EBV Associated Post-Transplant Lymphoproliferative Disorder Complicated with Haemophagocytic Lymphohistiocytosis After Allogeneic Stem Cell Transplant: A Rare Complication with Fatal Outcome

Authors

  • Tangia Muquith

DOI:

https://doi.org/10.37545/haematoljbd202064

Keywords:

EBV (Epstein –Barr virus), PTLD (Post transplant lymphoproliferative disorder), HLH (Haemophagocytic lymphohistiocytosis), ATG (Anti thymocyte globulin)

Abstract

Epstein –Barr virus related post-transplant lymphoproliferative disorder is a fatal and life-threatening complication because of immunocompromised state.1 Haemophagocytic lymphohistiocytosis is a sign of poor outcome in EBV associated PTLD after allogeneic stem cell transplantation. It is particularly common when in vivo T cell depletion strategies have been applied.2 In both situations, post-transplant lymphoproliferative disorder and Haemophagocytic lymphohistiocytosis, infection with EBV is the key mechanism. Here I present a case of 29 years old female with acute myeloid leukaemia after second allogeneic stem cell transplant, who developed PTLD complicated with Haemophagocytic lymphohistiocytosis secondary to Epstein – Barr virus (EBV) infection. Patient was treated with chemo immunotherapy and responded but ultimately died after 100 days of transplant. The association of HLH and EBV related PTLD is rare and data on outcome of these patients are limited with very high mortality.

Author Biography

Tangia Muquith

Department of Haematology-Oncology, National University Cancer Institute, Singapore

References

Helen E. Heslop. How I treat EBV lymphoproliferation. Blood. 2009; 114:002-4008.

Joost W. J. van Esser, Bronno van der Holt, Jan J. Cornelison. Epstein –Barr virus (EBV) reactivation is a frequent event after allogeneic stem cell transplantation (SCT) and quantitatively predicts EBV lymphoproliferative disease following T cell depleted SCT. Blood. (2001) 98 (4): 972-978.

Tasso L. His ED. The clinic pathologic spectrum of post transplantation lymphoproliferative disorders. Arch Pathol Lab Med. 2007; 131: 1209-1218.

Dierickx D, Tousseyn T, Gheysens O. How I treat posttransplant lymphoproliferative disorder. Blood 2015; 126: 2274- 2283.

Robert D Sandler, Rachel Scarlett. Diagnosis and management of secondary HLH/MAS following HSCT and CAR-T cell therapy in Adults; A review of the literature and a Survey of Practice within EBMT Centres. Front Immunol. 2020;11;524

Lehmberg K, Sprekels B, Nicholas KE. Malignancy associated Haemophagocytic lymphohistiocytosis in children and adolescents. Br J Haematol. 2015; May 4.

Kai Lehemberg, Kim E. Nicholas. Sheila Weitzman. Consensus recommendations for the diagnosis and management of Haemophagocytic lymphohistiocytosis associated with malignancies. Haematologica. 2015 Aug; 100(8): 997-1004

Amrita Desai, Agustin Pmentel. Adult Lymphoma associated Haemophagocytic Lymphohistiocytosis: A clinical case series in a predominantly Hispanic Cohort. J Med Cases.2020;11(8):256-261

J J Boelens, G Lazo, N M Walfraat. Epstein – Barr Virus associated Haemophagocytic lymphohistiocytosis after stem cell transplantation. Bone Marrow Transplantation.2006; 38:709-710.

S. Ali, A. puneet. Hemophagocytic lymphohistiocytosis is a sign of poor outcome in pediatric Epstein Barr Virus associated post- transplant lymphoproliferative disease after allogeneic hematopoietic stem cell transplantation. Paediatr Transplant. 2019 Feb;23(1)

Downloads

Published

2020-12-31

How to Cite

1.
Muquith T. EBV Associated Post-Transplant Lymphoproliferative Disorder Complicated with Haemophagocytic Lymphohistiocytosis After Allogeneic Stem Cell Transplant: A Rare Complication with Fatal Outcome. Haematol J Bangladesh [Internet]. 2020Dec.31 [cited 2021Feb.26];4(02):49-51. Available from: https://journal.hematologybd.org/index.php/haematoljbd/article/view/64

Issue

Section

Case Report(s)

Most read articles by the same author(s)

Similar Articles

You may also start an advanced similarity search for this article.