Acquired Hemophilia a Secondary to Autoimmune Pancreatitis with Elevated IG4

Case Report

Thromb Haemost Res. 2021; 5(2): 1058.

Acquired Hemophilia a Secondary to Autoimmune Pancreatitis with Elevated IG4

Li Y¹, Li J¹, He H², Cai R¹ and Feng Q¹*

1Department of Clinical Laboratory, Tai’an Central Hospital, Shandong Province, China

2Department of Blood Transfusion, Tai’an Hospital of Traditional Chinese Medicine, Shandong Province, China

*Corresponding author: Qiang Feng, Department of Clinical Laboratory, Tai’an Central Hospital, 29th Longtan Road, Tai’an City, Shandong Province, 271000, China

Received: March 22, 2021; Accepted: April 16, 2021; Published: April 23, 2021

Abstract

Acquired Hemophilia A (AHA) is a rare disease resulting from autoantibodies against Factor VIII (FVIII) that leads to bleeding. AHA associated with IgG4 related diseases is even rarer. The patient was diagnosed with IgG4 associated autoimmune pancreatitis in January 2019, and the condition improved after two hospitalizations. However, 22 months later, the patient was admitted to hospital due to generalized bleeding points. He was diagnosed with AHA and improved after hormone therapy and plasma exchange. Although IgG4 is associated with IgG4-related disease and AHA, its relevance to the etiology of both diseases is not well understood.

Introduction

Autoimmune pancreatitis (autoimmune pancreatitis, AIP) is a special type of chronic pancreatitis mediated by autoimmunity [1]. Type 1 AIP is local manifestations of IgG4 related diseases (IgG4 related disease, IgG4 RD) in pancreas which is often accompanied by extrapancreatic manifestations, such as sclerosing cholangitis, sialitis, dacryitis, retroperitoneal fibrosis, lymphadenopathy, interstitial pneumonia and renal tubulointerstitial nephritis. Glucocorticoid is effective, but often relapses [2].

Acquired Hemophilia A (AHA) is a disease caused by acquired autoantibodies that inhibit Factor VIII (FVIII) activity, and it leads to severe hemorrhages. The antibodies are mostly sub-classified as IgG4 and IgG1 [3]. Acquired hemophilia A associated with IgG4 related diseases is rare.

Case Presentation

A 65-year-old man was admitted to the stomatology department of our hospital due to bilateral submandibular gland tumors in November 2018. His laboratory findings showed raised serum IgG concentration of 44.34 g/l and slightly prolonged plasma Prothrombin Time (PT) 14.30s, while the other laboratory results were basically normal. The patient was discharged three days later without further diagnosis and treatment.

Due to skin and sclera yellow staining, skin pruritus, white pottery soil and weight loss of 5kg, the patient underwent MRI examination in a lower hospital, which indicated the tumor of common hepatic duct, so he was transferred to our hospital and admitted to the oncology department in January 2019. His laboratory findings showed raised serum IgG concentration of 44.34 g/l, hepatobiliary enzymes increased significantly and tumor markers elevated as shown in Table 1. Computed tomography showed Intrahepatic bile duct dilatation, gallbladder atresia, annular thickening of middle and upper segment of common bile duct, mild dilatation of bile duct in left lobe of liver, abnormal enhancement of pancreas and kidney. Comprehensive consideration was that sclerosing cholangitis and autoimmune pancreatitis were more likely. He was treated with Hydroprednisone (30mg/day) and Polyene phosphatidylcholine (465mg/day) for 3 days. The patient’s condition improved and asked to be discharged.

Citation: Li Y, Li J, He H, Cai R and Feng Q. Acquired Hemophilia a Secondary to Autoimmune Pancreatitis with Elevated IG4. Thromb Haemost Res. 2021; 5(2): 1058.