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EBV-related primary splenic lymphocyte-depleted classical Hodgkin lymphoma
  1. Vishwas Parekh,
  2. Deniz Peker
  1. Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama, USA
  1. Correspondence to Dr Deniz Peker, Department of Pathology, University of Alabama at Birmingham, 1802 6th Ave South, NP 3552, Birmingham, AL 35233-7331, USA; dpeker{at}


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.


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