Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 26, 2021; 9(30): 9159-9167
Published online Oct 26, 2021. doi: 10.12998/wjcc.v9.i30.9159
Hemophagocytic lymphohistiocytosis secondary to composite lymphoma: Two case reports
Jing Shen, Jing-Shi Wang, Jian-Lan Xie, Lin Nong, Jia-Ning Chen, Zhao Wang
Jing Shen, Jing-Shi Wang, Zhao Wang, Department of Hematology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing 100050, China
Jian-Lan Xie, Department of Pathology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing 100050, China
Lin Nong, Department of Pathology, Peking University First Hospital, Beijing 100034, China
Jia-Ning Chen, Department of Clinical Laboratory, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing 100000, China
Author contributions: Shen J and Wang JS conceived the topic, designed the study and revised the manuscript under the supervision of Wang Z; Shen J contributed to manuscript writing; Nong L contributed to data curation; Xie JL and Chen JN analyzed the data.
Informed consent statement: Consent was obtained from relatives of the patients for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Zhao Wang, MD, Professor, Department of Hematology, Capital Medical University Affiliated Beijing Friendship Hospital, No. 95 Yongan Road, Xicheng District, Beijing 100050, China. wangzhao@ccmu.edu.cn
Received: April 1, 2021
Peer-review started: April 1, 2021
First decision: April 28, 2021
Revised: May 19, 2021
Accepted: July 2, 2021
Article in press: July 2, 2021
Published online: October 26, 2021
Abstract
BACKGROUND

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disease caused by inherited pathogenic mutations and acquired dysregulations of the immune system. Composite lymphoma is defined as two or more morphologically and immunophenotypically distinct lymphomas that occur in a single patient. Here, we report two cases of HLH secondary to composite lymphoma with mixed lineage features of T- and B-cell marker expression both in the bone marrow and lymph nodes in adult patients.

CASE SUMMARY

Two patients were diagnosed with HLH based on the occurrence of fever, pancytopenia, lymphadenopathy, splenomegaly, hemophagocytosis and hyperferritinemia. Immunohistochemical staining of the axillary lymph node and bone marrow in case 1 showed typical features of combined B-cell and T-cell lymphoma. In addition, a lymph node gene study revealed rearrangement of the T-cell receptor chain and the immunoglobulin gene. Morphology and immunohistochemistry studies of a lymph node biopsy in case 2 showed typical features of T cell lymphoma, but immunophenotyping by flow cytometry analysis of bone marrow aspirate showed B cell lymphoma involvement. The patients were treated with high-dose methylprednisolone combined with etoposide to control aggressive HLH progression. The patients also received immunochemotherapy with the R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) regimen immediately after diagnosis. Both patients presented with highly aggressive lymphoma, and died of severe infection or uncontrolled HLH.

CONCLUSION

We present two rare cases with overwhelming hemophagocytosis along with composite T- and B-cell lymphoma, which posed a diagnostic dilemma. HLH caused by composite lymphoma was characterized by poor clinical outcomes.

Keywords: Hemophagocytosis, Hemophagocytic lymphohistiocytosis, Composite lymphoma, T-cell, B-cell, Case report

Core Tip: Lymphoma-associated hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening clinical disorder. In particular, composite lymphoma with mixed lineage features of T- and B-cell marker expression is extremely rare. We report two cases of HLH secondary to bi-lineage composite lymphoma with lymphocyte infiltrations both in the bone marrow and lymph nodes in adult patients. Case 1 showed typical features of combined B-cell and T-cell lymphoma in axillary lymph node and bone marrow biopsies. Case 2 showed typical features of T cell lymphoma in lymph node biopsy, and B cell lymphoma in bone marrow biopsy. Both patients highlight the diagnostic dilemma, therapeutic challenges and poor prognosis of HLH secondary to composite lymphoma.