1/68. tympanoplasty in a patient with hemophilia b.We report the case of a 36-year-old hemophilia b who suffered from cholesteatoma and underwent tympanoplasty. Though the factor ix activity was less than 1% of normal before surgery, adequate replacement of factor ix led to the achievement of hemostasis during and after surgery. The cholesteatoma was completely extirpated with matrix, and a type I canal-up tympanoplasty was subsequently performed. Careful preoperative evaluation and close cooperation with the hematologist are required if surgery is to be successful. We also present here the use of continuous administration of monoclonal antibody-purified factor ix concentrate, Christmassin M.- - - - - - - - - - ranking = 1keywords = ix (Clic here for more details about this article) |
2/68. Failure of immunosuppression in a severe haemophilia B patient with specific antibody.Prevention of a secondary response to factor ix by cyclophosphamide was attempted in an 11 year old patient with severe Christmas disease. An antibody to factor ix had been present for 4 years before immunosuppressive therapy was tried. Despite profound lymphopenia, synthesis of factor ix antibody was not depressed. The difficulties of modifying the anamnestic response to factor ix by chemical immunosuppression may be as real as has been reported for factor viii in classical haemophilia.- - - - - - - - - - ranking = 0.00032618219722462keywords = disease (Clic here for more details about this article) |
3/68. Remission of progressive multifocal leukoencephalopathy following highly active antiretroviral therapy in a patient with hiv infection.Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease resulting from lytic infection of oligodendrocytes by the papovavirus JC (JCV). PML has also been recognized as an AIDS-defining illness. The incidence of PML has increased since 1987 and it occurs in up to 4% of patients with AIDS. To date, there is no treatment available for PML and it usually results in death within 3-6 months of diagnosis. However, there are some reports of remission of PML after antiretroviral therapy. We report a 12-year-old child with hemophilia b and developing AIDS with the onset of PML. With highly active antiretroviral therapy, PML subsided with an increase of CD4 count from 10 to 300/microl in spite of about 1.0 X 10(4) human immunodeficiency virus (hiv)-1-rna copies. He has survived more than 1 year without specific therapy against JCV. Highly active antiretroviral therapy appears to have improved his prognosis in hiv-associated PML.- - - - - - - - - - ranking = 0.00032618219722462keywords = disease (Clic here for more details about this article) |
4/68. Hemorrhagic lymphadenopathy as a presenting feature of primary al amyloidosis.Lymphadenopathy associated with hemorrhage as a presenting feature of primary (AL) amyloidosis has not previously been described. We report two such cases one of whom had an acquired factor x and IX deficiency. The clinical presentations were characterized by sudden spontaneous enlargement of lymph nodes followed by partial regression. In both cases significant delay in diagnosis, and hence treatment, occurred due to the mode of presentation. One patient died with rapidly progressive disease but the other has had an excellent response to therapy with high-dose melphalan (HDM, 200 mg/m2) and peripheral blood stem cell rescue. AL amyloid should be considered in all patients presenting with hemorrhagic lymphadenopathy.- - - - - - - - - - ranking = 0.00032618219722462keywords = disease (Clic here for more details about this article) |
5/68. Drug-induced and traumatic nail problems in the haemophilias.Many persons with haemophilia suffer from hiv and receive highly active antiretroviral therapy. Three patients received indinavir and required surgery due to ingrown toenails. Two patients suffered from a traumatic subungual haematoma. The treatment protocol is described whereby the pressure exerted onto the germinal layer and the nail bed is relieved in order to alleviate pain and nail matrix damage.- - - - - - - - - - ranking = 1keywords = ix (Clic here for more details about this article) |
6/68. diagnosis of two related carriers of severe haemophilia B with no family history.Haemophilia B is an X-linked disease affecting 1 in 30 000 males. Carrier diagnosis is usually carried out only in female relatives of haemophilic males, and the likelihood of discovering a carrier without a haemophilic male is very low. In this report we present the cases of two related women without a family history of haemophilia who were diagnosed as haemophilia B carriers. Following a minor haemorrhage in the proband, she and her mother were thought to be haemophilia B carriers because of a low factor ix level (16 and 23 IU dL-1, respectively; normal values >50 IU dL-1). The non-sense mutation C31118T, which is associated with severe haemophilia B, was detected in both women. This allowed us to diagnose them as being definite carriers of severe haemophilia B and give appropriate genetic counselling.- - - - - - - - - - ranking = 0.00032618219722462keywords = disease (Clic here for more details about this article) |
