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| Managing blood requirement in a rare P-null phenotype patient during the COVID pandemic in a resource-limited setting |
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Veena Shenoy1, Linda John1, Rema Ganapathi2, R Janarthanan3, Suhas Udayakumaran4
1 Department of Transfusion Medicine, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham University, Kochi, Kerala, India
2 Department of Clinical Haematology, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham University, Kochi, Kerala, India
3 Department of Plastic and Reconstructive Surgery, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham University, Kochi, Kerala, India
4 Department of Paediatric Neurosurgery and Craniofacial Surgery, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham University, Kochi, Kerala, India
Click here for correspondence address and email
| Date of Submission | 19-May-2022 |
| Date of Acceptance | 05-Jun-2022 |
| Date of Web Publication | 26-Sep-2022 |
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 Abstract | | |
We report the case of a 5-year-old girl with a rare P-null phenotype who presented for a neurosurgical procedure at our center. The case is unique as this patient was one of the two P-null phenotype cases reported in India and we report how we could successfully arrange a rare blood unit for her. As it was challenging to find a donor for her, autologous blood was collected. Despite iron supplementation and erythropoietin injections, her hemoglobin remained low and the required number of autologous units could not be collected. As a consequence of the search initiated with the international and regional rare donor registries, a donor was identified in a remote village in India. Despite the logistic hurdles due to the COVID pandemic, the blood could be transported safely for performing the surgery. A centralized database of rare donors needs to be established to meet such requirements. Keywords: Anti-PP1Pk antibody, autologous, P-null
How to cite this URL:
Shenoy V, John L, Ganapathi R, Janarthanan R, Udayakumaran S. Managing blood requirement in a rare P-null phenotype patient during the COVID pandemic in a resource-limited setting. Asian J Transfus Sci [Epub ahead of print] [cited 2023 Mar 24]. Available from: https://www.ajts.org/preprintarticle.asp?id=356891 |
| Introduction | |  |
A rare blood group is defined when the phenotype occurs in 1/1000 population and includes either the absence of high-frequency antigen or multiple common antigens.[1] P-null phenotype individuals lack P1, P, and Pk antigens and develop a naturally occurring antibody directed to the high-frequency antigen PP1Pk complex, which has the potential to cause hemolytic transfusion reactions.
The prevalence of P null in the European population was estimated as low as 1/5.8 million, while it is unknown for the Indian population.[2] In India, only two cases were confirmed by molecular analysis. This case report is about the second case from India reported earlier by Kanani et al.[3] We report the challenges in transfusion management of a child requiring repeated transfusions among the unprecedented COVID-19 pandemic restrictions, which added to the logistical difficulties.
| Case Report | | |
A 5-year-old girl sustained a left fronto-temporo-parietal subdural hematoma following a fall from height and underwent craniectomy. She had cranioplasty with titanium mesh placement for the large skull defect. While she was getting treatment at another center, it was noticed that crossmatch compatible unit could not be obtained due to the presence of a panreactive alloantibody. Following this, she was identified as a rare P-null phenotype with anti-PP1Pk antibody.[3] In view of severe anemia (Hb 3.8 g/dl) and the nonavailability of compatible blood, she was transfused a small volume of least incompatible blood to which she showed features of hemolytic transfusion reaction.[3] Later, she developed wound dehiscence and presented with surgical site infection and titanium mesh exposure to our center in South India.
The patient required a microsurgical free-tissue transfer for covering the exposed implant. None of her immediate and extended family members possessed P-null phenotype. As it was challenging to find compatible blood, autologous blood donation was considered, and an elective surgical procedure was planned after 4 weeks. Her hemoglobin (11.5 g/dl) and hematocrit (35%) met the requirement for autologous donation. As peripheral veins were thin, blood collection was performed through a central venous catheter under general anesthesia while undergoing wound debridement. About 10% of her blood volume, 120 ml, was harvested through the femoral catheter. The autologous collection as well as her subsequent wound debridement dropped her hemoglobin to 9 g/dL. Iron supplementation was given at a dose of 10 mg/kg daily along with a diet rich in Vitamin C, iron, protein, and carbohydrates.
Injection erythropoietin (EPO) beta 25 IU/kg (400 IU) was given on day 1 and day 4 after autologous donation. The patient was planned for three autologous collections at weekly intervals. However, the second collection planned on day 7 was deferred due to hemoglobin < 11 g/dL. Despite increasing the EPO dose to 1000 IU on days 7, 9, and 11, her hemoglobin was persistently < 9.5 g/dl. Hence, further doses of EPO and autologous collections were withheld. The surgery for the scalp defect was performed after 3 weeks and the stored autologous blood was transfused. She continued to take iron supplementation and an iron-rich diet as she had to undergo further corrective surgeries.
