Primary Splenic Classic Hodgkin Lymphoma with Immune Thrombocytopenic Purpura and Extramedullary Hematopoiesis

BackgroundPrimary lymphomas of the spleen are rare, with primary splenic classic Hodgkin lymphoma (CHL) accounting for a minuscule fraction of these tumors, rendering it an exceptionally uncommon entity. Immune thrombocytopenic purpura (ITP) has been rarely reported in association with nodal CHL, and to our knowledge, there are no prior reports linking it with splenic CHL. Presented is an example of primary splenic CHL associated with ITP and extramedullary hematopoiesis (EMH), highlighting this unusual presentation of CHL.Patient presentationA 70-year-old man presented with severe epistaxis and wet purpura in the mouth and low platelets. He had noted night sweats and fever for three months prior to presentation and had chronic mild thrombocytopenia. Imaging studies showed splenomegaly with scattered hypodensities in the spleen but no lymphadenopathy. The initial clinical suspicion based on imaging results was an indolent lymphoma. He was treated with IV immunoglobulin, rituximab, steroids and eltrombopag with no response. He underwent a splenectomy with diagnostic and therapeutic intent. Histopathological evaluation was consistent with a primary splenic CHL. The background spleen showed EMH. His thrombocytopenia improved post-splenectomy.ConclusionWe report an uncommon example of primary splenic CHL with concurrent ITP and splenic EMH, highlighting the associated diagnostic challenges and reviewing the relevant literature.Additionally, we hope to emphasize the pivotal role of splenectomy in both diagnosis and management of this patient, as well as explore possible connections between splenic CHL, ITP and EMH.