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

Abstract

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a subtype of nonclassical Hodgkin lymphoma (HL). It resembles non-Hodgkin lymphoma (NHL), by expressing classic B cell markers such as CD20 and CD79a however lacks definitive HL markers (such as CD15 and CD30). T cell histiocyte-rich large B cell lymphoma (THRLBCL), on the other hand, is a distinct entity classified under NHL and considered a variant of diffuse large B cell lymphoma (DLBCL). NLPHL can look morphologically and immunologically similar to THRLBCL and often poses a diagnostic challenge. Neoplastic cells in both NLPHL and THRLBCL express B cell markers and are typically scattered in a background of reactive cells. The two major differences are the background cell type and the morphologic pattern. Despite having a phenotypic resemblance, they have distinct biologic behavior and clinical course. NLPHL typically has an indolent course, and THRLBCL has an aggressive course. Hence, differentiating these two entities is critical not only for prognosis but for treatment purposes. Of note, NLPHL has a small risk of transformation to an aggressive lymphoma such as THRLBCL.

Catch the Calcium: T-Cell Histiocyte-Rich B-Cell Lymphoma Presenting as Hypercalcemia

“…THRLBCL is distinguished by < 10% malignant B cells with a rich background of reactive T lymphocytes and histiocytes [4]. While it may appear morphologically similar to nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), THRLBCL has a much more aggressive course and worse overall prognosis [14][15][16]. It typically affects middle-aged men (median age of 40 years) who often present with advancedstage disease, frequently involving the spleen, liver, and bone marrow [4,[17][18][19].…”

“…THRLBCL is a rare, aggressive subtype of DLBCL that shares similar treatment options as DLBCL. R-CHOP is one of the mainstay systemic antineoplastic therapies for DLBCL and has been shown to have comparable outcomes in THRLBCL [4,14,15,[20][21][22][23]. Despite its efficacy, there are documented concerns with its toxicity and adverse effect profile limiting the ability of older patients (over 60 years of age) to complete therapy.…”

T cell/histiocyte rich large B cell lymphoma of ascending colon: a rare entity

“…Siricilla et al have reported a case of chemotherapy refractory THRLBCL like transformation of NLPHL which was then successfully treated with lenalidomide. 13 Surgical debulking followed by adjuvant chemotherapy (R-CHOP) is the mainstay for localized and non-metastatic gastrointestinal disease. El Weshi et al, has reported a 5-year overall survival rate of 46% with aggressive course and poor outcome.…”