Composite Blastoid Variant of Mantle Cell Lymphoma and Classical Hodgkin Lymphoma

Abstract

Composite lymphoma (CL) describes the rare occurrence of 2 or more distinct types of lymphoma in a single anatomical location. We present the case of a 78-year-old man presenting with a 3-month history of weakness, malaise, and increasing dyspnea. A lymph node excised from the posterior triangle of the neck revealed the coexistence of 2 morphologically and phenotypically distinct lymphoid neoplasms consistent with a blastoid variant of mantle cell lymphoma (MCL) occurring in composite with classical Hodgkin lymphoma (cHL), mixed cellularity subtype. A t(11;14)(q13;q32) translocation was demonstrated by fluorescence in situ hybridization in the MCL and Hodgkin Reed-Sternberg cells of the cHL. Multiplex polymerase chain reaction detected clonal Immunoglobulin heavy chain (VFR1-J, VFR2-J, and VFR3-J), clonal immunoglobulin light chain kappa (V-J and V/JC intron-kde) and clonal immunoglobulin light chain lambda (V-J) gene rearrangements in the MCL. This report represents the first case of a blastoid variant of MCL occurring in composite with cHL.

Divergent clonal evolution of a common precursor to mantle cell lymphoma and classic Hodgkin lymphoma

“…The clinical significance of HRS-like cells in MCL remains to be clarified. The reported cases were rarely managed with CHL appropriate regimens (Caleo et al 2003; Tinguely et al 2003; Hayes et al 2006; Schneider et al 2014; Giua et al 2015; Murray et al 2017; Kramer et al 2019), which is consistent with the current recommendations for management of CLL/SLL with “type I” HRS-like (Swerdlow et al 2017). Once a diagnosis of bona fide CHL is established, management with CHL appropriate regimen would be reasonable as demonstrated by control of CHL component in this case.…”

“…Clonally related HRS-like cells in MCL have only been reported in three prior cases (Supplemental Table S4; Schneider et al 2014; Murray et al 2017; Kramer et al 2019). In these studies, the clonal relationship was established by PCR studies of the IGHV regions after microdissection (Schneider et al 2014; Murray et al 2017) and confirmed by FISH studies showing IGH/ CCND1 translocation in both MCL and HRS-like cells (Schneider et al 2014; Murray et al 2017; Kramer et al 2019). Our case is exceptional as a bona fide CHL was diagnosed rather than MCL with HRS c-like cells, and a MCL component was not present in the biopsy with CHL either by flow cytometric study (with a sensitivity approaching 0.01%) or immunohistochemical studies (Supplemental Fig.…”

“…Intratumoral heterogeneity, subclonal architecture, and clonal dynamic changes after chemotherapy have been demonstrated (Bea et al 2013). The presence of Hodgkin/Reed–Sternberg (HRS)-like cells has been reported in rare patients suggesting clonal evolution HRS-like cells from MCL (Schneider et al 2014; Murray et al 2017; Kramer et al 2019). Interestingly, all these cases had scattered HRS-like cells in a background of typical MCL (Caleo et al 2003; Tinguely et al 2003; Hayes et al 2006; Schneider et al 2014; Giua et al 2015; Murray et al 2017; Kramer et al 2019).…”

“…The presence of Hodgkin/Reed–Sternberg (HRS)-like cells has been reported in rare patients suggesting clonal evolution HRS-like cells from MCL (Schneider et al 2014; Murray et al 2017; Kramer et al 2019). Interestingly, all these cases had scattered HRS-like cells in a background of typical MCL (Caleo et al 2003; Tinguely et al 2003; Hayes et al 2006; Schneider et al 2014; Giua et al 2015; Murray et al 2017; Kramer et al 2019). The mixed inflammatory microenvironment seen in classic Hodgkin lymphoma (CHL) was seldom observed, mimicking the so-called type I morphology with HRS-like cells in chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (de Leval et al 2004; Mao et al 2007; Xiao et al 2016).…”

Mantle cell lymphoma with EBV‐positive Hodgkin and Reed–Sternberg‐like cells in a patient after autologous PBSCT: Phenotypically distinct but genetically related tumors

“…However, the co‐existence of MCL and cHL is rare, and only eight cases have been described 5–11 . Of these, HRS cells were clonally related to the coexisting MCL in four cases 6,9–11 and unrelated in the remaining two 7 . A case of MCL, which relapsed with the presence of HRS‐like cells 9 years after autologous peripheral blood stem cell transplantation (auto‐PBSCT) is presented.…”