7/68. Hemorrhagic diathesis in a carrier of hemophilia b.A carrier of hemophilia b was found to have an unusually low factor ix level of 13 per cent. Her history of previous bleeding and the hospital course following elective dental extractions were consistent with a mild hemorrhagic diathesis. The patient is a member of a rare kindred of hemophiliacs. The mean level of factor ix in 12 carriers in this kindred was 42 per cent, with a range of 13 to 100 per cent. This patient represents the sixth reported case in which a female carrier of factor ix deficiency was symptomatic.- - - - - - - - - - ranking = 1keywords = ix (Clic here for more details about this article) |
8/68. Pulmonary hemorrhage in a hemophiliac simulating a lung neoplasm.We report an elderly, male smoker with moderate hemophilia b who presented with hemoptysis. Chest imaging revealed a well-circumscribed, pulmonary, mass lesion. Repeated bronchoscopy failed to make a diagnosis, and the patient ultimately underwent open thoracotomy with resection of the mass. Pathologic examination revealed hemorrhagic changes in association with bullous lung disease. This is the first report of symptomatic pulmonary hemorrhage in a patient with hemophilia b. hemorrhage into a pre-existing bulla can simulate a primary lung neoplasm.- - - - - - - - - - ranking = 0.00032618219722462keywords = disease (Clic here for more details about this article) |
9/68. gallbladder hematoma in a patient with hemophilia b, report of a case.The authors report the case of a 49-year-old man, hematoma of the gallbladder associated with hemophilia b. The patient was diagnosed incidentally by abdominal computed tomography with a gallbladder tumor. Endoscopic retrograde cholangiopancreatography revealed negative gallbladder and angiography showed no obvious abnormality in the cystic artery. magnetic resonance imaging showed a mass of mixed intensity on T2-weighted image and a mass of mixed intensity on the lining of the gallbladder wall. hemorrhage in the gallbladder was thought to be most likely, however, the gallbladder tumor could not to be neglected. cholecystectomy was performed on the patient and the pathological diagnosis was of a hemorrhage in the gallbladder. hematoma of the gallbladder is a rare complication in patients with hemophilia b, however, it leads sometimes to a fatal course and magnetic resonance imaging is very useful to diagnose hemorrhage in the gallbladder.- - - - - - - - - - ranking = 2keywords = ix (Clic here for more details about this article) |
10/68. Three episodes of delayed hemolytic transfusion reactions due to multiple red cell antibodies, anti-Di, anti-Jk and anti-E.There is no report in which three episodes of delayed hemolytic transfusion reaction (DHTR) occurred from multiple antibodies to red cells (RBCs) in the course of treatment of a patient. This paper describes episodes of anemia and hyperbilirubinemia in concert with the development of three alloantibodies in a multiple transfused patient. The patient was a 71-year-old male suffering from valvular heart disease and hemophilia b with a history of transfusions. Although he received compatible RBCs from 14 donors as judged by a crossmatch test using the albumin-antiglobulin method, three episodes of DHTR occurred after surgery. The first hemolytic episode on day 7 after surgery was due to anti-Di(a) because of clinical and laboratory evidence which included jaundice, sudden increases in total bilirubin (T-Bil) and lactate dehydrogenase (LD) levels, and a decrease (2.2 g/dl) in hemoglobin (Hb) level. The second hemolytic episode on day 16 resulted from newly producted anti-Jk(b). The patient experienced fever, fatigue, nausea and anorexia, and laboratory data showed a second increase in T-Bil, a second decrease (3 g/dl) in Hb, and moderate elevations of blood urea nitrogen (BUN) and creatinine (CRE) levels. The third hemolytic episode on day 39 was due to anti-E. The patient complained of fever and fatigue and had a third unexplained drop (1.5 g/dl) in Hb despite no bleeding. This is the first reported case in which three episodes of DHTR occurred from different red cell antibodies.- - - - - - - - - - ranking = 0.00032618219722462keywords = disease (Clic here for more details about this article) |
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