Lymphoproliferation in Inborn Errors of Immunity : The Eye Does Not See What the Mind Does Not Know
Copyright © 2022 Sharma, Pilania, Anjani, Sudhakar, Arora, Tyagi, Dhaliwal, Vignesh, Rawat and Singh..
Inborn errors of immunity (IEIs) are a group of heterogeneous disorders characterized by a broad clinical spectrum of recurrent infections and immune dysregulation including autoimmunity and lymphoproliferation (LP). LP in the context of IEI may be the presenting feature of underlying immune disorder or may develop during the disease course. However, the correct diagnosis of LP in IEI as benign or malignant often poses a diagnostic dilemma due to the non-specific clinical features and overlapping morphological and immunophenotypic features which make it difficult to treat. There are morphological clues to LP associated with certain IEIs. A combination of ancillary techniques including EBV-associated markers, flow cytometry, and molecular assays may prove useful in establishing a correct diagnosis in an appropriate clinical setting. The present review attempts to provide comprehensive insight into benign and malignant LP, especially the pathogenesis, histological clues, diagnostic strategies, and treatment options in patients with IEIs.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:13 |
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| Enthalten in: |
Frontiers in immunology - 13(2022) vom: 21., Seite 856601 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Sharma, Saniya [VerfasserIn] |
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| Links: |
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| Themen: |
Ig/TCR gene rearrangements |
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| Anmerkungen: |
Date Completed 24.05.2022 Date Revised 16.07.2022 published: Electronic-eCollection Citation Status MEDLINE |
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| doi: |
10.3389/fimmu.2022.856601 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM341257540 |
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| 520 | |a Copyright © 2022 Sharma, Pilania, Anjani, Sudhakar, Arora, Tyagi, Dhaliwal, Vignesh, Rawat and Singh. | ||
| 520 | |a Inborn errors of immunity (IEIs) are a group of heterogeneous disorders characterized by a broad clinical spectrum of recurrent infections and immune dysregulation including autoimmunity and lymphoproliferation (LP). LP in the context of IEI may be the presenting feature of underlying immune disorder or may develop during the disease course. However, the correct diagnosis of LP in IEI as benign or malignant often poses a diagnostic dilemma due to the non-specific clinical features and overlapping morphological and immunophenotypic features which make it difficult to treat. There are morphological clues to LP associated with certain IEIs. A combination of ancillary techniques including EBV-associated markers, flow cytometry, and molecular assays may prove useful in establishing a correct diagnosis in an appropriate clinical setting. The present review attempts to provide comprehensive insight into benign and malignant LP, especially the pathogenesis, histological clues, diagnostic strategies, and treatment options in patients with IEIs | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Review | |
| 650 | 4 | |a Ig/TCR gene rearrangements | |
| 650 | 4 | |a immunodeficiency | |
| 650 | 4 | |a inborn errors of immunity (IEI) | |
| 650 | 4 | |a lymphoma | |
| 650 | 4 | |a lymphoproliferation | |
| 700 | 1 | |a Pilania, Rakesh Kumar |e verfasserin |4 aut | |
| 700 | 1 | |a Anjani, Gummadi |e verfasserin |4 aut | |
| 700 | 1 | |a Sudhakar, Murugan |e verfasserin |4 aut | |
| 700 | 1 | |a Arora, Kanika |e verfasserin |4 aut | |
| 700 | 1 | |a Tyagi, Rahul |e verfasserin |4 aut | |
| 700 | 1 | |a Dhaliwal, Manpreet |e verfasserin |4 aut | |
| 700 | 1 | |a Vignesh, Pandiarajan |e verfasserin |4 aut | |
| 700 | 1 | |a Rawat, Amit |e verfasserin |4 aut | |
| 700 | 1 | |a Singh, Surjit |e verfasserin |4 aut | |
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