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CC BY-NC-ND 4.0 · J Lab Physicians 2023; 15(02): 316-320
DOI: 10.1055/s-0042-1757584
Case Series

Pure Red Cell Aplasia Encountered in a Tertiary Care Hematology Laboratory: A Series of Nine Distinctive Cases

Mansi Kala
1   Department of Pathology, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Jolly Grant Dehradun, Uttarakhand, India
,
Kunal Das
2   Division of Pediatric Oncology and Bone Marrow Transplantation, Department of Pediatrics, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Dehradun, Uttarakhand, India
,
Avriti Baveja
3   Division of Hemato-oncology and Bone Marrow Transplantation, Department of Medicine, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Dehradun, Uttarakhand, India
,
Manish Raturi 
4   Department of Immunohematology and Blood Transfusion, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Jolly Grant Dehradun, Uttarakhand, India
,
Meghali Dhebane
1   Department of Pathology, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Jolly Grant Dehradun, Uttarakhand, India
,
Sohaib Ahmad
5   Department of Medicine, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Jolly Grant Dehradun, Uttarakhand, India
,
Mansi Mehrotra
1   Department of Pathology, Himalayan Institute of Medical Sciences, Swami Rama Himalayan University, Jolly Grant Dehradun, Uttarakhand, India
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Abstract

Pure red cell aplasia (PRCA) is characterized by severe anemia with reticulocytopenia and bone marrow erythroblastopenia. The early erythroblasts are markedly decreased; however, in rare instances, they may be normal or raised in number. There are varied etiologies, namely congenital or acquired and primary or secondary. The congenital PRCA is known as “Diamond-Blackfan anemia.” Thymomas, autoimmune disease, lymphomas, infections, and drugs also may be familiar associates. However, the etiologies of PRCA are numerous, and many diseases/infections can be associated with PRCA. The diagnosis rests on clinical suspicion and appropriate laboratory workup. We evaluated nine cases of red cell aplasia, having severe anemia with reticulocytopenia. Nearly half of the cases showed adequate erythroid (> 5% of the differential count) but with a maturation arrest. The adequacy of the erythroid could confuse the hematologist and may even delay the diagnosis. Hence, it is empirical that PRCA could be considered a differential in every case of severe anemia with reticulocytopenia, even in the presence of adequate erythroid precursors in the bone marrow.

Keywords

Diamond-Blackfan anemia - red cell transfusion - erythroblasts - severe anemia

Informed Consent

As per our hospital transfusion policy, an informed consent is obtained from all blood recipients prior to blood administration.


Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the Institutional Ethics Committee [Ref No. SRHU/HIMS/RC/2022/156 dated 27/04/22] and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The article does not contain any regulated animal related research.


Authors' Contributions

M.K. and M.R. designed the concept. M.K., M.R., and K.D. did the literature search. M.K. and M.D. compiled the data. M.K., K.D., M.R., and A.B. prepared and edited and reviewed the manuscript. M.K., M.R., M.M., and S.A. reviewed the manuscript. M.R., as a guarantor, takes the responsibility of the integrity of the work, as a whole, right from its inception to being a published article.




Publication History

Article published online:
20 October 2022

© 2022. The Indian Association of Laboratory Physicians. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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