IHC stains:
Diagnosis:
Systemic EBV+ TCL of childhood vs EBV+ PTCL.
Systemic EBV+ TCL of childhood (revised name in 2016 WHO revision, called “lymphoproliferative disorder” in 2008 WHO) has a fulminant clinical course usually associated with a hemophagocytic syndrome. The change of the name reflects its fulminant clinical course. The differential diagnosis includes acute EBV-associated hemophagocytic lymphohistiocytosis (HLH), which can present acutely, but in some patients responds well to the HLH 94 protocol, and is not considered neoplastic. EBV+ PTCL, defined as demonstrating EBV in the majority of the neoplastic cells, are uncommon and included under the broad heading of PTCL, NOS. They are generally monomorphic and lack the angioinvasion and necrosis of extranodal NK/T-cell lymphoma. They most often present in older adults, and also can be seen in the post-transplant setting and other immunodeficiencystates.
Systemic EBV+ TCL of childhood vs EBV+ PTCL.
Systemic EBV+ TCL of childhood (revised name in 2016 WHO revision, called “lymphoproliferative disorder” in 2008 WHO) has a fulminant clinical course usually associated with a hemophagocytic syndrome. The change of the name reflects its fulminant clinical course. The differential diagnosis includes acute EBV-associated hemophagocytic lymphohistiocytosis (HLH), which can present acutely, but in some patients responds well to the HLH 94 protocol, and is not considered neoplastic. EBV+ PTCL, defined as demonstrating EBV in the majority of the neoplastic cells, are uncommon and included under the broad heading of PTCL, NOS. They are generally monomorphic and lack the angioinvasion and necrosis of extranodal NK/T-cell lymphoma. They most often present in older adults, and also can be seen in the post-transplant setting and other immunodeficiencystates.
![Picture](/uploads/2/5/3/2/25327069/published/ebv-tcl-child.jpeg?1488236352)
Jaffe ES, et al. Hematopathology. 2011