The P Phenotype in A Lahu Boy from Northern Thailand
Keywords:
p phenotype, Anti-P1PPkAbstract
Background: The p phenotype belongs to the P system. Group p lacks P1 ,P and Pk antigens so that the serum of a p individual contains naturally occurring hemolytic anti-P1PPk that reacts with P1 and P2 red cells. Objective: To report a case of the p phenotype in a Lahu boy. Materials and Methods: ABO, Rh and P grouping including antibody screening and identification were performed in blood sample from a Lahu boy. Results: A 7 year-old Lahu boy presented with fever, epistaxis, leukopenia and thrombocytopenia. The anemia was a result of iron deficiency and blood loss from epistaxis. Two units of packed red cells and 5 units of platelets were requested. Laboratory testing revealed that the patient belonged to blood group B, R2Rz (cDE/CDE) and p with anti-P1PPk in serum. His anti-P1PPk hemolyzed all panel cells and 8 units of donor red cells. Dithiothreitol-treated serum showed that the anti-P1 PPk was a mixture of IgM and IgG. Autocontrol was negative. Family study shows his father has group B, R1Rz (CDe/CDE), P1, his mother has group B, R1 R2 (CDe/cDE), P2 his sister has group O, R1R1 (CDe/CDe), P1 the matemal grandfather demonstrated the same group as the mother. Antibody screening in his family was negative. No additional p phenotype was found in this family. The blood bank could not provide packed red cells so he only received 5 units of platelets concentrates. The patient was treated with ferrous fumarate for iron supplementation and albendazole to kill hook worms and his
condition improved steadily. One month later the boy recovered completely. His blood was reconfrmed as a p phenotype with anti-P1 PPk in his serum. Conclusion: The p phenotype made a problem for blood transfusion due to anti-P1PPk. The anti-P1PPk was a mixture of IgM and IgG that could bind complement and show hemolytic pattern. This may have adverse consequences when transfusing P1or P2 red cells to a precipient. Therefore a precipient must only receive blood from a p donor.
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