---
title: "Angioimmunoblastic T‑cell Lymphoma Complicated by Secondary HLH Initially Misdiagnosed as Classica"
id: "pubmed-42826277"
canonical_url: "https://medichelpline.com/clinical-feed/pubmed-42826277"
content_type: "clinical_feed_article"
specialty: "Oncology"
source_name: "PubMed / NCBI"
source_url: "https://pubmed.ncbi.nlm.nih.gov/42826277/"
doi: "10.1097/MD.0000000000050979"
published_at: "2026-10-02T00:00:00.000Z"
evidence_level: "Journal Article"
license: "CC-BY-NC-4.0 / Informational Use"
---
# Angioimmunoblastic T‑cell Lymphoma Complicated by Secondary HLH Initially Misdiagnosed as Classica
## Provenance & Clinical Metadata
- **Canonical URL:** https://medichelpline.com/clinical-feed/pubmed-42826277
- **Specialty:** [Oncology](https://medichelpline.com/clinical-feed/oncology.md)
- **Primary Source:** PubMed / NCBI
- **Source URL:** [Original Journal Publication](https://pubmed.ncbi.nlm.nih.gov/42826277/)
- **DOI:** [10.1097/MD.0000000000050979](https://doi.org/10.1097%2FMD.0000000000050979)
- **Published At:** 2026-10-02T00:00:00.000Z
- **Evidence Rating:** Journal Article
## Executive GIST (TL;DR)
- A 72-year-old man was initially diagnosed outside as having classical Hodgkin lymphoma based on an inguinal lymph node biopsy but later found to have **angioimmunoblastic T‑cell lymphoma (AITL)** on pathological reassessment. - The patient presented with progressive fatigue, recurrent fever, severe pancytopenia, and splenomegaly; laboratory testing showed hypertriglyceridemia and marked hyperferritinemia consistent with **secondary hemophagocytic lymphohistiocytosis (HLH)**. - Reevaluation of archived lymph node tissue demonstrated Epstein-Barr virus‑encoded RNA positivity, T‑cell receptor gene rearrangement, and an immunophenotype diagnostic of nodal T‑follicular helper cell lymphoma, angioimmunoblastic type, with scattered Hodgkin/Reed‑Sternberg‑like cells. - Plasma Epstein‑Barr virus DNA was present at low levels. The disease was staged as Lugano stage III with B symptoms and classified as high risk by the International Prognostic Index. - A stepwise treatment approach was used: initial **HLH-directed therapy** with **etoposide** and **dexamethasone** to control hyperinflammation, followed by lymphoma-directed chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone (**CHOP**). - After six cycles of CHOP chemotherapy the patient achieved partial remission, but later had disease progression with clinically suspected recurrent HLH despite salvage therapy and died on March 29, 2025. - The case highlights diagnostic pitfalls when AITL shows Hodgkin/Reed‑Sternberg‑like cells, the association with **Epstein‑Barr virus**, the risk of HLH in T‑cell lymphomas, and the clinical importance of early pathological reassessment and prioritizing control of hyperinflammation when clinical and pathological findings are discordant. - Authors reported no funding or conflicts of interest. The report was published in Medicine (Baltimore) with PMID 42826277 and DOI 10.1097/MD.0000000000050979.
## Clinical Analysis & Structured Key Points
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Affiliations Expand ### Affiliations * 1 Department of Oncology, The People's Hospital of Rongchang District, Chongqing, China. * 2 Department of Cardiology, The People's Hospital of Rongchang District, Chongqing, China. * PMID: **42826277** * DOI: [ 10.1097/MD.0000000000050979 ](https://doi.org/10.1097/md.0000000000050979) Item in Clipboard Case Reports # Angioimmunoblastic T-cell lymphoma with secondary hemophagocytic lymphohistiocytosis initially misdiagnosed as classical Hodgkin lymphoma: A case report Rui Li et al. Medicine (Baltimore). 2026. Show details Display options Display options Format Abstract PubMed PMID Medicine (Baltimore) Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Medicine+%28Baltimore%29%22%5Bjour%5D&sort=date&sort_order=desc) * [ Search in NLM Catalog ](https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22Medicine+%28Baltimore%29%22%5BTitle+Abbreviation%5D) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42826277/) . 