IHC stains:
Systemic EBV+ T cell lymphoma of Childhood (WHO 2016)
Systemic EBV+ T-cell lymphoma of Childhood ( WHO 2008: Systemic EBV+ T-cell lymphoproliferative disease)
Definition A fulminant illness characterized by a clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype. Mostly in Asians or Native Americans. Children or young adults. It can occur shortly after primary acute EBV infection or develop in the clinical setting of CAEBV-T/NK. It is usually characterized by a rapid clinical progression, with multiple organ failure, sepsis, and death. A hemophagocytic syndrome is nearly always present. Differential Diagnosis Aggressive NK-cell leukemia is very similar to systemic EBV+ T-cell LPD arising in young children with similar clinical presentation, presence of EBV in proliferating cells, and systemic hemophagocytosis. Phenotype is different. Expression NK cell markers, and do not show clonal T-cell receptor gene rearrangement. Distinction from CAEBV may be difficult in some cases based on morphologic grounds alone. In situ hybridization for EBER followed by clonality analysis is essential to avoid misdiagnosis. Systemic EBV+ T-cell lymphoma of the elderly is a rare, recently described disease that shares similar clinical and pathologic findings with the disease in children and young adults, but there are some differences. Generalized LAD is more common, no history of CAEBV-T/NK, and hemophagocytosis or involvement of bone marrow at initial presentation is rare. History of hepatitis B or C virus infection. |