1/205. Transient hypoplastic anemia caused by primary human parvovirus B19 infection in a previously untreated patient with hemophilia transfused with a plasma-derived, monoclonal antibody-purified factor viii concentrate.BACKGROUND: Modern plasma-derived clotting factor concentrates are produced using various virus-inactivation protocols and are assumed to be safer than they were previously with regard to the risk for transmitting viral infections such as human immunodeficiency virus, hepatitis b, and hepatitis c. The risks from viruses that are relatively resistant to the current inactivation procedures remain uncertain. PATIENT: A 7-year-old with mild hemophilia a who had not been previously infused with any blood products was treated with a plasma-derived, monoclonal antibody-purified factor viii concentrate to cover orthopedic surgery after traumatic fracture of his left arm. RESULTS: A typical primary human parvovirus (HPV)-B19 infection was observed associated with transient hypoplastic anemia. retrospective studies including serologic examination and polymerase chain reaction analysis confirmed that the HPV-B19 infection was transmitted by the factor viii concentrate. CONCLUSIONS: Clotting factor concentrates for the treatment of hemophilia retain a risk for HPV-B19 contamination. HPV-B19 viral infection might induce hypoplastic anemia in these patients, particularly during enhanced hemopoiesis after acute blood loss.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
2/205. Management of liver failure in a haemophilic patient co-infected with human immunodeficiency and hepatitis c viruses.We present a case of liver failure in a haemophilic patient coinfected with transfusion acquired human immunodeficiency (HIV) and hepatitis c (HCV) viruses. The case illustrates the interaction of multiple viruses with accelerated progression to end stage liver disease and ultimately death. We report the impact on the patient management of two liver biopsies, which diagnosed an initial drug induced hepatitis and subsequently an atypical HCV related hepatitis.- - - - - - - - - - ranking = 5keywords = deficiency (Clic here for more details about this article) |
3/205. Characteristics of septic arthritis in human immunodeficiency virus-infected haemophiliacs versus other risk groups.The cases are presented of four haemophiliacs infected with human immunodeficiency virus (HIV) and with septic arthritis among the 340 patients followed at our centre. The data of these cases and 39 additional HIV-infected haemophiliacs with septic arthritis, identified in a literature search, are reviewed. The spectrum of bacterial pathogens is limited and somewhat different from that in other risk groups. The localization is exclusively to joints affected by haemophilic arthropathy. The laboratory picture is characterized by the absence of peripheral leucocytosis, varying CD4-helper cell counts, a high erythrocyte sedimentation rate and fever. The clinical picture mimics that of haemarthrosis, often causing a delay in diagnosis. Treatment with systemic antibiotics is often sufficient, obviating the need for arthrotomy and open drainage. prognosis related to the joint function is relatively good, but poor when related to the medium- to long-term survival of the patient.- - - - - - - - - - ranking = 5keywords = deficiency (Clic here for more details about this article) |
4/205. 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 = deficiency (Clic here for more details about this article) |
5/205. Manifestations of factor viii inhibitor in the head and neck.hemophilia a is the most severe of the inherited bleeding disorders. Otolaryngologists are frequently asked to assist in the care of a patient with this disorder who has intractable bleeding from sites in the head and neck. This disorder is transmitted in an X-linked recessive manner and results from factor viii deficiency. Factor replacement therapy has contributed much to the management of acute bleeding episodes and the prevention of long-term sequelae in patients with hemophilia. A consequence of factor replacement therapy that occurs in as much as 16% of patients is the development of antibodies to the exogenous factor. These antibodies inactivate any supplemental factor viii, resulting in continued bleeding despite attempts at replacement therapy. This poses an extremely challenging clinical problem because other standard therapies have had limited usefulness. Activated prothrombin complex has been shown to be efficacious in the treatment of patients with inhibitors. This has led to the production of factor viii inhibitor bypassing activity (FEIBA), which contains activated forms of factors II, VII, IX, and X. The mechanism of action of this preparation remains unknown, but it seems to bypass the need for factor viii in the clotting cascade. factor viii inhibitor bypassing activity is not as effective as factor viii concentrates in patients with normally responsive hemophilia, and patients with low inhibitor levels may be given larger doses of factor viii. disseminated intravascular coagulation is most often mentioned as a potential complication of the administration of factor viii inhibitor bypassing activity, although it has been rarely reported. Otolaryngologists should be familiar with this clinical phenomenon and understand its implications. Two patients with bleeding diatheses were recently treated at our institution.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
