Prednisone and low-dose activated prothrombin complex concentrates for FVIII inhibitor in nonhaemophilic patients.
Eight nonhaemophilic patients with factor VIII (FVIII) inhibitors were reported. There was no difference in sex distribution. Median age at diagnosis was 62 years (ranging from 14 to 73 years). No associated disorders were revealed and all the patients were presented with severe muscular or arthral bleeding. Inhibitor titre was measured by the Bethesda method, which were 6.4, 126.0, 155.0, 4.8, 56.0, 13.5, 35.0 and 150.0 BU mL-1, respectively, at diagnosis. FVIII:C levels were less than 1 U dL-1 in seven patients and less than 2 U dL-1 in one patient. The median vWF:Ag level was 210% (ranging from 80% to 340%). All the patients had good response to activated prothrombin complex concentrates for acute bleeding episodes and prednisone for inhibitor elimination. Inhibitors completely eliminated in seven patients within a follow-up duration over 1 year, and one patient died of intracranial haemorrhage when her inhibitor titre decreased to 4.5 BU mL-1 and FVIII:C increased to 21 U dL-1.
Ji
L
L
Blood Diseases Hospital, CAMS & PUMC, Tianjin, P. R. China.
Yang
R
R
Yang
D
D
Chen
Z
Z
Xing
S
S
Tian
M
M
Sun
Y
Y
D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000096:biosynthesis*; D002118:Calcium / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D005165:Factor V / Q000627:therapeutic use*; D005169:Factor VIII / Q000276:immunology*; D015951:Factor Xa / Q000627:therapeutic use*; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy; D006470:Hemorrhage / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010743:Phospholipids / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use*; D012189:Retrospective Studies
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D010743:Phospholipids; D005165:Factor V; D005169:Factor VIII; D015951:Factor Xa; D002118:Calcium; D011241:Prednisone