The search for P-null donors was initiated with rare donor registries within the country and globally. Frozen units from international registries were not considered as our center did not have a facility for deglycerolization. The Indian Council of Medical Research-National Institute of Immunohematology, Mumbai, the reference laboratory in India provided the details of a P-null donor. The donor was traced to a remote village in Western India, 1600 km away from our blood center. Due to interstate travel restrictions during the first wave of COVID-19, the donor was transported to a neighboring blood center around 100 km away from his village. The patient's sample was sent to this blood center for pretransfusion testing and the unit was found compatible. The blood unit was transported maintaining the cold chain and stored at our center. The surgery was uneventful, and the blood unit was transfused for postoperative anemia (Hb: 6.8 g/dl). The patient required repeat surgery after 4 months for the removal of titanium mesh as it was repeatedly exposed and infected. Testing of the previous donor extended family for P-null phenotype was deferred due to logistic reasons and the blood was collected from the same donor after multiple counseling sessions. The patient required a blood transfusion during the surgery and was discharged with a plan for autograft cranioplasty after 6 months. On her last follow-up visit, her hemoglobin was 12.6 g/dl, and she was doing well at the time of manuscript submission.
| Discussion | | |
It is always a challenging task to find compatible blood for rare blood group phenotypes such as P null. Anti-PP1Pk is an antibody that reacts with red cells expressing high-prevalence antigens P and P1 Pk antigens.
Screening of family members is usually the first and most feasible option to find compatible blood; however, it was not fruitful in our case. Autologous blood donations are not commonly performed in the pediatric age group due to the low blood volume, small blood collection sets, difficult peripheral venous access, and patient noncooperation.[4] The British Committee for Standards in Haematology (BCSH) guidelines recommend that child shall be able to comprehend and cooperate with the donation process and to obtain parental consent.[5] In our case, the 5-year-old child was cooperative and consent from the parent was obtained. The lower age limit for autologous blood donation was not addressed and hence the decision to collect blood was made based on her clinical condition and the rarity of the blood group.
Intraoperative blood salvage was not considered in this case as the wound was infected. Bacterial infection, low ferritin, and hyperparathyroidism in children can be attributed to poor hemoglobin improvement following the EPO.[6] With preoperative anemia and poor response to EPO, the required number of autologous blood units was not obtained before surgery in this child. In such situations, collaboration with national and international reference laboratories and registries is required to help meet the blood requirements. Facilities for freezing and thawing red blood cells have to be standardized to make use in critical situations at a regional level.
| Conclusion | | |
Well-planned patient blood management strategies by the multidisciplinary team can manage the repeated transfusion needs of rare blood group patients. A centralized database of rare donors is the need of the hour and the collaboration of regional registries is essential for this. The role of rare donor registries is unparalleled when patients require urgent transfusions.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Acknowledgements
The authors would like to acknowledge Dr.SanmukhR.Joshi, Lok Samarpan Blood Bank, and Research Centre, Surat for guidance. The authors would like to acknowledge Dr. Swati Kulkarni, Scientist, ICMR-National Institute of immunohematology, Mumbai for providing the rare donor details and counseling the donor. We also immensely thank the blood donor. We thank Dr. Shashikant Patil & the blood bank staff at Arpan blood center, Nashik, and staff at Amrita Institute blood center.Authors acknowledge Dr Mohandoss Murugesan for reviewing the manuscript.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Hamilton JR, Bailey DJ. Identification of Antibodies to Red Cell Antigens.In Cohn CS, Delaney M, Johnson ST, Katz LM,. Tech. Manual, 20th ed., Bethesda, Maryland: AABB; 2020. P 423.  |
| 2. | Shastry S, Satyamoorthy K, Acharya K, Reddy VR, Mohan G, Deepika C, et al. Deletion in the A4GALT Gene Associated with Rare “P null” Phenotype: The First Report from India. Transfus Med Hemother 2020;47:186-9. |
| 3. | Kanani A, Senjaliya S, Rajapara M, Aeschlimann J, Westhoff C, Joshi S. 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. Transfus Med Hemother 2021;48:240-3. |
| 4. | Contreras M, Chapman CE. Autologous transfusion and reducing allogeneic blood exposure. Arch Dis Child 1994;71:105-7. |
| 5. | Voak D, Finney RD, Forman K, Kelsey P, Mitchell R, Murphy MF, et al. Guidelines for autologous transfusion. I. Preoperative autologous donation. Transfus Med 1993;3:307-16. |
| 6. | Van Damme-Lombaerts R, Herman J. Erythropoietin treatment in children with renal failure. Pediatr Nephrol 1999;13:148-52. |
Correspondence Address:
Veena Shenoy,
Department of Transfusion Medicine, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham, Ponekkara P. O., Kochi - 682 041, Kerala
India
 Source of Support: None, Conflict of Interest: None DOI: 10.4103/ajts.ajts_60_22
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