Plasmablastic Lymphoma: A Challenging Diagnosis

Introduction: Plasmablastic lymphoma (PBL) is an aggressive lymphoma characterized by early relapses and subsequent chemotherapy resistance representing therefore diagnostic and therapeutic challenge with a very poor outcome.

Case Presentation: A 53-year-old- female patient presented with dyspnea and general status alteration due to anemia; diagnosed as hemolytic anemia and treated by corticotherapy without amelioration. The most recent hospitalization revealed worsening of her symptoms with weight loss of 5 kilos in one week, anorexia and lumbar pain. Physical examination showed isolated splenomegaly with no signs of bleeding, adenopathy nor a palpable breast mass. Extended laboratory tests were normal, except normocytic anemia with hemoglobin =8.76 g/dl; hct=26.6%; MCV=89.2 fl and a low haptoglobin concentration level <0.04 g/l with a high value of LDH 1165 UI/litre. Hypercalcemia of 12 mg/dl was also noted with low PTH. To complete the workup: imaging showed a total body CT scanner with absence of adenopathy, a magnetic resonance imaging (MRI) with no metastatic bone lesions confirming the diagnosis and a full body PET CT scanner revealing diffuse hyperactivity of the bone marrow with hypermetabolic splenomegaly, hepatomegaly and bone lytic lesion of some cervical vertebras. Bone marrow aspirate, biopsy and flow cytometry were done and in favor of lymphoma. An immunohistochemical profile shows positivity and correlates with morphology. It was compatible with Plasmablastic lymphoma (PBL). Patient received three cycles of chemotherapy (lenalidomide and CHOP) and immunomodulatory agents. But she passed away three months later.

Conclusion: Despite the recent advances in therapy of aggressive lymphomas, patients with PBL have the poorest outcome. Moreover, due to its challenging diagnosis and related complications, management of PBL remains to discuss on a case by case basis.