A rare case of diffuse large B-cell lymphoma-associated hemophagocytic lymphohistiocytosis

Abstract

Introduction Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. Herein, we aimed to report a diffuse large B-cell lymphoma (DLBCL) case that was presented as HLH. Case report A 32-year-old man presented to a hospital with complaints of vomiting, nausea and diarrhea in October 2019. Fever and hepatosplenomegaly was detected in physical investigation. Bone marrow aspiration investigation revealed the hemaphagocytosis. HLH-2004 protocol was started for hemophagocytosis. Whole body magnetic resonance imaging (MR) revealed no lymphadenopathy. Bone marrow biopsy revealed high-grade B-cell lymphoma, favoring DLBCL. There were no pathologic cells in lumber puncture investigation. Management and outcome He was diagnosed with secondary hemaphagocytic syndrome due to DLBCL, and chemotherapy was switched to rituximab, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin (R-EPOCH) regimen. After three cycles of R-EPOCH chemotherapy regimen, complete remission was confirmed with positron emission tomography-computerised tomography (PET-CT) scan. Discussion Our patients’ findings are suitable for six out of eight criteria of hemaphagocytic syndrome. The H-score of our patient was more than 250, reflecting the >99% probability of HLH syndrome. Compatible with literature knowledge, our patient had responded very well to etoposide-containing regimens. In our patient, no lymphadenopathy was detected by physical examination or MR scan, and the diagnosis of DLBCL was only made by the result of bone marrow investigation. In conclusion, herein, we have reported a DLBCL case that had presented with HLH, and clinicians should be aware that B-cell lymphomas may be the underlying cause of HLH.

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

“…The literature review performed for the present study showed recently reported cases of DLBCL with HLH, which were similar to the clinical manifestation of the current patient, but none of them presented with plasmacytic differentiation and MYD88 L265P mutation. Specifically, after R-CHOP chemotherapy, few patients achieved remission ( 23 , 24 ), and others achieved transient remission but relapsed soon after or died immediately ( 25 , 26 ). In the summary of a clinical case series, researchers suggested that after the combination of HLH-directed therapy and malignancy-focused interventions, treatment should be decided on a case-by-case basis ( 27 ).…”

Cd5+ Diffuse Large B Cell Lymphoma of the Liver Presenting as Cholestatic Transaminitis.

“…EBV is a less common cause of non-GCB type DLBCL and has been associated with EBV-positive DLBCL of the elderly along with 5 to 10% of DLBCL not otherwise speci ed [4]. These infections can set off immune cascades in the body triggering sporadic cases of secondary HLH [5]. HLH is a life-threatening disorder of immune activation known to be caused by hematologic malignancies, most commonly NK/T-cell lymphomas [6] however it can be uncommonly associated with non-Hodgkin's B-cell lymphomas [7].…”

“…Computed tomography of the abdomen and pelvis with contrast showed splenomegaly measuring 18. 5 *EBV viral capsid antibody (VCA) IgM negative <36, EBV VCA antibody IgG positive >21.9, EBV nuclear antigen antibody negative <18, EBV early antigen IgG positive > 10.9…”

Clinical features and prognostic analysis of lymphoma‑associated hemophagocytic syndrome: A report of 139 cases

“…The 5-year OS and RFS of patients who received chemotherapy were significantly higher than those in the patients who did not receive chemotherapy. The aggressive treatment of primary tumors in cases of tumor-associated HPS has been recommended (13,27,31). Although the current study focused on a small number of patients, it indicates that chemotherapy for lymphoma has great potential in the treatment of LAHS, and can improve patient prognosis.…”