1/24. Spontaneous isolated lesser sac hematoma in a patient with hemophilia.In patients with hemophilia, hematomas in the mesentery and bowel wall have been described uncommonly. The lesser sac is a rare site of spontaneous hemorrhage even in patients with bleeding diathesis; only a single case of isolated lesser sac hemorrhage has been reported in a hemophiliac patient. We report a similar case with no history of trauma. He recovered with administration of factor viii concentrate.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
2/24. Long-lasting remission and successful treatment of acquired factor viii inhibitors using cyclophosphamide in a patient with systemic lupus erythematosus.Acquired deficiency of clotting factor viii (FVIII) is a rare bleeding diathesis seldom encountered in systemic lupus erythematosus (SLE). Reduction of FVIII activity by autoantibodies can cause potentially life-threatening situations. Herein, an SLE patient with a positive lupus anticoagulant (LAC) test who abruptly developed metrorrhagia 4 yr after diagnosis is reported. Coagulation tests revealed FVIII activity reduced to 3% and a prolonged aPTT. FVIII inhibitor(s) were found to be as high as 3.0 Bethesda Units. plasmapheresis, immunoglobulins, prednisolone and FVIII plasma concentrates induced the cessation of metrorrhagia, but the clotting tests were barely improved. One month later, extensive ecchymosis appeared and worsened, despite re-administration of the previous therapy. pulse cyclophosphamide followed by oral administration was then started with normalization of coagulation parameters and long-lasting disease remission.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
3/24. Surgery-associated factor viii inhibitors in patients without hemophilia.The acquisition of antibodies to factor viii (ie, factor viii protein) by patients without hemophilia is associated with conditions such as pregnancy, lymphoma, and autoimmune disorders. We present three patients who acquired factor viii antibodies in the postoperative setting. Preoperatively, none gave a history of bleeding even with past surgeries and all had normal coagulation tests. Within days of surgery (bowel resection, cholecystectomy, coronary bypass), a bleeding diathesis emerged with prolonged partial thromboplastin time, decreased factor viii levels, and demonstrable factor viii antibodies. All patients required multiple blood transfusions and urgent reexploration for hemostatic control. All were treated with high dose human factor viii infusions, corticosteroids, and various forms of immunosuppression. We wish to raise awareness of surgery as a precipitating cause of acquired hemophilia, which is something to be considered with unusual postoperative bleeding. This syndrome is remarkable for its abrupt onset within days of surgery, dramatic bleeding, subsequent persistence, but potential reversal by immunosuppression.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
4/24. Acquired hemophilia masked by warfarin therapy.People without hemophilia but with autoantibodies specifically directed against the procoagulant activity of factor viii are known to have acquired hemophilia. The bleeding diathesis in these patients is often severe and life-threatening. The definite laboratory diagnosis of this disorder includes demonstration of low factor viii levels in plasma with a high titer of factor viii inhibitors, but the initial suspicion for its presence should rise in view of a prolonged partial thromboblastin time (PTT) and a normal prothrombin time associated with an acquired bleeding disorder. Oral anticoagulant treatment is known to prolong PTT as well, and the merger of these 2 situations may cause delayed diagnosis of acquired hemophilia with devastating consequences. We describe here the first reported case of acquired hemophilia diagnosed in a patient treated with warfarin. In such patients prolonged PTT may be ascribed to warfarin therapy rather than to acquired hemophilia, thus causing a dangerous delay in diagnosis.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
5/24. Bell's palsy during interferon therapy for chronic hepatitis c infection in patients with haemorrhagic disorders.Two adult patients with life-long severe haemorrhagic disorders commenced on interferon-alpha2b therapy for chronic hepatitis c infection. Both developed Bell's palsy several weeks after commencing therapy, They were started on steroids and, in addition, the first patient discontinued interferon-alpha2b therapy while the second patient elected to continue with therapy. In both cases facial paralysis improved over the ensuing weeks. Bell's palsy is often idiopathic but has been reported. in association with herpesviruses. It is not a recognised complication of chronic hepatitis b or C infection, or interferon-alpha2b therapy. However, the interferons are associated with numerous adverse reactions including various neuropsychiatric manifestations and neurological syndromes. There are several reports of nerve palsies, including optic tract neuropathy, occurring during interferon therapy, and immune-based mechanisms are thought to play a role in the aetiopathogenesis. No reports of Bell's palsy in association with interferon therapy were identified in our literature search, although one possible case has been reported to the Committee of safety in medicine. Although Bell's palsy in our patients may have occurred by chance, a neuropathic effect of interferon-alpha2b on the facial nerve cannot be excluded and we urge physicians using interferons to be aware of this potential side-effect.- - - - - - - - - - ranking = 2059.9849160388keywords = haemorrhagic disorder (Clic here for more details about this article) |
