scholarly journals 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

2021 ◽  
pp. 1-4
Author(s):  
Ashish N. Kanani ◽  
Snehal B. Senjaliya ◽  
Manisha M. Rajapara ◽  
Judith Aeschlimann ◽  
Connie M. Westhoff ◽  
...  

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.

2015 ◽  
Vol 61 (6) ◽  
pp. 522-528
Author(s):  
Eiko Date ◽  
Shinichi Miyama ◽  
Nagisa Kawashiri ◽  
Kasumi Suzuki ◽  
Youko Fuke ◽  
...  

Author(s):  
Leandro Dinalli Santos ◽  
Carolina Bonet Bub ◽  
Maria Giselda Aravechia ◽  
Eduardo Peres Bastos ◽  
Jose Mauro Kutner ◽  
...  

2019 ◽  
Vol 10 (2) ◽  
pp. 137-139
Author(s):  
Elida Marpaung

Latar belakang: Protein Kidd merupakan transporter urea pada sel darah merah. Walaupun jarang, adanya antibodi terhadap antigen ini dapat menyebabkan reaksi transfusi dan hemolytic disease of the newborn. Keberadaan anti-Jka dan anti-Jkb cukup jarang ditemukan pada pemeriksaan identifikasi antibodi pasien. Studi ini melaporkan kasus pasien dengan keberadaan anti-Jka and anti-Jkb, yang mendapat darah dengan kadar aglutinasi terendah pada kondisi dimana darah kompatibel sulit didapat sementara tindakan transfusi sangat dibutuhkan segera. Penyajian Kasus: Wanita, 36 tahun, G4P3A0, datang dengan perdarahan vaginam sejak sebulan terakhir. Dari hasil pemeriksaan USG, didapatkan adanya mola hidatidosa. Pasien memerlukan terapi kuret segera setelah anemia terkoreksi (Hb 8.3 g/dL). Pada uji kecocokan pre-transfusi dengan prosedur skrining antibodi yang dilanjutkan dengan identifikasi antibodi, ditemukan anti-Jka dan anti-Jkb. Dari setidaknya 50 darah donor yang dilakukan uji kecocokan, tidak ditemukan darah yang kompatibel, sehingga pasien diputuskan untuk mendapat transfusi menggunakan darah inkompatibel dengan derajat aglutinasi terendah (level 2) dari 5 level, disertai dengan pemantauan ketat terhadap potensi terjadinya reaksi transfusi. Demam dan pruritus dilaporkan dalam 24 jam setelah transfusi, dan membaik setelah pemberian injeksi difenhidramin, deksametason, dan parasetamol. Kesimpulan: Transfusi dengan darah yang inkompatibel merupakan pilihan terakhir bila tidak ditemukan darah donor yang kompatibel. Reaksi transfusi merupakan efek yang sulit dihindari, tetapi dapat dilakukan pemantuan ketat. Pemilihan darah dengan level aglutinansi terendah adalah keputusan terbaik, mengingat tindakan medis diperlukan segera untuk menyelamatkan nyawa. Pada kasus ini, pasien mendapat tatalaksana optimal dari aspek tindakan operasi dan respon transfusi, yang ditunjukkan melalui kenaikan nilai Hb yang bermakna. Sementara itu, efek samping reaksi transfusi yang muncul hanya ringan dan dapat ditanggulangi dengan pemberian obatobatan. (Health Science Journal of Indonesia 2019;10(2):137-9) Kata kunci: reaksi transfusi, inkompatibilitas, kelompok darah Kidd   Abstract Background: Kidd protein is red blood cell’s (RBC) major urea transporter. Albeit rare, the presence of antibodies against Kidd antigen may cause significant hemolytic transfusion reaction and hemolytic disease of the newborn. Yet, anti-Jka and anti-Jkb are rare to be discovered during antibody identification. This paper reported “bestmatched” transfusion practice in a patient with anti-Jka and anti-Jkb, where compatible PRC cannot be found, but transfusion is urgently needed. Case Presentation: A 36 years old, G4P3A0 female, came with continuous vaginal bleeding for the past one month before admission. USG revealed hydatidiform mole. She needed immediate curettage following correction of her anemia (Hb 8.3g/dL). After antibody screening procedure followed by antibody identification, we found a positive anti-Jka and anti-Jkb in her blood sample. At least 50 blood donors were tested for compatibility and none was a match. She was then transfused with the lowest agglutination blood available (level 2 of 5 levels), with a closed monitoring to anticipate the possibility of transfusion reaction development. Fever and pruritus transpired within 24 hours post transfusion and it resolved following diphenhydramine, dexamethasone, and paracetamol injection. Conclusion: Incompatible blood transfusion is the last option when compatible blood cannot be found. The development of transfusion reaction is inevitable, but it can be anticipated by closed monitoring. In restricted setting, blood transfusion with the lowest level of agglutination is acceptable when transfusion is imperative. In this case, the patient got optimal treatment in term of the medical surgery and transfusion response, which was shown by the significant increase of Hb level. Meanwhile, the adverse transfusion reaction was only mild, and could be treated with medicine. (Health Science Journal of Indonesia 2019;10(2):137-9) Keywords: Transfusion reaction, incompatibility, Kidd blood group


