In this report, we present an extremely rare case of Hodgkin lymphoma with severe thrombocytopenia, where extramedullary hematopoiesis (EMH) and hemophagocytosis were simultaneously observed within the lymph nodes. Approximately 40 days before the patient's death, computed tomography performed at another hospital revealed enlargement of deep lymph nodes, including hilar, mediastinal, and mesenteric lymph nodes. Thirteen days before death, the patient was admitted to the hospital with marked hematuria, systemic purpura, mild renal dysfunction, and severe thrombocytopenia
two days later, he was transferred to our institution. On admission, almost all lymph nodes were enlarged, which led us to suspect malignant lymphoma. Blood examination findings suggested that immune thrombocytopenia was a possible cause of severe thrombocytopenia. However, due to severe thrombocytopenia, we could not perform a pathological diagnosis via excisional biopsy of the lymph nodes. Despite treatment with dexamethasone (DEX) pulse therapy, immunoglobulin therapy, and platelet transfusion, the platelet count did not increase. Ultimately, the patient died due to multi-organ hemorrhage. Postmortem pathological examination revealed classic Hodgkin lymphoma in the enlarged lymph nodes, along with EMH and hemophagocytosis within the same lymph nodes. We conducted a literature review and found no prior reports of cases where malignant lymphoma coexisted with EMH and hemophagocytosis within the same lymph node, making this the first reported case.