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Arch Pediatr Adolesc Med. 2006;160:1178-1179.

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Denouement and Discussion: Anaplastic Large Cell Lymphoma With Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) may occur in a primary form, often associated with mutations in the perforin gene responsible for natural killer cell cytotoxic activity,¹ or secondary to underlying rheumatic disease (most commonly systemic-onset juvenile rheumatoid arthritis), infection (usually herpesvirus infections such as Epstein-Barr virus or virtually any bacteria, fungus, or parasite), immunodeficiency (Griscelli syndrome), lysinuric protein intolerance, and malignancies. Lymphomas are the most common malignancies associated with HLH, and although HLH is frequently associated with subcutaneous T-cell lymphomas,² it is exceedingly rare as part of anaplastic large cell lymphoma (ALCL), as was observed in this case.³

Lymphomas, including Hodgkin and non-Hodgkin (NHL), account for 10% to 15% of childhood cancer in developed countries, and ALCL accounts for 10% of NHL in childhood. Anaplastic large cell lymphoma is a mature T-cell or null-cell lymphoma that has been recognized as a distinct entity in the classification of NHL.⁴ The 2 subtypes of . . . [Full Text of this Article]

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