1/8. Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor v deficiency.We report a newborn infant girl, born to consanguineous parents, with recurrent intracranial hemorrhage secondary to congenital factor v deficiency with factor V inhibitor. Repeated transfusions of fresh-frozen plasma (FFP) and platelet concentrates, administrations of immunosuppressive therapy (prednisolone and cyclophosphamide), and intravenous immunoglobulin failed to normalize the coagulation profiles. Exchange transfusion followed-up by administrations of activated prothrombin complex and transfusions of FFP and platelet concentrates caused a temporary normalization of coagulation profile, enabling an insertion of ventriculoperitoneal (VP) shunt for progressive hydrocephalus. The treatment was complicated by thrombosis of left brachial artery and ischemia of left middle finger. The child finally died from another episode of intracranial hemorrhage 10 days after insertion of the VP shunt.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
2/8. life-threatening bleeding and acquired factor v deficiency associated with primary systemic amyloidosis.Acquired factor x deficiency has been described in patients with amyloidosis but acquired factor v deficiency is quite rare. We report here a case of life-threatening bleeding and acquired factor v deficiency associated with primary amyloidosis. A 50-year-old man who had no previous hemorrhagic diathesis was referred to our hospital because of recurrent epistaxis, gingival bleeding and hemospermia. The laboratory examination revealed that both the prothrombin time (PT) and the activated partial thromboplastin time (aPTT) were significantly prolonged, and factor V activities were markedly decreased to 14-39% of the normal value. Other coagulation factors such as fibrinogen, prothrombin, factor VII, factor viii, factor ix and factor X were subnormal and normal. transaminases were slightly elevated but serological tests of hepatitis b and hepatitis c were negative. Mild hepatosplenomegaly was noted without sign of liver cirrhosis. The PT and aPTT obtained 8 years ago when he received a cholecystectomy due to cholecystitis were both normal. Specific assays for the detection of factor V inhibitor were repeatedly performed but no factor V inhibitor was found. Furthermore, a significant recovery of the infused factor V was noted shortly after an intravenous administration of 5-10 U fresh frozen plasma, but it did not last more than 6 h. melena, bleedings into the left shoulder and buttock, and finally mortal retroperitoneal hemorrhage developed despite repeated infusions of large amounts of fresh frozen plasma. Acquired factor v deficiency associated with primary amyloidosis was suspected but histological diagnosis was not obtained because of the severe bleeding tendency. autopsy revealed hepatosplenomegaly and massive deposits of AL amyloid in the liver, spleen, heart and other parenchymal organs. Perivascular amyloid deposition and factor v deficiency are both thought to be the cause of the severe hemorrhagic tendency seen in this patient.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
3/8. High-dose intravenous immunoglobulin treatment in two patients with acquired factor V inhibitors.We report two patients who developed acquired factor V (FV) inhibitors not related to exposure to bovine thrombin. Associated conditions were found in one patient (surgery, antibiotic administration) but none in the other one. Bleeding complications occurred only in the patient with idiopathic FV inhibitor, leading to packed red cell infusion. Laboratory findings showed the presence of specific FV inhibitors with titers of 5.5 and 5 Bethesda units, respectively. These two patients received high-dose intravenous immunoglobulin and FV levels normalized within a few days with a concomitant disappearance of FV inhibitors.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
4/8. pregnancy and oral contraceptives in factor v deficiency: a study of 22 patients (five homozygotes and 17 heterozygotes) and review of the literature.Information on the effect of pregnancy or oral contraceptives (OC) in congenital factor V (FV) deficiency is scanty. The personal investigation of five homozygous and 17 female heterozygous showed that patients with severe deficiency bleed considerably at the time of delivery. However, bleeding can be controlled properly by administration of fresh frozen plasma with excellent foetal outcome. The safe level for adequate haemostasis seems around 25% of normal. On the contrary, heterozygote patients show no significant postpartum bleeding and therefore need no substitution therapy. Oral contraceptives were taken and well tolerated by four of our homozygous patients and appear to be beneficial because they cause a decrease in menometrorrhagies thereby improving the anaemia and decreasing transfusional needs. One patient took hormonal replacement therapy with no undue effects. No thrombosis was noted in the propositae during oral contraceptive therapy. The review of the literature has allowed the gathering of information on 20 additional pregnancies. The foetal outcome was satisfactory in every instance. Excessive bleeding was noted in 11 pregnancies. In seven of the remaining pregnancies, no undue bleeding was noted thanks to appropriate substitution therapy. In the remaining two pregnancies no bleeding was noted and no substitution therapy was given. No data are apparently available in the literature about the use of OCs in FV deficiency.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