2011 ◽  
Vol 139 (7-8) ◽  
pp. 518-522
Author(s):  
Radmila Jovanovic ◽  
Nevenka Bujandric ◽  
Slobodanka Lisulov ◽  
Sanja Bogdanovic

Introduction. Transfusion management of patients who are alloimmunized against high-prevalence erythrocyte antigens is often problematic. Strategy management depends, not only on the specific clinical circumstances of the patient, but also on the acceptable time frame. In patients without clinically significant antibody incompatible transfusion it may be less harmful than delaying medical intervention. Case Outline. We report a 57-year-old female from Libya, blood group O, RhD-positive, who was treated at the Institute of Cardiovascular Diseases of Vojvodina. At the Blood Transfusion Institute of Vojvodina, during pretransfusion testing an IgG alloantibody of unknown specificity was determined. A total of 200 blood units (O, RhD-positive) were crossmatched, but positive reactions indicating that the donor units were incompatible for that specific patient. By testing the patient?s family members in Tripoli, six compatible blood units were found and applied during and after surgery. Due to the deterioration of the patient?s condition a rapid transfusion was required; however cross-match compatible blood was not available. After a biological crossmatch to predict the clinical significance of this antibody, 12 units of erythrocytes with the lowest positive cross-match reactions, were transfused to the patient without any adverse effects. Good tolerance of the units suggested that the present antibodies were not clinically significant. Later on, a rare alloantibody directed to the high frequency Gerbich blood group antigens was identified by the Foundation Central Laboratory, Blood Transfusion Service in Bern, Switzerland. Conclusion. In cases of emergency patients with alloantibodies against high frequency Gerbich, when autologous or compatible alogenous transfusion is unavailable, blood with the lowest positive cross-match reaction could be transfused if the biological cross-match is negative. Formation of a national register of donors with rare blood groups and their connection with international registers is of crucial significance in the management of patients requiring antigen negative blood otherwise unavailable from routine blood banks.


2014 ◽  
Vol 5 ◽  
pp. BTRI.S14578 ◽  
Author(s):  
Girolamo A. Ortolano ◽  
Barry Wenz

Osteoarthritis (OA) is the most prevalent musculoskeletal disease in humans and domestic animals. It causes significant clinical problems and substantial health care costs. In the absence of disease-modifying medical intervention, therapy is currently restricted to palliative measures prior to surgical intervention. We review the pathogenesis, as well as conservative and emerging restorative therapeutic approaches, including cytokines, stem cells, and platelets. The various methods of platelet concentrate preparations and their reported outcomes are discussed. Data collected from the use of intra-articular platelet therapy (IAPT) in dogs are reviewed, which suggest that this approach may delay or in some cases even obviate the need for surgical intervention.


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