Yamanaka H and Harada H: A patient with hypereosinophilic syndrome that manifested with acquired hemophilia and elevated IgG4: A case report. J Med Case Rep. 6:632012. View Article : Google Scholar : PubMed/NCBI
14	Sugino K, Gocho K, Ishida F, Kikuchi N, Hirota N, Sato K, Sano G, Isobe K, Sakamoto S, Takai Y, et al: Acquired hemophilia A associated with IgG4-related lung disease in a patient with autoimmune pancreatitis. Intern Med. 51:3151–3154. 2012. View Article : Google Scholar : PubMed/NCBI
15	Sekiguchi H, Horie R, Aksamit TR, Yi ES and Ryu JH: Immunoglobulin G4-related disease mimicking asthma. Can Respir J. 20:87–89. 2013. View Article : Google Scholar : PubMed/NCBI
16	Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Yoshino T, Nakamura S, Kawa S, et al: Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 22:21–30. 2012. View Article : Google Scholar : PubMed/NCBI
17	Kessler CM: Acquired factor VIII autoantibody inhibitors: Current concepts and potential therapeutic strategies for the future. Haematologica. 85:(10 Suppl). S57–S61; discussion 61–63. 2000.
18	Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Sumida T, Mimori T, Tanaka Y, et al: A novel clinical entity, IgG4-related disease (IgG4RD): General concept and details. Mod Rheumatol. 22:1–14. 2012. View Article : Google Scholar : PubMed/NCBI
19	Kamisawa T, Takuma K, Tabata T, Inaba Y, Egawa N, Tsuruta K, Hishima T, Sasaki T and Itoi T: Serum IgG4-negative autoimmune pancreatitis. J Gastroenterol. 46:108–116. 2011. View Article : Google Scholar : PubMed/NCBI
20	Ebbo M, Daniel L, Pavic M, Sève P, Hamidou M, Andres E, Burtey S, Chiche L, Serratrice J, Longy-Boursier M, et al: IgG4-related systemic disease: Features and treatment response in a French cohort: Results of a multicenter registry. Medicine (Baltimore). 91:49–56. 2012. View Article : Google Scholar : PubMed/NCBI