BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated w...BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated with autoimmune diseases,cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid(BP) is a chronic autoimmune subepidermal blistering disease associatedwith tissue-bound and circulating autoantibodies against BP antigens 180 (BP180)and 230 (BP230). AHA-associated BP has a high mortality rate;hence, the understandingof this disease must improve.CASE SUMMARY A 69-year-old man presented with erythema, blisters, blood blisters, and crustsaccompanied by severe pruritus for more than 20 days, and ecchymosis andswelling on his left upper arm for 3 days. Pathological examination revealed asubepidermal blister that contained eosinophils. Laboratory tests showed that theBP180 autoantibody levels had increased, isolated activated partial thromboplastintime was notably prolonged (115.6 s), and coagulation FⅧ activity wasextremely low (< 1.0%). Furthermore, the FⅧ inhibitor titer had greatlyincreased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospitalfor effective treatment;however, he died after 2 days.CONCLUSION AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effectivetreatment are necessary.展开更多
BACKGROUND Acquired hemophilia is rare.In some cases,the bleeding in muscle causes compartment syndrome.However,it is not clear whether fasciotomy should be performed for the compartment syndrome caused by acquired he...BACKGROUND Acquired hemophilia is rare.In some cases,the bleeding in muscle causes compartment syndrome.However,it is not clear whether fasciotomy should be performed for the compartment syndrome caused by acquired hemophilia because of the risk of bleeding and the unknown functional results.CASE SUMMARY A 75-year-old woman was admitted with severe pain of the right forearm with no preceding traumatic event.The right forearm was obviously swollen,and stretch pain was observed.Subcutaneous hematomas were suspected in various parts of the body.Compartment pressure was 110 mmHg on the volar side.Activated partial thromboplastin time(aPTT)was prolonged to 54.9 s.Fasciotomy was performed,and hematoma was observed in the volar compartment.Postoperative laboratory examinations revealed a low level of factor Ⅷ(FⅧ)activity(12.5%)and a high level of FⅧ inhibitor(15.2 bethesda units/mL).Acquired hemophilia A was diagnosed.Though recombinant clotting factors were administered,transfusion of red blood cells reached 46 units(140 mL/unit).Hemostasis was achieved 9 d after fasciotomy.The total cost of the clotting factor concentrates administered reached 28834600 yen.With prednisolone,FⅧ activity and aPTT recovered gradually.Final function of the hand was good in the index finger and excellent in the others.CONCLUSION Fasciotomy resulted in good function of the hand in a case of non-traumatic compartment syndrome caused by acquired hemophilia,but life-threatening bleeding occurred,and the cost of clotting factor treatment was high.Preparation of sufficient blood transfusion,preoperative administration of recombinant activated clotting factor Ⅶ,and prompt fasciotomy could be ideal for such cases.展开更多
Acquired hemophilia A （AHA） is a rare, hemorrhagic autoimmune disease, whose pathogenesis involves reduced coagulation factor Ⅷ （FⅧ） activity related to the appearance of inhibitors against FⅧ. Common etiologic...Acquired hemophilia A （AHA） is a rare, hemorrhagic autoimmune disease, whose pathogenesis involves reduced coagulation factor Ⅷ （FⅧ） activity related to the appearance of inhibitors against FⅧ. Common etiological factors include autoimmune diseases, ma-lignancy, and pregnancy. We report two cases of AHA in solid cancer. The first case is a 63-year-old man who developed peritoneal and intestinal bleeding after gastrectomy for gastric cancer. He was diagnosed with AHA, and was treated with prednisone, followed by cyclophosphamide. In the second case, a 68-year-old man developed a subcutaneous hemorrhage. He was diagnosed with AHA in hepatocellular carcinoma on CT imaging, and treated with rituximab alone. Hemostasis was achieved for both patients without bypassing agents as the amount of inhibitors was reduced and eradicated. However, both patients died within 1 yeardue to cancer progression. Successful treatment for AHA in solid cancer can be diffcult because treatment of the underlying malignancy is also required.展开更多
基金Supported by Traditional Chinese Medicine Research Program of Hebei Provincial Administration of Traditional Chinese Medicine,No.2025313 and No.2025448.
文摘BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated with autoimmune diseases,cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid(BP) is a chronic autoimmune subepidermal blistering disease associatedwith tissue-bound and circulating autoantibodies against BP antigens 180 (BP180)and 230 (BP230). AHA-associated BP has a high mortality rate;hence, the understandingof this disease must improve.CASE SUMMARY A 69-year-old man presented with erythema, blisters, blood blisters, and crustsaccompanied by severe pruritus for more than 20 days, and ecchymosis andswelling on his left upper arm for 3 days. Pathological examination revealed asubepidermal blister that contained eosinophils. Laboratory tests showed that theBP180 autoantibody levels had increased, isolated activated partial thromboplastintime was notably prolonged (115.6 s), and coagulation FⅧ activity wasextremely low (< 1.0%). Furthermore, the FⅧ inhibitor titer had greatlyincreased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospitalfor effective treatment;however, he died after 2 days.CONCLUSION AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effectivetreatment are necessary.
文摘BACKGROUND Acquired hemophilia is rare.In some cases,the bleeding in muscle causes compartment syndrome.However,it is not clear whether fasciotomy should be performed for the compartment syndrome caused by acquired hemophilia because of the risk of bleeding and the unknown functional results.CASE SUMMARY A 75-year-old woman was admitted with severe pain of the right forearm with no preceding traumatic event.The right forearm was obviously swollen,and stretch pain was observed.Subcutaneous hematomas were suspected in various parts of the body.Compartment pressure was 110 mmHg on the volar side.Activated partial thromboplastin time(aPTT)was prolonged to 54.9 s.Fasciotomy was performed,and hematoma was observed in the volar compartment.Postoperative laboratory examinations revealed a low level of factor Ⅷ(FⅧ)activity(12.5%)and a high level of FⅧ inhibitor(15.2 bethesda units/mL).Acquired hemophilia A was diagnosed.Though recombinant clotting factors were administered,transfusion of red blood cells reached 46 units(140 mL/unit).Hemostasis was achieved 9 d after fasciotomy.The total cost of the clotting factor concentrates administered reached 28834600 yen.With prednisolone,FⅧ activity and aPTT recovered gradually.Final function of the hand was good in the index finger and excellent in the others.CONCLUSION Fasciotomy resulted in good function of the hand in a case of non-traumatic compartment syndrome caused by acquired hemophilia,but life-threatening bleeding occurred,and the cost of clotting factor treatment was high.Preparation of sufficient blood transfusion,preoperative administration of recombinant activated clotting factor Ⅶ,and prompt fasciotomy could be ideal for such cases.
文摘Acquired hemophilia A （AHA） is a rare, hemorrhagic autoimmune disease, whose pathogenesis involves reduced coagulation factor Ⅷ （FⅧ） activity related to the appearance of inhibitors against FⅧ. Common etiological factors include autoimmune diseases, ma-lignancy, and pregnancy. We report two cases of AHA in solid cancer. The first case is a 63-year-old man who developed peritoneal and intestinal bleeding after gastrectomy for gastric cancer. He was diagnosed with AHA, and was treated with prednisone, followed by cyclophosphamide. In the second case, a 68-year-old man developed a subcutaneous hemorrhage. He was diagnosed with AHA in hepatocellular carcinoma on CT imaging, and treated with rituximab alone. Hemostasis was achieved for both patients without bypassing agents as the amount of inhibitors was reduced and eradicated. However, both patients died within 1 yeardue to cancer progression. Successful treatment for AHA in solid cancer can be diffcult because treatment of the underlying malignancy is also required.
