Medicine · 2026
Angioimmunoblastic T-cell lymphoma with secondary hemophagocytic lymphohistiocytosis initially misdiagnosed as classical Hodgkin lymphoma: A case report.
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Li Rui, Chen Yong, Liu Xuefen, Gao Yuying, Zhu Nengping, Deng Changyu, Li Yue
Аннотация
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.
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.