Primary isolated bone marrow diffuse large B-cell lymphoma with the initial presentation as severe thrombocytopenia, successfully treated with chemotherapy: a case report and review of the literature

Secondary bone marrow involvement of non-Hodgkin's lymphoma (NHL) is relatively common. However, primary isolated bone marrow involvement in NHL, successfully treated without any relapse, is quite rare, except in several leukemia/ lymphoma cases which are considered to primarily involve the bone marrow. We here report a case of primary bone marrow diffuse large B cell lymphoma (PBML/DLBCL), in which the patient presented a prolonged high-grade fever and systemic purpura due to severe thrombocytopenia, which was successfully treated with systemic chemotherapy and remains in complete remission (CR) at 2.5 years after the initial diagnosis. Review of 53 cases of PBML/DLBCL presented in the literature suggests that the prognosis for this eternity is unfavorable, but rituximab-based therapeutic strategies, including autologous stem cell transplantation are promising for improving their outcomes.