Hemophagocytic lymphohistiocytosis as an onset of diffuse large B cell lymphoma: A case report

• Main Authors: Cao, Y. (Author), Fu, G. (Author), Zhao, X. (Author), Zhou, H. (Author), Zou, L. (Author)
• Format: Article
• Language: English
• Published: Spandidos Publications 2022
• Subjects: bone marrow biopsy; bortezomib; diffuse large Bcell lymphoma; hemophagocytic lymphohistiocytosis; myeloid differentiation primary response 88 L265P; NFκB signaling pathway
• Online Access: View Fulltext in Publisher

 A 53yearold male presented with a 1month history of hyperpyrexia. The clinical manifestations revealed hemo phagocytic lymphohistiocytosis (HLH). Although a lymph node biopsy could not be obtained, a bone marrow biopsy revealed the activated Bcell subtype of diffuse large Bcell lymphoma (DLBCL). After being treated with HLH1994 (dexamethasone and etoposide), a rituximabcontaining chemotherapy and target agents involving bortezomib, the patient achieved remission. To understand the molecular profile of patient, nextgeneration sequencing and MYD88 L265P mutation examinations were performed, and the patient was determined to be positive for the MYD88 L265P muta tion. Reports of DLBCL with plasmacytic differentiation and a MYD88 innate immune signal transduction adaptor L265P mutation concurrent with HLH are rare. Early recognition, precise diagnosis and timely therapy are pivotal in improving patient prognosis. Furthermore, molecular profiling enables researchers to develop potential therapies aimed at the acti vated NFκB and endoplasmic reticulum stress signaling pathways. The present study highlights this pathogenesis and provides suggestions for further individualized therapeutics. © 2022 Spandidos Publications. All rights reserved.