A 32-year-old primigravid woman developed ACQUIRED factor VIII inhibitor after delivery. She presented with postpartum hemorrhage and large hematoma in episiotomy site. Laboratory examinations showed markedly prolonged activated partial thromboplastin time, low levels of factor VIII (8%), and factor VIII inhibitor (2 Bethesda units). The bleeding was controlled successfully using combined treatment with factor VIII, intravenous immunoglobulin, steroids, and recombinant factor VIIa. Six months after delivery, factor VIII inhibitor was not present and factor VIII concentration increased to normal range.ACQUIRED HEMOPHILIA is a life-threatening disorder. Precise screening of coagulation factors is essential for diagnosis of persisting postpartum hemorrhage.

ACQUIRED HEMOPHILIA (AHA) is a relatively rare disease that occurs in patients with no previous family history of HEMOPHILIA. The spontaneous development of autoantibodies (IgG1 and IgG4) against factor VIII has been reported as the most probable cause of AHA. AHA has been reported in association with other conditions, including some autoimmune bullous skin diseases, such as bullous pemphigoid, pemphigus vulgaris, and pemphigus foliaceous. To the best of our knowledge, only 21 cases of AHA with skin autoimmune bullous diseases have been reported so far. Herein, we report a 63-year-old male with a previous history of pemphigus vulgaris who developed large ecchymotic areas on his lower abdomen and forearms after the second infusion of rituximab. Based on coagulation factors evaluation, he was diagnosed with AHA. Treatment with factor VII led to the improvement in his coagulation status, but unfortunately, he passed away because of inferior wall myocardial infarction four days later.

ACQUIRED HEMOPHILIA is a rare condition, and is due to the production of auto antibodies in adult life which inactivate factor VIII. The use of high-dose rFVIIa (270 m/kg) in some clinical trials and registries, appears safe, when use for congenital and ACQUIRED haemophilia.A 59-year-old man with a 3-year history of GI Bleeding was admitted to the Shahid Ghazi /Tabriz hospital. He has no family medical history of HEMOPHILIA or any other bleeding disorders and he has history of several episodes of GI Bleeding (Recurrent GIB).After treatment with several protocols for Gastritis and Ulcer and H.pylori Infection and transfusion therapies (including packed red blood cells, fresh frozen plasma, and cryoprecipitate) and administration of human antihemophilic globulin, corticosteroid, and cyclophosphamide bleeding was not still stopped. He was diagnosed with ACQUIRED factor VIII (FVIII) deficiency based on the laboratory examination: Factor VIIIc=20%, Inhibitor to F VIII=0.74 BU, Mixed aPTT=83.9 Sec. At the time of his last admission to our hospital, he has a history of Melena and Anemia and Malaise.Lab data were Hb=5.6 g/dl, Hct=23.5%. Because the patient has recently undergone transfusion of blood products, Factor VIII Inhibitor and Factor assays with apparent lupus inhibitor interference and Indirect Coomb's Negative has been reported for him. He was immediately given rFVIIa, initially dosed 90 m/kg every 3 hours for 2 days but hemostasis was still not achieved. An effective response to treatment was observed by using of single dose therapy of rFVIIa as 270mg/kg, for 2 days. The GI bleeding of the patient was slowing immediately and complete hemostasis was achieved within 24 hours. No serious adverse events were reported.

ACQUIRED HEMOPHILIA is a rare but serious bleeding disorder characterized by the development of autoantibodies (inhibitors) against plasma coagulation factors, most commonly FVIII. It classically presents with the sudden onset of bleeding symptoms in a patient with no past or family history of bleeding disorder. It is thought to be exceedingly rare in the pediatric population with an estimated annual incidence to be 0.045 per million. Pediatric ACQUIRED HEMOPHILIA has been described in association with autoimmune conditions, infection and antibiotics, most commonly penicillin- like antibiotics. Unlike classical HEMOPHILIA where hemarthrosis is the characteristic bleeding manifestation, most patients with ACQUIRED HEMOPHILIA present with bleeding into the skin, subcutaneous tissue and muscles, hematuria, hematemesis or melena and postoperative bleeding. Severe subcutaneous bleedings following venipuncture and intramuscular injections have been described.

