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Case study of the serological diagnosis and transfusion management of a pregnant, Nigerian woman with Sickle Cell Diseas

Case History. June 2007 IBTS advised 29 year old patient 9-10 weeks pregnant and has sickle cell diseaseHad received 2 units of blood in Nigeria, date unknown11.05.07 blood requested, Anti E found in enzyme with weakly positive reaction in IAT with 3rd cell of DiaMed ID panel (R2R2). Case Histor

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Case study of the serological diagnosis and transfusion management of a pregnant, Nigerian woman with Sickle Cell Diseas

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    1. Case study of the serological diagnosis and transfusion management of a pregnant, Nigerian woman with Sickle Cell Disease Liz Murphy Irish Blood Transfusion Service

    2. Case History June 2007 – IBTS advised 29 year old patient 9-10 weeks pregnant and has sickle cell disease Had received 2 units of blood in Nigeria, date unknown 11.05.07 blood requested, Anti E found in enzyme with weakly positive reaction in IAT with 3rd cell of DiaMed ID panel (R2R2)

    3. Case History contd. Patient typing: O+,C+, c+, E-, e+, Cw-, K- Fy (a-b-), Jk (a+b+) , Le (a-b+) , M+, N+, S-, s+, P1+ 2 units crossmatched (O+,C+,c-,E-,e+,K-, Jk (a+b+) and O+,C+,E-,Cw -,K-) both E-,K-,CMV- crossmatch compatible blood transfused. On 11/05/07 Hb level = 7.8g/dl and 7.5g/dl.

    4. Case History contd. 12.05.2007 – Patient transferred to different hospital. Hb 8.9g/dl and 8.8g/dl Anti-E detected 2 crossmatch compatible units (O+,C+,c+,E-e+,K- and O+,C+,c-,E-,e-,Cw -,K-,S-,Jk (a-b+)) infused. On 13/05/07, Hb 10.3g/dl

    5. Case History contd. Saturday 26.05.2007 Patient admitted to latter hospital through A&E Hb 5g/dl Four units of screened blood were requested: O+,E-,K-,S-,Fya- One C+ unit gave complete reaction on IAT crossmatch Three Ror units compatible

    6. Case History contd. Unit 1 O+,C+,E-,Cw -,K-,S-,Fya-,Jk (a-b+). Unit 2 O+,C-,E-,Cw-,K-,S-,M-,Fya-,Jk (a+b+). Unit 3 O+,C-,E-,Cw -,K-,S-,Fya-,Jk (a+b+). Unit 4 O+,C-,E-,Cw-,K-,S-,Fya-,Jk (a+b+). The three compatible Ror units were re-crossmatched over the weekend with a fresh sample and unit 2 was weakly incompatible.

    7. Sample referred to IBTS On 30.05.07 a sample taken on 29.05.07 K.J. typed as C+, c+, E-, e+,K-,Lua-. DCT gave a very weak positive reaction for IgG and C3d, negative for IgA, IgM and C3c. Strong non specific autoantibody in tube saline @18şC and enzyme (papain) treated cells.

    8. IBTS contd. 4/11 cell ID IAT card negative, the auto was +w and some positive reactions showed a mixed-field pattern. A tube pre-warm IAT/IgG showed only slight variation in strength of rxs and auto - ? Anti-C, E, Lua, Bg and a possible Cw Sample tested positive for leucocyte antibodies.

    9. Sample bled 31/05/07 Eluate from fresh sample only very weakly positive with 4/11 cells, no apparent specificity. Allo-adsorptions x 2 with OR1R1 and Orr. Conclusion:plasma appears to contain an apparent allo-anti-C, anti-E, anti-Lua, and Leucocyte antibodies

    10. Transfusion History On 31/05/07 K.J. received the two compatible Ror units (3and 4)-Hb=4 g/dl. On 01/06/07 Orr (C-,E-,Cw-,K-,S-,Fya-,M-Lua-, Jk(a+b+) compatible unit infused. On 02/06/07 –Hb=7g/dl. 3 Orr (C-,E-,Cw-K-,S-,Fya-Lua-) and 1 ORor (C-,E-,Cw-,K- S-Fya-, Lua- ) infused. On 04/06/07and 05/06/07 Hb=11g/dl.

    11. Referral to IBGRL Sample on K.J. referred on 05/06/07. IBGRL report (26/06/07) confirmed the presence of anti-C+E+Lua and a further (much weaker) antibody to a high incidence Rh antigen. Selected RH antigen typing: V/VS+, hrs+, hrb+. This would appear to exclude anti-hrb specificity, likely antibody to high frequency Rh antigen e-related.

    12. IBGRL Report contd. Because J.K. is V/VS+ she can make anti-C (actually anti-Ce or Rh7). She has the Cdes haplotype (Ce-) and therefore she can make anti-Ce (reacts with C and normal e on same haplotype). Both of the compatible hrb-cells were Cdes ie(C+Ce-) Suspect anti-D but inconclusive due to lack of serum Anti-Fy3 excluded. Req. repeat sample.

    13. Referral to IBGRL 30 July, 2007 IBTS results of referred sample. Auto in IAT gone from a (+w) to a (++) All 11 cells (++) except for OR2R2 cell which was (+s) Following differential alloadsorptions x 4 with OR1R1 and Orr cells - Anti-C+E+ leucocyte antibodies were confirmed in this sample. Anti-Lua was not.

    14. Report from IBGRL 11 September 2007 Confirmed continued presence of strong anti-C (anti-Ce) +E + antibody to a high incidence e-related antigen Not possible to confirm continued presence of anti-Lua as rr cells are now reactive by IAT with untreated cells as well as papain treated cells

    15. IBGRL Report contd. Adsorption with papain treated r’r’ and r”r” cells removed all reactivity and anti-D comprehensively excluded. Using raw unadsorbed serum all cells were incompatible with the exception of one example each of hrb-, Rhnull and -D-/-D- cells. Compatible phenotypes extremely rare.

    16. Available Stock Blood of choice is Ror cells of African ethnic origin. Rhnull 2 units in Birmingham but K+. -D-/-D- only group As (no Os). hrb- 4 units frozen in Liverpool (same donor as IBGRL hrb- cells). rr random Caucasian donor - incompatible with patient’s r phenotype.

    17. Eight Rh haplotypes and their frequencies in English, Nigerian and Hong Kong Chinese populations

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