title: Primary cutaneous follicular helper T-cell lymphoma treated with allogeneic bone marrow transplantation: immunohistochemical comparison with angioimmunoblastic T-cell lymphoma.
abstract: Primary cutaneous follicular helper T (TFH)-cell lymphoma has recently been proposed, and is characterized by proliferation of malignant T cells expressing TFH-cell markers, such as CXCL13, accompanied by numerous reactive B cells. We report here a patient whose skin histology showed massive infiltration of both T and B cells, with a proliferation of arborizing high endothelial venules and follicular dendritic cells. Infiltrating T cells were positive for CXCL13, programmed death (PD)-1, inducible T-cell co-stimulator, and BCL-6. Southern blot analyses using DNA from the skin revealed monoclonality of both T and B cells. The patient had marked resistance to treatments, and complete remission was achieved only after allogeneic stem cell transplantation. The present case showed overlapping features with angio-immunoblastic T-cell lymphoma (AITL), although systemic symptoms were not observed. Further study is needed to define the criteria of this provisional entity, representing the cutaneous counterpart of the nodal follicular peripheral T-cell lymphoma or AITL.
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tokyo, 7-3-1, Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
authors.forename: Hanako
authors.initials: H
authors.lastname: Ohmatsu
authors.forename: Makoto
authors.initials: M
authors.lastname: Sugaya
authors.forename: Hideki
authors.initials: H
authors.lastname: Fujita
authors.forename: Takafumi
authors.initials: T
authors.lastname: Kadono
authors.forename: Shinichi
authors.initials: S
authors.lastname: Sato
mesh_terms: D000328:Adult; D000954:Antigens, Surface / Q000032:analysis; D016026:Bone Marrow Transplantation; D054382:Chemokine CXCL13 / Q000032:analysis; D020566:Dendritic Cells, Follicular / Q000737:chemistry; D042783:Endothelial Cells / Q000737:chemistry; D005260:Female; D006801:Humans; D060889:Inducible T-Cell Co-Stimulator Protein / Q000032:analysis; D016410:Lymphoma, T-Cell, Cutaneous / Q000737:chemistry* / Q000473:pathology* / Q000628:therapy; D008565:Membrane Proteins / Q000032:analysis; D061026:Programmed Cell Death 1 Receptor / Q000032:analysis; D017464:Receptors, Complement 3d / Q000032:analysis; D012878:Skin Neoplasms / Q000737:chemistry* / Q000473:pathology* / Q000628:therapy; D006377:T-Lymphocytes, Helper-Inducer; D014184:Transplantation, Homologous; D014699:Venules / Q000737:chemistry
chemical_list: D000954:Antigens, Surface; C110663:CXCL13 protein, human; D054382:Chemokine CXCL13; C556692:ICOS protein, human; D060889:Inducible T-Cell Co-Stimulator Protein; C069886:L-selectin counter-receptors; D008565:Membrane Proteins; C105992:PDCD1 protein, human; D061026:Programmed Cell Death 1 Receptor; D017464:Receptors, Complement 3d