6/24. Diagnosis of hemophilia made after intraoperative bleeding during attempted penetrating keratoplasty in an elderly patient.PURPOSE: To report an unusual case where the diagnosis of hemophilia was made after attempted penetrating keratoplasty in an elderly patient. methods: A 75 year old white male with a full-thickness corneal scar in the visual axis and a visually significant cataract OD was to undergo penetrating keratoplasty and cataract extraction with lens implantation for visual rehabilitation. There was no history of bleeding diathesis given. RESULTS: During placement of the Flieringa ring, a progressively enlarging 360 degrees subconjunctival hemorrhage was observed. Given the unusual bleeding,the procedure was aborted and the patientwas referred to the hematology service for further evaluation. Laboratory studies revealed a diagnosis of atypical hemophilia of mild severity. CONCLUSIONS: The initial diagnosis of hemophilia in any elderly patient is unusual. This case is even more unusual asthe diagnosis of a bleeding disorder was first considered after excessive subconjunctival hemorrhage developed during attempted penetrating keratoplasty.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
7/24. Successful treatment of patients with rheumatic disorders and acquired factor viii inhibitors with cyclophosphamide and prednisolone combination therapy: two case reports.Acquired haemophilia associated with autoimmune disorders can be fatal and has been reported to be refractory to steroid therapy alone. We report two cases of female patients, aged 24 years and 54 years, with acquired haemophilia caused by factor viii inhibitors. Underlying diseases were systemic lupus erythematosus in the 24-year-old patient and rheumatoid arthritis in the 54-year-old patient. Both conditions were nearly quiescent when the patients manifested haemorrhagic diathesis. In response to combination therapy with prednisolone and cyclophosphamide, coagulation abnormalities were resolved together with complete elimination of factor viii inhibitors in both patients. Thus, combination therapy with alkylating agents may be recommended as initial therapy for the management of autoimmune patients with factor viii inhibitors.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
8/24. Acquired hemophilia a presenting as a bleeding diathesis in a postpartum patient: diagnosis and management.Antibodies against factor viii (FVIII) can develop in non-hemophiliac patients, causing the rare condition of acquired hemophilia. In 7.3% of the patients, the FVIII inhibitors appear either during pregnancy or in the postpartum period. In this case report, we present a non-hemophiliac patient, who presented five months postpartum with intermittent heavy vaginal bleeding, easy bruising, hemarthrosis, and recurrent frank hematuria. The woman presented to the emergency room with hematuria. Coagulation screening tests showed a prolonged APTT. Using a standard diagnostic algorithm, a factor viii inhibitor was detected. The treatment, cause of disease, and prognosis of this woman is presented in this paper as well as a literature review of acquired hemophilia a associated with pregnancy.- - - - - - - - - - ranking = 4keywords = diathesis (Clic here for more details about this article) |
9/24. Two patients with haemophilia and acute leukaemia.Acute leukaemia is the commonest form of malignancy in childhood. The coincidental development of leukaemia in children or adults with haemophilia is extremely rare, although cases of leukaemia and other malignancies have been reported previously in hiv-positive subjects. Of a total of 440 people with haemophilia registered with our society, two were diagnosed with acute leukaemia last year. The development of leukaemia in a subject with haemophilia has previously been reported from our country in 1985, but the negative hiv status of these recent cases is very interesting. The first case involved a 14-year-old boy with moderate haemophilia A, who developed acute lymphoblastic leukaemia (ALL) [French-American-British (FAB) classification L2]. The second subject was a 16-year-old boy who had moderately severe haemophilia A with no previous family history, and developed acute nonlymphocytic (myelomonocytic) leukaemia (FAB-M4). Both patients received conventional chemotherapy and this report discusses the potential problems in management of such cases, including diagnosis and administration of chemotherapy in subjects with a pre-existing haemorrhagic disorder. Extensive cutaneous and mucosal bleeding, as well as bleeds in joints previously affected by haemarthrosis and alterations of haematological values were all initially suggestive of the development of inhibitors against factor viii, but the appearance of blasts in the peripheral blood and bone marrow led to the definitive diagnosis. The risk of bleeding, due to the combination of both leukaemia and the consequences of the chemotherapy, was overcome by the administration of coagulation factor concentrates (daily initially followed by prophylactic doses after successful induction of remission in both patients). The young patient with ALL is now receiving the maintenance phase of the Children's Cancer Study Group 1961 protocol and is in the 15th month of follow-up, without any complications. The other case relapsed in the seventh month, developing enterobacter sepsis, and died. An important lesson to be learnt from these cases is that the possible diagnosis of leukaemia should not be overlooked in a patient with haemophilia and severe haemorrhagic problems, if the first-line differential diagnosis of inhibitor development against factor viii (or IX) has been excluded.- - - - - - - - - - ranking = 411.99698320776keywords = haemorrhagic disorder (Clic here for more details about this article) |
10/24. Management of pregnancy in a patient with severe haemophilia A.Haemophilia A is a bleeding disorder that has a spectrum of manifestations ranging from persistent bleeding after minor trauma to spontaneous haemorrhage. As an X-linked disease, it has a rare occurrence in females. We report a case of a pregnant patient with severe haemophilia A, who received epidural analgesia during labour. The prepartum, intrapartum and postpartum care of a patient with such a bleeding diathesis is discussed.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
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