A Rare Case of Grey Zone Lymphoma Successfully Treated with Brentuximab Vedotin and R-CHP Chemotherapy

Abstract

Introduction. The diagnosis of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and classical Hodgkin’s lymphoma (cHL), also referred to as grey zone lymphoma (GZL), is a challenging diagnosis. There are no standardized guidelines; however, evidence strongly suggests that DLBCL-based regimens are effective in the treatment of GZL. Brentuximab vedotin (BV) is an anti-CD30 antibody drug conjugate that has established efficacy in relapsed/refractory Hodgkin and some T-cell lymphomas. There is some evidence that BV has a positive response in non-Hodgkin lymphoma (NHL) with a wide range of CD30 expressions—including GZL.Case. We present a case of a patient initially diagnosed with cHL who underwent repeat biopsy which was revealed to be GZL. Based on PET scanning and immunohistochemical studies, she was classified as a stage IIIA CD20+/CD30+ GZL patient. Given her strong CD30 expression, she underwent 6 cycles of R-BV-CHP (rituximab, brentuximab vedotin, cyclophosphamide, doxorubicin, and prednisone) chemotherapy and achieved complete response (CR) both clinically and radiographically.Discussion. Given the rarity of GZL, this case illustrates the immense challenges in making the diagnosis, discusses the current treatment options, and suggests that BV may be a viable therapeutic candidate in the treatment of GZL.

Transition Between Diffuse Large B-Cell Lymphoma and Classical Hodgkin Lymphoma– Our Histopathological and Clinical Experience With Patients With Intermediate Lymphoma

“…Extensive study of histological changes is indispensible for even an experienced hematologist hence core biopsy sampling often has limited value in the diagnosis of GZL, and excision of the complete lymph node is preferred. What may alert the pathologist is the contrasting morphological and immunohistological pictures, which can warrant detailed study, possibly further immunostaining or indicate repeated sampling [6][7][8]. In our patients the two patterns could be clearly distinguished with initial DLBCL morphology in 6 cases and cHL (morphology) in 3.…”

“…The disease can be managed using DLBCL-type (R-CHOP, DA-EPOCH-R), or cHL-type (ABVD, escBEACOPP) protocols. Literature data and studies with larger numbers of cases show that DLBCL-type treatments appear to be more effective [3][4][5][6][7][8]. However, experts' opinions on the use of rituximab are less clear: while Evens et al [3] found therapies with additional rituximab more favourable in terms of progression-free survival, Sarkozy et al [5] did not find rituximab-containing protocols more favourable either for event-free or for overall survival.…”

“…During their first treatment a total of 6 patients received rituximab; besides the two HL patients treated with ABVD there was one more patient who did not receive rituximab, she was initially diagnosed with GZL and was treated with brentuximab-vedotin in addition to the CHOP regime due to CD20 negativity and strong CD30 positivity. Scarce data are available on the use of brentuximab vedotin; one case has been published where BV was combined with R-CHP [7], and in a phase I/II clinical trial PMBL and DLBCL patients also received the same therapeutic combination (BV + R-CHP) [9]. Brentuximab vedotin can be effective in CD30 positive DLBCL cases [10].…”

“…When gray zone lymphoma was first described, the process was mainly believed to be of mediastinal localization, affecting mostly young, male patients. However, literature reports published in recent years have shown that the non-mediastinal form is just as, if not more common than the cases with primarily mediastinal involvement [ 3 , 4 , 7 ]. There are differences in the features of the two clinical presentations: while patients with MGZ tend to be younger, at earlier stages of the disease and often have bulky tumors, cases with NMGZ lymphoma are characterized by older age, more advanced stages of the disease, extensive extranodal involvement, and frequent bone marrow infiltration.…”

Gray zone lymphoma effectively treated with cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab chemotherapy: A case report

“…Here, we report the case of a patient with GZL, which is rare in Asian populations[ 6 - 9 ], who was successfully treated with cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab (R-CHOP) chemotherapy.…”