Female ; Hemophilia A ; complications ; Humans ; Middle Aged ; Myasthenia Gravis ; complications

Adult ; Hemophilia A ; complications ; Humans ; Leukemia, Promyelocytic, Acute ; complications ; Male

Aged ; Female ; Hemophilia A ; complications ; Hemorrhage ; etiology ; Humans ; Mediastinal Diseases ; etiology

Adult ; Compartment Syndromes ; etiology ; Hemophilia A ; complications ; Humans ; Leg ; Male

Hemophilia is an X-linked recessive inherited bleeding disorder. Despite the improved treatment in recent years with the advent of replacement therapies, the progression of atherosclerosis is not slowed down after the reduction of clotting factors in hemophilia. As life expectancy increases, more hemophilia patients will suffer from age-related cardiovascular diseases. Since cardiac surgery needs heparinization and cardiopulmonary bypass (CPB), it is extremely challenging to balance hemostasis and coagulation in patients with hemophilia. Here we report three cases of hemophilia patients who underwent cardiac surgery successfully.
Cardiac Surgical Procedures/adverse effects* ; Cardiopulmonary Bypass ; Hemophilia A/complications* ; Humans

Surgical treatment is the main treatment for hemophilia arthritis, including synoviectomy, joint replacement and joint fusion. Synoviectomy is suitable for early hemophilia synovitis, and is divided into radiation, chemical, arthroscopy, and open operation. Radionuclides were recommended as the first choice due to its positive efficacy and less side effects, but exsit some problems such as scarcity of nuclides. Chemical synoviectomy is cheap and easy to operate, which is suitable for developing countriesm, while mutiple doses and pain after injection are main fault. Synoviectomy under arthroscope has a significant effect on the advanced lesion, but has a higher surgical risk. Open surgery with severe trauma and postoperative joint stiffness, is rarely performed. Joint replacement could effectively improve range of motion in advanced patients and is suitable for joints with high range of motion. Arthrodesis are effective in improving symptoms but lead to loss of range of motion and are suitable for joints with low range of motion. Operation for hemophilia arthritis has some problems, such as single operation, untimely diagnosis and treatment in early stage, and unsatisfactory curative effect in late stage. In addition, the treatment of hemophilia arthritis should focus on the early treatment, the formation of the whole process, the system of individual treatment concept.
Arthrodesis ; Hemophilia A/complications* ; Humans ; Joint Diseases ; Synovectomy ; Synovitis ; Treatment Outcome

Aged, 80 and over ; Dyspnea ; etiology ; Glottis ; pathology ; Hematoma ; complications ; Hemophilia A ; complications ; Humans ; Laryngeal Diseases ; complications ; Male

We report a case of hemophilic pseudotumor in the ulna of a 6-year-old boy treated with radiation therapy. A total dose of 900 cGy in 6 fractions was given in 6 consecutive days. Progression of cystic changes was halted within a month. New bone formation and trabeculation were found on the 4th month. Complete healing of the lesion and bony replacement were found on the 12th month. The patient was followed up to 72 months and there was no evidence of recurrence and no bone growth disturbance. Radiation therapy can be an effective alternative modality in treating hemophilic pseudotumor.
Bone Cysts/radiotherapy* ; Bone Cysts/pathology ; Bone Cysts/etiology* ; Case Report ; Child ; Hemophilia A/pathology ; Hemophilia A/complications* ; Human ; Male ; Ulna/pathology*

Systemic lupus erythematosus (SLE) complicated with acquired hemophilia A (AHA) is a rare condition with frequently delayed diagnosis and a high mortality rate, so it is necessary to strengthen the understanding of this disease. In this study, the characteristics and treatment in 1 case of SLE complicated by AHA is reported and analyzed, and a literature review is conducted. The patient was a 29-year-old young female with a 10-year history of SLE, the main clinical manifestation was severe abdominal bleeding. Laboratory tests revealed that the activated partial thromboplastin time (APTT) was notably prolonged (118.20 s), and the coagulation factor VIII activity (FVIII꞉C) was extremely decreased (0.20%) with high-titer of factor VIII (FVIII) inhibitor (31.2 BU/mL). After treating with high-dose glucocorticoid, immunoglobulin, cyclophosphamide, rituximab, blood transfusion, and intravenous infusion of human coagulation FVIII, the coagulation function and coagulation FVIII꞉C were improved, and FVIII inhibitor was negative without serious adverse reactions. During the next 5-year follow-up, the patient's condition was stable and no bleeding occurred. In the case of coagulation dysfunction in SLE, especially with isolated APTT prolongation, AHA should be screened. When the therapeutic effects of glucocorticoid combined with immunosuppressants are not desirable, rituximab could be introduced.
Female ; Humans ; Adult ; Hemophilia A/therapy* ; Rituximab ; Glucocorticoids ; Factor VIII ; Lupus Erythematosus, Systemic/complications* ; Hemorrhage/complications*

Acquired hemophilia A (AHA) is anunusual disease resulting from autoantibodies (inhibitors) against coagulation factor VIII (FVIII) and clinically manifests as bleeding, which sometimes can cause potentially limb-threatening or life-threatening situations. AHA is associated with cancers, auto-immune disorders, infections, dermatologic conditions and certain medications, among which it is commonly secondary to multiple rheumatologic conditions, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), pollymyositis, autoimmune hemolytic anemia and undifferentiated connective tissue disease. In autoimmune diseases, it may be the result of autoantibody producing against FVIII, and some cases of AHA may act as the first manifestation of SLE. AHA should be suspected in patients who have spontaneously hemorrhagic events with an isolated prolonged activated partial thromboplastin time (APTT), reduced FVIII activity and a negative lupus anticoagulant (LA). When FVIII inhibitor is found, it can be diagnosed. The management of AHA focuses on the following goals: (1) controlling and preventing bleeding, (2) eradication of the inhibitor, (3) treatment of the underlying disease. Here, a case of AHA in a patient with lupus is reported. A 53-year-old man with a 4-year history of SLE developed arthralgia and ecchymotic skin lesions after arthrocentesis of knee joint. Ultrasound confirmed the presence of an intramuscular hematoma. Coagulation tests revealed that FVIII activity reduced to 1% and a prolonged APTT (92.2 s), FVIII inhibitors were found to be as high as 60.0 Bethesda Units. Initial treatments with methylprednisolone 200 mg/d were started but new hemorrhagic manifestation occurred and hisbiological indexes were not good. Then the patient was treated with intravenous pulse corticosteroids (methylprednisolone 500 mg/d), intravenous cyclophosphamide, and also plasma and prothrombin complex infusion. Subsequently, FVIII activity returned within normal ranges, FVIII inhibitors decreased and clinical improvement was significantly obtained. The patient's condition kept stable till now.Hemorrhagic events due to production of antibodies directed against coagulation factors were rarely observed in SLE and attentions should be paid to the association between SLE and AHA.Bypass treatment was considered as the immediate antihemorrhagic treatment. Corticosteroid combined with immunosuppressor was recommended as the main therapy to eradicate the inhibitors. However we still lack the therapeutic guidelines and standardized treatment in patients of AHA with SLE at present.
Autoantibodies ; Hemophilia A/complications* ; Humans ; Lupus Erythematosus, Systemic/complications* ; Male ; Middle Aged ; Partial Thromboplastin Time