Small intestinal presentation of nodular lymphocyte-predominant Hodgkin lymphoma with T cell/histiocyte-rich B cell lymphoma-like areas—with review of literature on extranodal presentation of this disease

Abstract

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), accounts for ∼5% of all cases of Hodgkin lymphoma and is characterized by involvement of the peripheral lymph nodes. NLPHL occurs in young adults and is associated with frequent relapses. In 3% to 7% of cases, NLPHL progresses to a diffuse large B cell lymphoma. Furthermore, a proportion of NLPHL also have areas with features of T cell/histiocyte-rich large B cell lymphoma (THRLBCL), either at presentation or on follow-up. Here, we describe a 32-year-old man who presented to the emergency department with small bowel perforation. The resected small bowel showed fullthickness mural ulceration and involvement by a lymphoma with features of NLPHL that also had areas resembling THRLBCL. The patient had axillary lymphadenopathy, biopsy of which showed NLPHL with focal THRLBCLlike areas. Such a lymphoma presenting as small intestinal lesion/perforation has not been reported in the literature before. We take this opportunity to review the literature on extranodal presentations of NLPHL and discuss the natural history of this disease.

A Case of Chemotherapy-Refractory “THRLBCL like Transformation of NLPHL” Successfully Treated with Lenalidomide

“…NLPHL typically presents with localized disease, involving the peripheral lymph nodes. Spleen and extranodal disease at presentation is rare [ 14 ], and the clinical course is indolent [ 15 ]. THRLBCL is aggressive and typically presents in advanced stages, with nearly 50% of cases having bone marrow, spleen, and liver involvement [ 16 ].…”

“…Traditional therapy for stage I/II NLPHL is full nodal excision followed by either a “watch or wait strategy” or localized radiation [ 21 – 25 ]. The challenging part of NLPHL is the relapsing nature of the disease and the potential risk (3–7%) [ 14 ] of transformation into a large B cell lymphoma, sometimes even decades later [ 26 ]. THRLBCL is one of the most common types of large B cell lymphoma, seen as a result of transformation of NLPHL [ 27 ].…”

Nodular Lymphocyte Predominant Hodgkin Lymphoma of the Ileum

“…Bohn-Sarmiento et al have reported a case of primary NLPHL involving the appendix in a 62-year-old male who presented with acute appendicitis [ 6 ]. More recently, Bagwan et al have described a 32-year-old male who was being evaluated for axillary lymphadenopathy when he presented with an acute abdomen and was found to have small bowel perforation associated with NLPHL in addition to a T-cell/histiocyte-rich large B-cell lymphoma-like component [ 7 ]. Both components also were present in an axillary lymph node biopsy specimen.…”

First case of T-cell/histiocyte-rich-large B-cell lymphoma presenting with duodenal obstruction

“…TCHRBCL represents 1–3% of all DLBCL in recent series (5, 7, 8). TCHRBCL cases mostly present in advanced stages ranging from 53 to 91%.…”