Isolated Cutaneous Anaplastic Large Cell Lymphoma Progressing to Severe Systemic Disease With Myocardial Involvement and Central Nervous System Infiltration

Abstract

Anaplastic large cell lymphoma (ALCL) is a rare tumor comprising around 10–15% of childhood lymphomas. We describe the case of a female who initially presented with localized skin disease associated with an insect bite. However, she subsequently relapsed with widespread systemic ALK-positive ALCL that included lymphoma deposits in the myocardium, a very rare manifestation. Her disease responded well to chemotherapy but she later developed a fatal relapse in the CNS. We also present data on an immune response to ALK, demonstrating a fluctuation in the levels of circulating antibodies to ALK corresponding to the different phases of her illness. Pediatr Blood Cancer 2008;50:879–881.