2026 Oct 2;105(40):e50979. doi: 10.1097/MD.0000000000050979. ### Authors [Rui Li](https://pubmed.ncbi.nlm.nih.gov/?term=Li+R&cauthor_id=42826277)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42826277/#short-view-affiliation-1 "Department of Oncology, The People's Hospital of Rongchang District, Chongqing, China."), [Yong Chen](https://pubmed.ncbi.nlm.nih.gov/?term=Chen+Y&cauthor_id=42826277)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42826277/#short-view-affiliation-1 "Department of Oncology, The People's Hospital of Rongchang District, Chongqing, China."), [Xuefen Liu](https://pubmed.ncbi.nlm.nih.gov/?term=Liu+X&cauthor_id=42826277)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42826277/#short-view-affiliation-1 "Department of Oncology, The People's Hospital of Rongchang District, Chongqing, China."), [Yuying Gao](https://pubmed.ncbi.nlm.nih.gov/?term=Gao+Y&cauthor_id=42826277)[ 2 ](https://pubmed.ncbi.nlm.nih.gov/42826277/#short-view-affiliation-2 "Department of Cardiology, The People's Hospital of Rongchang District, Chongqing, China."), [Nengping Zhu](https://pubmed.ncbi.nlm.nih.gov/?term=Zhu+N&cauthor_id=42826277)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42826277/#short-view-affiliation-1 "Department of Oncology, The People's Hospital of Rongchang District, Chongqing, China."), [Changyu Deng](https://pubmed.ncbi.nlm.nih.gov/?term=Deng+C&cauthor_id=42826277)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42826277/#short-view-affiliation-1 "Department of Oncology, The People's Hospital of Rongchang District, Chongqing, China."), [Yue Li](https://pubmed.ncbi.nlm.nih.gov/?term=Li+Y&cauthor_id=42826277)[ 2 ](https://pubmed.ncbi.nlm.nih.gov/42826277/#short-view-affiliation-2 "Department of Cardiology, The People's Hospital of Rongchang District, Chongqing, China.") ### Affiliations * 1 Department of Oncology, The People's Hospital of Rongchang District, Chongqing, China. * 2 Department of Cardiology, The People's Hospital of Rongchang District, Chongqing, China. * PMID: **42826277** * DOI: [ 10.1097/MD.0000000000050979 ](https://doi.org/10.1097/md.0000000000050979) Item in Clipboard Cite Display options Display options Format Abstract PubMed PMID Linked Discoveries ## Abstract **Rationale:** Angioimmunoblastic T-cell lymphoma (AITL), classified as nodal T-follicular helper cell lymphoma, angioimmunoblastic type, is an aggressive lymphoma frequently complicated by immune dysregulation. Owing to overlapping histopathological features, particularly Hodgkin/Reed-Sternberg-like cells, AITL may be initially misdiagnosed as Hodgkin lymphoma, leading to delayed recognition and inappropriate management. **Patient concerns:** A 72-year-old man presented with progressive fatigue, recurrent fever, severe pancytopenia, and splenomegaly after an outside diagnosis of Hodgkin lymphoma based on inguinal lymph node biopsy. **Diagnoses:** Laboratory findings revealed hypertriglyceridemia and marked hyperferritinemia, consistent with secondary hemophagocytic lymphohistiocytosis (HLH). Reevaluation of archived lymph node tissue demonstrated Epstein-Barr virus-encoded RNA positivity, T-cell receptor gene rearrangement, and an immunophenotype diagnostic of nodal T-follicular helper cell lymphoma, angioimmunoblastic type with scattered Hodgkin/Reed-Sternberg-like cells. Plasma Epstein-Barr virus DNA was detected at low levels. The disease was staged as Lugano stage III with B symptoms and classified as high risk by the International Prognostic Index. **Interventions:** HLH-directed therapy with etoposide and dexamethasone was initiated for rapid disease control, followed by cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy for lymphoma treatment. **Outcomes:** Partial remission was achieved after 6 cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. The patient later experienced disease progression with clinically suspected recurrent HLH despite salvage therapy and died on March 29, 2025. **Lessons:** This case underscores the diagnostic pitfalls of AITL complicated by HLH and highlights the importance of early pathological reassessment and a stepwise treatment approach prioritizing control of hyperinflammation in patients with discordant clinical and pathological findings. **Keywords:** Epstein–Barr virus; angioimmunoblastic T-cell lymphoma; dexamethasone; etoposide; hemophagocytic lymphohistiocytosis. Copyright © 2026 the Author(s). Published by Wolters Kluwer Health, LLC. [PubMed Disclaimer](https://pubmed.ncbi.nlm.nih.gov/disclaimer/) ## Conflict of interest statement The authors have no funding and conflicts of interest to declare. ## References 1. 1. Zhang Q, Yin L, Lai Q, Zhao Y, Peng H. Advances in the pathogenesis and therapeutic strategies of angioimmunoblastic T-cell lymphoma. Clin Exp Med. 2023;23:4219–35. 2. 1. Mohammed Saleh MF, Kotb A, Abdallah GEM, Muhsen IN, El Fakih R, Aljurf M. Recent advances in diagnosis and therapy of angioimmunoblastic T cell lymphoma. Curr Oncol. 2021;28:5480–98. 3. 1. Lunning MA, Vose JM. Angioimmunoblastic T-cell lymphoma: the many-faced lymphoma. Blood. 2017;129:1095–102. 4. 1. Al-Samkari H, Berliner N. Hemophagocytic lymphohistiocytosis. Annu Rev Pathol. 2018;13:27–49. 5. 1. Griffin G, Shenoi S, Hughes GC. Hemophagocytic lymphohistiocytosis: an update on pathogenesis, diagnosis, and therapy. Best Pract Res Clin Rheumatol. 2020;34:101515. 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