title: Cutaneous Involvement in a Case of Intravascular T-Cell Lymphoma With a γδ Phenotype.
abstract: Intravascular lymphomas (IVL) are uncommon variants of extranodal non-Hodgkin which are usually difficult to diagnose because of their lack of clinical uniformity. Most cases are of B-cell differentiation followed by natural killer/T-cell differentiation and underlying CD30 lymphoproliferative conditions. Epstein-Barr virus is pathogenically related in most of the natural killer/T-cell variants, and the skin is a common site of presentation noted in approximately 40% of cases. Recently, cases with uncommon phenotypes have been described, expanding our understanding of the pathogenesis of this condition. In this report, we describe a 67-year-old man with a 3-month history of constitutional symptoms associated with linear purpuric macules on the trunk, pancytopenia, and high levels of serum lactate dehydrogenase. He had been followed for longstanding adenopathy and hepatosplenomegaly. Skin biopsy demonstrated a intravascular lymphocytic proliferation with positivity for CD3, CD2, CD5, and γδ T-cell receptor marker; in situ hybridization Epstein-Barr virus RNA was negative. The patient was subsequently treated with chemotherapy and allogenic stem cell transplant. He remains in complete remission 6 months posttransplant. Although the presence of hepatosplenomegaly led to consideration of a hepatosplenic T-cell lymphoma, it was pre-existing for several years making the diagnosis doubtful. To our knowledge, this is the first case report of an IVL γδ T-cell lymphoma.
authors.affiliation: *Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, IL; and †Division of Hematology-Oncology, Department of Medicine, Robert H. Lurie Comprehensive Cancer Center, Northwestern University, Chicago, IL.
authors.forename: M Estela
authors.initials: ME
authors.lastname: Martinez-Escala
authors.forename: Lauren M
authors.initials: LM
authors.lastname: Guggina
authors.forename: Jonathan
authors.initials: J
authors.lastname: Cotliar
authors.forename: Jane N
authors.initials: JN
authors.lastname: Winter
authors.forename: Joan
authors.initials: J
authors.lastname: Guitart
mesh_terms: D000368:Aged; D014408:Biomarkers, Tumor / Q000235:genetics*; D001706:Biopsy; D017024:Chemotherapy, Adjuvant; D019676:Genes, T-Cell Receptor delta; D019675:Genes, T-Cell Receptor gamma; D020022:Genetic Predisposition to Disease; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D016399:Lymphoma, T-Cell / Q000235:genetics* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D016410:Lymphoma, T-Cell, Cutaneous / Q000235:genetics* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D008297:Male; D020360:Neoadjuvant Therapy; D010641:Phenotype; D012074:Remission Induction; D012878:Skin Neoplasms / Q000235:genetics* / Q000276:immunology / Q000556:secondary / Q000628:therapy; D033581:Stem Cell Transplantation; D016896:Treatment Outcome; D019043:Vascular Neoplasms / Q000235:genetics* / Q000276:immunology / Q000473:pathology / Q000628:therapy
chemical_list: D014408:Biomarkers, Tumor