Background and Objectives: HEMOPHILIA is a hereditary bleeding disorder. It is divided to HEMOPHILIA A, B and C depending on deficiency of clotting factors involved. HEMOPHILIA A is due to deficiency of factor VIII, HEMOPHILIA B is result of factor IX deficiency and HEMOPHILIA C is due to factor XI deficiency. Genetic abnormality resides in chromosome X in 80% of the cases. The women are carriers only, while men experience full picture of the disease. Materials and Methods: In this study, experiments were carried out on 241 patients. All patients with coagulation deficiency, who were referred to Shahid Gazi Hospital, were examined by imaging technique. In patients with joint hemorrhage, plain films were taken, however when extra joint hemorrhage were seen, CT scan or ultrasonography were techniques of choice. In some cases all of these techniques were performed. Results: We report radiological findings observed in 241 patients. Of these, 192 were HEMOPHILIAcs, 164 suffering from HEMOPHILIA A, and 28 from HEMOPHILIA B. Knee joint was involved in 160, ankle in 62, elbow in 28, wrist in 34, shoulder in 26 and hip in 29 patients. Pseudo tumor was seen in 3 patients. Conclusion: Sometimes death may results from hemorrhage. Treatment is mandatory, but treatment with factor or frozen serum is very expensive. Because HEMOPHILIA is X linked, birth control in these patients is very important. Although the prevention of child bring in carriers and patients seem to be difficult by instruction, instruction can be helpful in preventing birth of male infants.    

HEMOPHILIA is a congenital X-linked hemorrhagic disorder. Its most frequent sign is an uncontrollable and prolonged bleeding, that results from lack or absence of some coagulation factors in the blood, Hemorrhage can occur even without any obvious cause. This bleeding often happens in joints. The symptoms include: 1. Bruising: the bruise resulted from little injuries that can result in a big hematoma, and 2.Bleeding: nose bleeding or bleeding in the mouth or gingiva, teeth brushing, bleeding after a minor cut and or dentistry operations; Joints are one of the common parts affected by bleeding in hemophilic individuals; Bleeding in the joints can make the joints swollen and painful. It also causes loss in the joints movements, deformities and resulting staggering and possible joints deformity; Bleeding inside the muscles makes the muscles swollen, hot to touch and painful. Swollenness resulted from bleeding in these areas can cause prolonged injuries and deformation through increasing the pressure on the tissues and the nerves. This video clip demonstrates a hemophilic patient and related signs and symptoms.

The development of inhibitors against administered clotting factors may render replacement therapy ineffective for some HEMOPHILIA patients. Such patients are therefore at the highest risk of developing arthropathy. Elective orthopedic surgery (EOS) in hemophilic patients having such inhibitors remains a rare, expensive, and difficult surgery, whose management represents a significant challenge. We report the case of a 35-year-old man with a severe form of HEMOPHILIA A (factor VIII<1%), who was suffering from repetitive spontaneous hemarthrosis, especially in his knee joints that had consequently become more susceptible to bleeding. The patient had a history of high levels of factor VIII inhibitor (>5.0 Bethesda Unit [BU]/ml) as shown by the factor VIII inhibitor assay; therefore, we began treatment with factor VIIa for his mildto- moderate bleeding (90 mg/kg intravenous bolus injections). The interval between injections varied with the severity of the hemorrhage in each bleeding episode. The inhibitor level reduced to 3.1 BU/ml after three months, to 1.6 BU/ml after six months, and disappeared completely after one year of treatment. We administered factor VIII at a dose of 50 IU/kg every eight hours during the first three post-operative days, then continued administration with a dose of 40 IU/kg every 12 hours for another four days, and observed a very good response to treatment with no bleeding. Recombinant activated factor VII (rFVIIa) is not an inhibitor-removal strategy, but an inhibitor-bypassing product. However, in our patient, the treatment of mild-to-moderate bleeding with short-term use of rFVIIa and no exposure to factor VIII caused a gradual reduction in the inhibitor level over a period of 1 year.