First Case in Korea of a Patient With Anti-PP1Pk Antibodies: Successful Blood Management via Acute Normovolemic Hemodilution

Ha, Changhee; Choi, Sooin; Yu, HongBi; Chun, Sejong; Kim, Kyeong-Hee; Lee, Jong‐Hwan; Han, In Woong; Cho, Duck

doi:10.3343/alm.2019.39.6.602

Cited by 2 publications

(1 citation statement)

References 8 publications

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“…All p phenotype individuals have anti-PP1Pk in the plasma, and complement-mediated hemolysis is thought to be the cause of the hemolytic transfusion reaction (HTR) [2], hemolytic disease of the fetus and the newborn (HDFN) [3], and spontaneous abortions [4] reported in these patients. PP1Pk is encoded by A4GALT , and the P-null phenotype results from a variety of mutations at the genomic level [5]. This report describes a case that presented with an alloantibody to an HFA.…”

Section: Introductionmentioning

confidence: 99%

P-Null Phenotype Due to a Rare Frame-Shift Mutation and with Allo-Anti-PP1Pk Causing a Severe Hemolytic Transfusion Reaction: A Case Report with Clinical Management

Kanani¹,

Senjaliya²,

Rajapara³

et al. 2021

Transfus Med Hemother

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Introduction:The identification of alloantibodies to high-frequency antigens (HFA) and subsequent transfusion management can be challenging and often poses a problem in finding the compatible blood for transfusion. The aim of this study was to investigate the specificity of the antibody to the HFA causing a hemolytic transfusion reaction (HTR) and procure the compatible blood unit for future transfusion. Case presentation:A 4-year-old female met with a head injury that led to intracranial bleeding and surgical intervention was required to remove blood clots. In the face of anemia, blood transfusion was planned. The pretransfusion tests on her blood sample revealed the presence of a pan-reactive alloantibody with hemolytic properties. She was transfused with 10 mL of the least incompatible red blood cells (RBCs) to which she reacted with signs of clinical hemolysis, i.e., chill, rigor, fever, and hemoglobinuria, on 3 different occasions. Despite her anemia, she was managed by medical intervention only. Her antibody reacted with all RBCs tested, except autologous and P-null (p phenotype) cells. Her RBCs did not react with anti-PP1Pk, which corroborated her phenotype as P-null. The genomic study revealed she was hemi- or homozygous or for a deletion of 26-bp in A4GALTexon 3, previously reported as causing the P-null phenotype and designated A4GALT*01N.019.Conclusion:This report documents a rare case of the P-null phenotype with an alloanti-PP1Pk causing a severe HTR to transfusion of the trial dose of the least incompatible blood. The case is the first example of this specific A4GALTmutation found in India.

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Section: Introductionmentioning

confidence: 99%

P-Null Phenotype Due to a Rare Frame-Shift Mutation and with Allo-Anti-PP1Pk Causing a Severe Hemolytic Transfusion Reaction: A Case Report with Clinical Management

Kanani¹,

Senjaliya²,

Rajapara³

et al. 2021

Transfus Med Hemother

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Alloantibodies to High-Incidence Antigen: Review of Cases and Transfusion Experiences in Korea

Choi

Chung

Cho

et al. 2019

KJBT

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Antibodies to high-incidence red blood cell antigens should be considered if panagglutination reactions are noted in all panel cells, and negative reactions to autologous red blood cells are detected on antibody screening and identification tests. In Korea, most of those antibodies are identified through international reference laboratories. To prevent a hemolytic transfusion reaction, antigen-negative red cells should be provided for those patients who have antibodies to red cell antigens. However, this is nearly impossible when the antibody has specificity to high-incidence red cell antigen. In those cases, transfusion of autologous blood, cryopreserved rare blood and the least incompatible blood components can be considered. In the case of surgery, acute normovolemic hemodilution or intraoperative blood salvage can also be considered. For the patients who have antibodies to high-incidence red cell antigens, it should be discussed to set up a national reference laboratory to quickly identify antibody specificities, and to consider establishing rare blood donor registry and frozen rare blood storage/supply system. This article reviews characteristics of antibodies to high-incidence antigens found in Koreans and also the transfusion experiences of those patients based on literature. (Korean J Blood Transfus 2019;30:101-112)

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First Case in Korea of a Patient With Anti-PP1Pk Antibodies: Successful Blood Management via Acute Normovolemic Hemodilution

Cited by 2 publications

References 8 publications

P-Null Phenotype Due to a Rare Frame-Shift Mutation and with Allo-Anti-PP1Pk Causing a Severe Hemolytic Transfusion Reaction: A Case Report with Clinical Management

P-Null Phenotype Due to a Rare Frame-Shift Mutation and with Allo-Anti-PP1Pk Causing a Severe Hemolytic Transfusion Reaction: A Case Report with Clinical Management

Alloantibodies to High-Incidence Antigen: Review of Cases and Transfusion Experiences in Korea

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