How We Managed Primary Immunodeficiency Disorders in Pakistan – A Case Based Study

Authors

  • Asifa Noor Department of Pediatric Hematology, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan
  • Mehjabeen Imam Department of Transplant Immunology, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan.
  • Tasneem Farzana Department of Clinical Hematology, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan.
  • Saqib Hussain Ansari Department of Pediatric Hematology, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan.
  • Uzma Zaidi Department of Clinical Hematology, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan.
  • Saima Munzir Department of Clinical Hematology, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan.
  • Tahir Sultan Shamsi Department of Clinical Hematology, National Institute of Blood Disease & Bone Marrow Transplantation, Karachi, Pakistan.

Keywords:

Primary immunodeficiency disorder, Severe combined immunodeficiency, Hematopoietic stem cell transplantation, Graft versus host disease

Abstract

Introduction: This article emphasizes on the importance of time frame for diagnosis and management of immunodeficiency syndromes and also highlights the diagnostic dilemma that most often such cases are misdiagnosed or undiagnosed which contributes to significant morbidity and mortality. Case Presentation: This article with the help of five exemplary cases illustrates the different types of primary immunodeficiencies including severe combined immunodeficiency (SCID), and their varied clinical presentations and management and also describes the difficulty in reaching final diagnosis and a brief review of literature on primary immunodeficiency and its therapeutic approach. Management and Outcome: All the patients described in this case based study underwent Hematopoietic stem cell transplant; the definitive therapeutic approach for all kinds of immunodeficiency syndromes. Four out of five patients were successfully treated, unfortunately one could not survive. Discussion: The purpose of this article is to raise public health awareness regarding prevalence of primary immunodeficiency disorders and their possible cure via hematopoietic stem cell transplant, if diagnosed and managed promptly.

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Published

2020-12-22

How to Cite

1.
Noor A, Imam M, Farzana T, Ansari SH, Zaidi U, Munzir S, Shamsi TS. How We Managed Primary Immunodeficiency Disorders in Pakistan – A Case Based Study. Nat J Health Sci [Internet]. 2020Dec.22 [cited 2023Feb.7];4(1):34-41. Available from: https://ojs.njhsciences.com/index.php/njhs/article/view/64

Issue

Section

Case Report

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