5/8. pulmonary embolism in a patient with severe congenital deficiency for factor V during treatment with fresh frozen plasma.thrombosis is a rare complication in patients with congenital clotting factor deficiencies. In most cases, it is related to inherited procoagulant factors, use of central venous catheters or administration of coagulation factor concentrates. There are only a few case reports about thrombotic events during treatment with fresh frozen plasma (FFP). We report the case of a patient with homozygous inherited factor v deficiency, who developed a pulmonary embolism at a time of treatment with methylene blue treated FFP (MBFFP). The patient had only two other factors predisposing to thrombosis and both were acquired: obesity and bed rest. He started anticoagulant treatment with low molecular weight heparin (LMWH) while the deficient factors were replaced with MBFFP. After 8 days of treatment the patient developed a severe respiratory insufficiency. Pulmonary haemorrhage was considered among the differential diagnosis and LMWH was stopped. An inferior vena cava filter was placed without any further thrombotic complications. To our knowledge, there are no reports about patients with clotting factor deficiencies who developed a thrombotic event during treatment with MBFFP.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
6/8. factor v deficiency and its reversal with gluten restriction. In a patient with celiac disease.Hemorrhagic manifestations in patients with celiac disease are uncommon and, when present, are usually due to a deficiency of the vitamin k-dependent clotting factors. A patient with celiac disease was seen with a severe bleeding diathesis associated with deficiencies of factor V. The deficiencies of vitamin k-dependent clotting factors were rapidly reversed with parenteral administration of phytonadione. Factor V levels returned to normal levels only after gluten restriction.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |
7/8. Hypotensive reactions to white cell-reduced plasma in a patient undergoing angiotensin-converting enzyme inhibitor therapy.BACKGROUND: Hypotensive reactions to platelet transfusions performed with white cell (WBC)-reduction filters with negatively charged surfaces have been reported recently in patients taking angiotensin-converting enzyme (ACE) inhibitors. Experimental studies have shown that the filter material can activate bradykinin, which may cause symptoms in patients with reduced bradykinin catabolism. Symptomatic adverse reactions after the administration of fresh-frozen plasma (FFP) through a WBC-reduction filter have not been reported in a patient on ACE Inhibitor medication. CASE REPORT: A 58-year-old man with congenital coagulation factor v deficiency and hypertension treated with an ACE inhibitor was admitted for rehabilitation after orthopedic surgery. On 3 consecutive days, he received FFP through a WBC-reduction filter; within minutes of the beginning of each infusion, he experienced a drop in blood pressure, facial erythema, abdominal pain, and anxiety. When the infusions were stopped, symptoms quickly abated without treatment. Multiple prior transfusions of unfiltered FFP and FFP filtered through a WBC-reduction filter made by a different manufacturer, as well as subsequent transfusions of unfiltered FFP, had not produced such reactions. CONCLUSION: Facial flushing, hypotension, and abdominal pain after FFP administration in a patient on ACE inhibitor medication appeared to be associated with a specific type of WBC-reduction filter. This association and other reported studies suggest that special caution is warranted when patients who are treated with ACE inhibitors receive blood components administered through WBC-reduction filters capable of generating bradykinin.- - - - - - - - - - ranking = 1keywords = administration (Clic here for more details about this article) |
8/8. Hemostatic treatment after tooth extraction in a patient with factor v deficiency.BACKGROUND: factor v deficiency is a very rare hereditary coagulation disorder and tooth extraction in the patient with factor v deficiency has not been reported except in one case. patients AND TREATMENT: A 38-year-old woman with factor v deficiency was referred for extraction of the impacted lower third molar. After intravenous administration of frozen fresh plasma (FFP) and recognition of an increase of factor V level from 1-31%, upper and lower third molars were extracted. Eighteen and 48 h after the extraction, factor V was intermittently supplemented by injection of 4 and 2 units of FFP, respectively, and factor V was maintained above 12%. To form fast coagula and to protect the wound, the lower extraction socket was filled with a fibrin glue composed of factor xiii and fibrinogen (Beriplast P) and a plastic splint was applied. The wound was healed and epithelized within 2 weeks after the extraction without any bleeding or infectious consequences. CONCLUSION: Extraction in the patient with factor V hereditary deficiency is safely performed by both supplementation of factor V and application of local hemostasis.- - - - - - - - - - ranking = 0.5keywords = administration (Clic here for more details about this article) |