6/205. Nasal haemophilic pseudotumour.Haemophilic pseudotumour or haemophilic cyst is a rare complication of haemophilia, occurring in one to two per cent of individuals with a severe factor viii or IX deficiency. We report a case of a haemophilic pseudotumour in a hitherto unreported site, the bony nasal pyramid, and believe this case is also unique on account of it having occurred in a patient with mild haemophilia. The diagnosis and treatment of this rare condition is also reviewed.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
7/205. Intraosseous hematoma in a newborn with factor viii deficiency.We present an unusual case of an intraosseous hematoma in a newborn with a known bleeding disorder. This cephalohematoma was diagnosed shortly after birth, was entirely within the bony skull, and was in fact determined to be an intraosseous hematoma. The initial CT scans showed the unusual appearance and location of the lesion; later scans showed a significant amount of remodeling, with resolution of the hematoma. Although the coagulopathic diagnosis was independent of this finding, a bleeding disorder might be considered in other patients with similar CT findings.- - - - - - - - - - ranking = 4keywords = deficiency (Clic here for more details about this article) |
8/205. Septic arthritis in hemophilia with central venous catheter: a case report.A case is reported of septic arthritis in a child with human immunodeficiency virus-negative hemophilia a associated with a staphylococcus aureus catheter-associated septicemia. The infection occurred in relation to the use of a totally implantable central venous catheter. The organism was eventually eradicated with antibiotics injected via the catheter. With increasing use of such catheters in the hemophilic population, clinicians should be alerted to the possibility of septic arthritis for prompt diagnosis and treatment.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
9/205. A case of hemophilia a associated with Hodgkin's disease.Lymphoreticular malignancies are more common in patients with hemophilia, but it is usually attributed to human immunodeficiency virus (HIV) infection associated with repeated use of blood products. However, there are a couple of hemophiliac patients with malignancies but without HIV infection in the literature. We report a case of a hemophiliac patients who had Hodgkin's disease at 2.3 years old without any congenital or acquired immunodeficiency and without use of any blood products. This patient showed that malignancy can develop in hemophiliacs without HIV infection, but further studies are needed to clarify whether hemophiliacs are more susceptible to malignancies.- - - - - - - - - - ranking = 2keywords = deficiency (Clic here for more details about this article) |
10/205. cardiac tamponade due to post-cardiac injury syndrome in a patient with severe haemophilia A and hiv-1 infection.An 18-year-old man with severe haemophilia A (FVIII:C < 1%) and human immunodeficiency virus 1 (hiv-1) infection was admitted to the hospital with fever and chest pain for 7 days. Eight weeks prior to his admission he had an accident for which he underwent, at another hospital, clinical and laboratory examination that revealed bone fractures of the nose cavity, and he was given factor viii concentrates for seven days due to nasal bleeding. On admission, chest roentgenogram showed a large cardiac silhouette and echocardiography confirmed the presence of a large quantity of pericardial fluid. A presumptive diagnosis of the post-cardiac injury syndrome was made and he was given anti-inflammatory drugs plus infusion of recombinant factor viii concentrate (35 units kg-1 b.i.d.). On the seventh day he exhibited cardiac tamponade for which he underwent subxiphoid pericardiotomy with drainage of approximately 1500 mL of bloody exudate. He had an uncomplicated recovery and 10 days later he left hospital. He was given a continuous prophylactic treatment of 15 units kg-1 of recombinant FVIII every 2 days for 6 months, and 30 months after this episode the patient is free of any symptom.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
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