PMID- 8693515
OWN - NLM
STAT- MEDLINE
DCOM- 19960829
LR  - 20190727
IS  - 0041-1132 (Print)
IS  - 0041-1132 (Linking)
VI  - 36
IP  - 5
DP  - 1996 May
TI  - Serologic evidence that factor IX inhibitor in the plasma of hemophilia B 
      patients detects factor IX on normal red cells.
PG  - 467-9
AB  - BACKGROUND: Patients with hemophilia B lack factor IX (F IX). These patients may 
      become alloimmunized after the transfusion of F IX concentrates and may develop F 
      IX inhibitors, which have been characterized as polyclonal IgG4 alloantibodies. 
      Two cases in which F IX inhibitors caused difficulty in compatibility testing and 
      antibody identification were encountered. It was hypothesized that, because F IX 
      is present in normal plasma, it might be adsorbed by red cells in vivo and then 
      be detected during antibody screening tests with serum containing F IX 
      inhibitors. CASE REPORT: Sera from two African American half-brothers with 
      hemophilia B were incompatible with all common and rare red cell phenotypes 
      tested in the anti-human globulin test, but did not react with each other's red 
      cells. The brothers' red cell antibodies were neutralized with both normal plasma 
      and a commercially available F IX concentrate, which indicated that the red cell 
      incompatibility was most probably caused by their F IX inhibitors. Red cells from 
      an unrelated patient with hemophilia B and a very low titer of F IX inhibitor 
      were tested against the half-brothers' sera and did not react. The compatible red 
      cells from one of the half-brothers and the unrelated patient with hemophilia B 
      adsorbed F IX from normal plasma or F IX concentrate after 37 degrees C 
      incubation; this rendered them incompatible with the plasma containing F IX 
      inhibitor from the other half-brother. CONCLUSION: F IX appears to be present on 
      normal red cells and may be detected during compatibility and antibody 
      identification procedures when serum or plasma containing F IX inhibitors is 
      tested.
FAU - Swanson, J L
AU  - Swanson JL
AD  - Department of Laboratory Medicine and Pathology, University of Minnesota Hospital 
      and Clinic, Minneapolis, USA.
FAU - Moertel, C L
AU  - Moertel CL
FAU - Stroncek, D F
AU  - Stroncek DF
FAU - Key, N S
AU  - Key NS
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Transfusion
JT  - Transfusion
JID - 0417360
RN  - 9001-28-9 (Factor IX)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Erythrocytes/*chemistry
MH  - Factor IX/analysis/*antagonists & inhibitors
MH  - Hemophilia A/*blood
MH  - Humans
MH  - Male
EDAT- 1996/05/01 00:00
MHDA- 1996/05/01 00:01
CRDT- 1996/05/01 00:00
PHST- 1996/05/01 00:00 [pubmed]
PHST- 1996/05/01 00:01 [medline]
PHST- 1996/05/01 00:00 [entrez]
AID - 10.1046/j.1537-2995.1996.36596282593.x [doi]
PST - ppublish
SO  - Transfusion. 1996 May;36(5):467-9. doi: 10.1046/j.1537-2995.1996.36596282593.x.