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

Abstract

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.