1/933. The management of a person with haemophilia who has a fixed flexed hip and intractable pain. The clinical picture of a fixed flexed hip associated with pain in a person with haemophilia is suggestive of a haemorrhage in that area. Sonography facilitates differentiation between a haemarthrosis, intraperitoneal haemorrhage, subperiosteal bleed, a bleed into the soft tissue around the hip joint or a psoas haematoma. All these aforementioned causes may result in the same clinical presentation. Two cases are described in which coxhaemarthrosis resulted in a flexion contracture of the joint associated with severe intractable pain. Narcotic drugs failed to alleviate the severe pain. Joint aspiration produced dramatic pain relief and early joint rehabilitation. ( info) |
2/933. Management of a premature infant with moderate haemophilia A using recombinant factor viii. The birth of a very premature infant with haemophilia A is a rare event. In this case report the problems posed in the management of a child with a factor viii level of 0.03 IU mL-1 born at 28 weeks of gestation and weighing 1590 g are considered. The value of recombinant factor viii, the pharmacokinetics of factor VIII in this situation and the importance of close cooperation between paediatricians and haematologists are discussed. ( info) |
3/933. 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. ( info) |
4/933. 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. ( info) |
5/933. Treatment with recombinant activated factor VII in a patient with hemophilia a and an inhibitor: advantages of administration by continuous infusion over bolus intermittent injections. Recent studies have shown that treatment with a continuous infusion of recombinant activated factor VII (rFVIIa) is far more convenient than administration by bolus intermittent injections and may allow a substantial reduction in the dose. We present the case of a 26-year-old patient with hemophilia a, who had a high-titer inhibitor to both human and porcine factor viii, and who had recently been admitted to hospital because of a bilateral severe ilio-psoas hematoma. Two subsequent courses of treatment with rFVIIa by bolus intermittent injection showed only a partial efficacy. A further administration of rFVIIa was therefore carried out using a continuous infusion regimen that proved to be fully efficacious. During the continuous infusion course levels of factor VII coagulant activity were in the range 18.2-5.2 U/ml, while the prothrombin time, expressed as an international normalized ratio, remained within the range 0.57-0.71. The continuous infusion, compared with the administration of the bolus intermittent infusion, reduced the amount of rFVIIa required by approximately 40-50%. Statistical analysis demonstrated that there was a strong positive correlation between the rate of infusion of rFVIIa and levels of factor VII coagulant activity (r = 0.941; P < 0.001), and a very significant negative correlation between levels of factor VII coagulant activity and prothrombin time values (r = -0.897; P < 0.001). In accordance with previous findings, our experience confirms that, when prolonged therapy is required, treatment with rFVIIa by continuous infusion is more convenient than administration of bolus intermittent injections, and may allow the saving of a large amount of drug. Moreover, we suggest potential additional advantages of the continuous infusion regimen over bolus intermittent injections, such as a better efficacy and a stronger correlation between prothrombin time and factor VII coagulant activity levels. ( info) |
6/933. Central venous catheter-associated thrombosis in severe haemophilia. Significant subclavian vein thromboses associated with indwelling fully implanted (port-a-cath) devices are described in two boys with severe haemophilia A and factor viii inhibitors. Investigations were prompted by prominent chest wall veins in one case, whereas the thrombosis was a chance finding in the other case during investigation of mechanical dislocation of the catheter tubing. Extensive collateral venous circulations were demonstrated by venography in both instances indicating that the thrombus had been present for some time. Possible contributing factors to the thromboses included desensitization therapy (both patients), high-dose FEIBA (in one patient) and use of lower doses of heparin for line flush than that recommended by some authors. Neither patient had a familial or non-familial predisposition to thrombosis. ( info) |
7/933. Hemophiliacs bone pseudotumors. Four cases of proved hemophiliac pseudotumors caused by intraosseous bleeding are reported. Five lesions were found at the uncommon locations involving the cranial vault, mandible, phalanx, distal femur and distal tibia. The conventional radiographic and computed tomographic findings are expansile osteolytic destruction, cortical thinning, partial breaking cortex or pathological fracture, and sometimes associated soft tissue mass. Ultrasonographic feature of one case at the phalanx shows cortical expansion and thinning contained mixed echogenicity in the medullary canal with soft tissue extension. T99m DTPA of one case at the distal femur shows increased vascular flow and uptake at right distal tibia and left distal femur. ( info) |
8/933. Antifactor VIII antibody inhibiting allogeneic but not autologous factor viii in patients with mild hemophilia a. Two unrelated patients with the same Arg2150His mutation in the factor viii (FVIII) C1 domain, a residual FVIII activity of 0.09 IU/mL, and inhibitor titres of 300 and 6 Bethesda Units, respectively, were studied. Further analysis of patient LE, with the highest inhibitor titer, showed that (1) plasma or polyclonal IgG antibodies prepared from LE plasma inhibited the activity of allogeneic (wild-type) but not of self FVIII; (2) the presence of von Willebrand factor (vWF) increased by over 10-fold the inhibitory activity on wild-type FVIII; (3) the kinetics of FVIII inhibition followed a type II pattern, but in contrast to previously described type II inhibitors, LE IgG was potentiated by the presence of vWF instead of being in competition with it; (4) polyclonal LE IgG recognized the FVIII light chain in enzyme-linked immunosorbent assay and the recombinant A3-C1 domains in an immunoprecipitation assay, indicating that at least part of LE antibodies reacted with the FVIII domain encompassing the mutation site; and (5) LE IgG inhibited FVIII activity by decreasing the rate of FVIIIa release from vWF, but LE IgG recognized an epitope distinct from ESH8, a murine monoclonal antibody exhibiting the same property. We conclude that the present inhibitors are unique in that they clearly distinguish wild-type from self, mutated FVIII. The inhibition of wild-type FVIII by LE antibody is enhanced by vWF and is associated with an antibody-dependent reduced rate of FVIIIa release from vWF. ( info) |
9/933. Ultrastructure of the haemophilic synovial membrane and electron-probe X-ray analysis of haemosiderin. An ultrastructural study of the haemophilic synovial membrane revealed the presence of solitary siderosomes, compound siderosomes and a peppering of the cell cytoplasm with electron-dense particles. These changes were found in synovial intimal cells, subsynovial macrophages and fibroblasts. Electron-probe X-ray analysis of siderosomes revealed the presence of iron and traces of phosphorus. On the basis of previous and present studies it is postulated that haemosiderin is essentially a condensate of hydrated ferric oxide and that a variable amount of phospholipid material lies in company with it in the siderosome. ( info) |
10/933. The locked patella. An unusual complication of haemophilia. Mechanical derangements of the knee are an uncommon complication of chronic haemophiliac arthropathy. Two patients with locking of the patella were treated by manipulation. The mechanism of the injury was forced flexion of the knee joint beyond the limit of its restricted range. The injury is a serious one and may take six months to recover. ( info) |