Subcutaneous Panniculitis-like T-Cell Lymphoma (SPTCL) With Hemophagocytic Lymphohistiocytosis (HLH) Syndrome in Children and Its Essential Role of HAVCR2 Gene Mutation Analysis.

BACKGROUND We identified 3 adolescents with alpha-beta subtype subcutaneous panniculitis-like T-cell lymphoma. CASE PRESENTATION Three patients presented with prolonged fever, abnormal skin lesions, and cytopenia described in the context. All had the same disease entity, which showed the prolonged duration of B systemic symptoms till diagnosis, difficulty to distinguish from autoimmune diseases, presence of hemophagocytic lymphohistiocytosis syndrome, good response, and remained on long-term remission with nonchemotherapy treatment, which included oral corticosteroid and cyclosporin. CONCLUSIONS Although diagnosis can only be "highly suspected" with pathologic review, some cases may need multiple serial skin biopsy to clarify diagnosis because of the discrete distribution of specific histology. T-cell receptor gene rearrangement, which demonstrates a monoclonal pattern of alpha and beta chain gene, is the essential requirement for specific diagnosis. The role of molecular analysis by identification of germline hepatitis A virus cellular receptor 2 (HAVCR2) gene mutation can be much valuable in classifying susceptible patients.