title: EBV-related primary splenic lymphocyte-depleted classical Hodgkin lymphoma.
abstract: Lymphocyte-depleted classical Hodgkin lymphoma (LDCHL) is the rarest entity among the classical Hodgkin lymphoma (CHL) subtypes, accounting for <1% of CHL cases in Western countries. Similarly, primary splenic Hodgkin lymphoma is a rare diagnosis among all cases of lymphomatous involvement of spleen. Representing a combination of these two rare entities is the primary splenic LDCHL, with only one case reported in the literature. Here, we report second case of primary splenic LDCHL with a unique presentation. The patient was a 60-year-old Caucasian HIV-negative woman with a history of extranodal pleuropulmonary marginal zone lymphoma (MZL) treated with rituximab who presented with new-onset splenomegaly. Splenectomy revealed large number of Hodgkin/Reed-Sternberg cells (CD15+, CD30+, PAX5+ and EBER+) with the background consisting of histiocytes and smaller number of lymphocytes and eosinophils. There was no evidence of nodal or other extranodal site involvement. Thus, in addition to being a very rare entity, this case also provides an additional point of interest: the disease arose in the backdrop of previous diagnosis of extranodal pleuropulmonary MZL successfully treated with rituximab. We discuss the potential implications.
authors.affiliation: Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama, USA.
authors.forename: Vishwas
authors.initials: V
authors.lastname: Parekh
authors.affiliation: Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama, USA.
authors.forename: Deniz
authors.initials: D
authors.lastname: Peker
mesh_terms: D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D006689:Hodgkin Disease / Q000209:etiology / Q000473:pathology*; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000209:etiology / Q000473:pathology; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000473:pathology*; D013154:Spleen / Q000473:pathology*; D013160:Splenic Neoplasms / Q000473:pathology*
keywords: CANCER; HAEMATO-ONCOLOGY; HEMATOPATHOLOGY; HISTOPATHOLOGY; ONCOLOGY