Primary cutaneous T-cell lymphoma not otherwise specified (NOS) with expression of cytotoxic molecules: Case report in a pediatric patient

Primary cutaneous T-cell lymphomas NOS (CTCL not otherwise specified) involve a heterogeneous group of neoplasms that affect the skin, with no evidence of extracutaneous involvement at the time of diagnosis. The association with hemophagocytic lymphohistiocytosis (HLH) syndrome has been documented....

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Bibliographic Details
Published in:Pediatric hematology oncology journal 2021-09, Vol.6 (3), p.135-138
Main Authors: Rossetti, E., Guitter, M., Peruzzo, L., Cervini, A.B., Centeno, M., Sánchez la Rosa, C., Galluzo, L., Pennella, C., Felice, M.S.
Format: Article
Language:English
Subjects:
Children
Double negative (CD4/CD8) primary cutaneous T-cell lymphoma
Hemophagocytic lymphohistiocytosis
Primary cutaneous T-cell lymphomas NOS
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Summary:Primary cutaneous T-cell lymphomas NOS (CTCL not otherwise specified) involve a heterogeneous group of neoplasms that affect the skin, with no evidence of extracutaneous involvement at the time of diagnosis. The association with hemophagocytic lymphohistiocytosis (HLH) syndrome has been documented. They are rare in children, and usually are characterized by a dismal prognosis. We report an unusual case of a pediatric patient diagnosed as acute pityriasis lichenoide et varioliformis (PLEVA), with a torpid clinical course, association with HLH and a subsequent diagnosis of double negative (CD4/CD8) primary CTCL, with expression of cytotoxic molecules.
ISSN:2468-1245
2468-1245
DOI:10.1016/j.phoj.2021.08.003