Acquired hemophilia A associated with therapeutic cupping

Acquired hemophilia A is defined as the development of factor VIII inhibitors in a nonhemophilic patient. The inhibitors can develop in association with autoimmune disease, allergic drug reactions [1,2], malignancies [2], and pregnancy [3]. It is rare, but frequently complicated by severe bleeding and compartment syndrome [1,4]. Therapeutic cupping is a traditional remedy done by applying heated cup over the back and limbs to cause congestion. This procedure is mostly used by South Asian and Middle Eastern countries. We report a 58-year-old woman who presented with extensive and compressive bruising which led to pending compartment syndrome of her left thigh in 2 days after cupping. The coagulopathy was first considered owing to new-onset severe deep tissue bleeding. Acquired hemophlia A was diagnosed with prolonged activated partial thromboplastin time (83 seconds) and incorrectable mixing plasma test. Quantitative assays revealed reduced level of factor VIII (<0.1%) and the presence of factor VIII inhibitors (20 BU) in the serum. Fresh frozen plasma transfusion, coagulation factor supply, and immunosuppressive therapy with steroid began immediately after diagnosis was made. Her activated partial thromboplastin time was gradually normalized. She was discharged home with improving symptoms and minimized bruises 1 week later. To our knowledge, acquired hemophilia A associated with therapeutic cupping has never been mentioned before in the literature. Acquired hemophilia A is defined as the development of factor VIII inhibitors in a nonhemophilic patient. The inhibitors can develop in association with autoimmune disease, allergic drug reactions [1,2], malignancies [2], and pregnancy [3]. Therapeutic cupping is a traditional remedy done by applying heated cup over the back and limbs to cause congestion. In this article, we report a case of acquired hemophilia A associated with therapeutic cupping. A 58-year-old woman presented with bruises over her left arm and thigh which she first noticed 2 days earlier after cupping. Her medical history included schizophrenia, paranoid type, and bullous pemphigoid. Her hemostasis was previously normal and she did not take any anticoagulants or nonsteroidal anti-inflammatory drugs. The patient received cupping over her left arm and thigh to relieve joint pain and soreness. However, the bruising created by cupping became extensive and compressive which led to pending compartment syndrome of her left thigh in the past 2 days. There was no nose bleeding, hematuria, bloody stool, or accompanying hemoptysis. She denied having family history of bleeding disorder. On examination, her vital signs were stable and afebrile, but the patient looked in pain. No other bruising or active bleeding disclosed except extensive and compressive bruising over her left thigh and arm (Figs. 1 and 2). The laboratory tests revealed a white blood cell count of 14100 μL with a hemoglobin level of 9.9 g/dL, a platelet count of 283 × 10³ μL. Liver and kidney function were normal. Computed tomography scan of the lower limb showed poor expansion of the left popliteal and femoral veins and soft tissue swelling with mass effect (Fig. 3). Coagulation studies revealed normal prothrombin time and international normalized ratio, and prolonged activated partial thromboplastin time of 83 seconds (normal, <28 seconds). Initial 1:1 mixing of patient's plasma with the normal plasma resulted in partial correction of the activated partial thromboplastin time. The activated partial thromboplastin time remained prolonged on incubation period. The patient's factor VIII level was less than 0.1%, factor IX level was 62.3%, and the factor VIII inhibitor was 20 BU. The lupus anticoagulant and tumor marker screen tests showed negative results. Acquired hemophilia A was diagnosed. Fresh frozen plasma transfusion (4 U) and active factor VII (NovoSeven 6000 μg stat and q2h) were given to our patient. Oral prednisolone therapy (45 mg/d) was also added. Her activated partial thromboplastin time was gradually normalized. The pain was improved with minimized bruise and hematoma. She was discharged home with oral prednisolone therapy 1 week later. The etiology of acquired hemophilia A remains unclear. About half of the cases are associated with specific conditions, such as the postpartum period [3], allergic drug reactions, hematologic and solid malignancies, or autoimmune diseases [1,2]. Unlike in congenital hemophilia, bleeding tends to occur in soft tissue, muscle, the retroperitoneal space, and the gastrointestinal and genitourinary tract. The incidence rate of acquired hemophyllia is rare, about 1 person per million each year, but it is frequently complicated by severe bleeding and compartment syndrome [1,4]. The diagnosis can be difficult to make. Poor response to blood product transfusion and coagulation factor supply further worsens the outcome of the illness. Immunosuppressive therapy with steroid or cyclophosphamide should begin immediately after diagnosis is made [5]. Therapeutic cupping is a traditional treatment of Chinese medicine. It is used to treat shoulder, arm, and leg pain. A heated cup is attached to the skin surface to cause local congestion which is believed to relieve muscle spasm by temperature and negative pressure. These procedures seem to be harmless, but complication of dermal burn injury had been reported [6]. To our knowledge, acquired hemophilia A associated with therapeutic cupping has never been mentioned before. This case teaches us about acquired hemophilia A and its unusual etiology.