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Open Access 01-12-2024 | Thalassemia | Original Article

Neuroimaging features of primary central nervous system post-transplantation lymphoproliferative disorder following hematopoietic stem cell transplant in patients with β-thalassemia: a case series and review of literature

Authors: Xueqing Yang, Xi Deng, Meiqing Wu, Sean W. Chen, Muliang Jiang, Liling Long, Bihong T. Chen

Published in: Insights into Imaging | Issue 1/2024

Abstract

Purpose

Primary central nervous system post-transplantation lymphoproliferative disorder (PCNS-PTLD) is a rare but serious complication of hematopoietic stem cell transplantation (HSCT) in patients with severe β-thalassemia. This study aimed to assess the clinical presentation, pathological characteristics, neuroimaging findings, and treatment strategies in patients with β-thalassemia who developed PCNS-PTLD and to compare a case series from our transplant center to reported cases from literature.

Methods

We retrospectively reviewed our hospital database and identified four cases of pathologically confirmed PCNS-PTLD without a history of systemic PTLD in patients with severe β-thalassemia after HSCT. We also performed a relevant literature review on PCNS-PTLD.

Results

The median time from transplantation to diagnosis of PCNS-PTLD was 5.5 months. Intracerebral lesions were usually multiple involving both supratentorial and infratentorial regions with homogeneous or rim enhancement. All patients had pathologically confirmed PCNS-PTLD with three patients having diffuse large B-cell lymphoma and the fourth patient having plasmacytic hyperplasia. There was low response to treatment with a median survival of 83 days.

Conclusion

PCNS-PTLD should be considered in the differential diagnosis of patients with β-thalassemia who had an intracranial lesion on neuroimaging after HSCT.

Critical relevance statement

This case series with a comprehensive review of neuroimaging and clinical characteristics of children with primary central nervous system post-transplantation lymphoproliferative disorder should advance our understanding and improve management of this rare yet severe complication following transplant for β-thalassemia.

Key points

• We assessed clinical presentation, treatment strategies, and neuroimaging characteristics of PCNS-PTLD in patients with β-thalassemia after transplantation.

• Patients with β-thalassemia may have post-transplantation lymphoproliferative disorder presenting as brain lesions on neuroimaging.

• Neuroimaging findings of the brain lesions are helpful for prompt diagnosis and proper management.

Graphical Abstract

Available only for authorised users

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Title: Neuroimaging features of primary central nervous system post-transplantation lymphoproliferative disorder following hematopoietic stem cell transplant in patients with β-thalassemia: a case series and review of literature
Authors: Xueqing Yang
Xi Deng
Meiqing Wu
Sean W. Chen
Muliang Jiang
Liling Long
Bihong T. Chen
Publication date: 01-12-2024
Publisher: Springer Vienna
Keywords: Thalassemia
Stem Cell Transplantation
Stem Cell Transplantion
Stem Cell Transplantation
Magnetic Resonance Imaging
Magnetic Resonance Imaging
Cranial MRI
Published in: Insights into Imaging / Issue 1/2024
Electronic ISSN: 1869-4101
DOI: https://doi.org/10.1186/s13244-024-01605-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Neuroimaging features of primary central nervous system post-transplantation lymphoproliferative disorder following hematopoietic stem cell transplant in patients with β-thalassemia: a case series and review of literature

Abstract

Purpose

Methods

Results

Conclusion

Critical relevance statement

Key points

Graphical Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

Critical relevance statement

Key points

Graphical Abstract

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