Hemophagocytic Lymphohistiocytosis during the Treatment of T-lymphoblastic Lymphoma

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A 12-year-old Japanese boy presented with epigastric pain and swelling of the neck. Biopsy of the cervical lymph node and the bone marrow showed diffuse proliferation of lymphoblastic cells with precursor T-cell immunophenotypes. The patient was diagnosed with stage IV T-lymphoblastic lymphoma (T-LBL) and complete remission was successfully achieved after induction chemotherapy without any adverse events. At 10 months after diagnosis, he presented with an abrupt onset of transfusion-refractory thrombocytopenia during maintenance therapy. Bone marrow examinations revealed hypoplastic marrow with phagocytosis of the blood cells by multinucleated giant cells with foamy cytoplasm and a diagnosis of hemophagocytic lymphohistiocytosis (HLH) was confirmed. No evidence of T-LBL relapse was found by positron emission tomography. He progressed to severe bone marrow failure and underwent allogeneic stem cell transplantation (SCT) from his human leukocyte antigen (HLA)-matched brother. He achieved complete remission with partial hematological recovery. Unfortunately, the patient died from relapsed T-LBL at 6 months after SCT. The onset of HLH during childhood cancer therapy is extremely rare, but these conditions often develop chemoresistance resulting in a lethal outcome. Therefore, they may require more intensive myeloablative therapy with stem cell rescue.

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