title: Intralymphatic cutaneous anaplastic large cell lymphoma/lymphomatoid papulosis: expanding the spectrum of CD30-positive lymphoproliferative disorders.
abstract: Intravascular large B-cell lymphomas and EBV NK/T-cell lymphomas commonly follow an aggressive clinical course. We recently reported an entirely intravascular anaplastic large cell lymphoma (ALCL) in the skin with a surprisingly indolent clinical course; interestingly, this lymphoma involved the lymphatic rather than the blood vasculature. We hypothesized that intravascular skin-limited ALCL is distinct from aggressive systemic intravascular lymphomas in its intralymphatic localization and clinical course. We now describe 18 cases of cutaneous intravascular large cell lymphoproliferations from 4 institutions. All 12 intravascular large T-cell lesions were intralymphatic; the majority (9) were CD30 T-cell lymphoproliferative disorders (TLPDs), 5 further classified as intravascular ALK ALCL. One ALK ALCL and 2 benign microscopic intravascular T-cell proliferations were also intralymphatic. A single case of otherwise typical cutaneous follicle center lymphoma contained intralymphatic centroblasts. The clinical and pathologic characteristics of the CD30 TLPDs were similar to those of their extravascular counterparts, including extralymphatic dermal involvement in a subset, DUSP22-IRF4 translocations in half of tested ALK ALCLs, and associated mycosis fungoides in 1; most were skin-limited at baseline and remained so at relapse. All 5 cases of intravascular large B-cell lymphoma involved the blood vasculature and behaved in a clinically aggressive manner; the ALK ALCL, although intralymphatic, was systemic and clinically aggressive. We propose that cutaneous ALK ALCL and related CD30 ALK TLPDs involving the lymphatics are part of an expanding spectrum of CD30 TLPDs. The identification of intralymphatic as distinct from blood vascular localization may provide critical prognostic and therapeutic information.
authors.affiliation: Departments of *Pathology #Dermatology, Stanford University School of Medicine, Stanford †UCLA Medical Center, Los Angeles ‡San Diego Pathologists Medical Group, San Diego, CA §Department of Dermatology, Mayo Clinic, Jacksonville, FL ∥Department of Pathology, Massachusetts General Hospital, Boston, MA ¶Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
authors.forename: Mark A
authors.initials: MA
authors.lastname: Samols
authors.forename: Albert
authors.initials: A
authors.lastname: Su
authors.forename: Seong
authors.initials: S
authors.lastname: Ra
authors.forename: Mark A
authors.initials: MA
authors.lastname: Cappel
authors.forename: Abner
authors.initials: A
authors.lastname: Louissant
authors.forename: Ryan A
authors.initials: RA
authors.lastname: Knudson
authors.forename: Rhett P
authors.initials: RP
authors.lastname: Ketterling
authors.forename: Jonathan
authors.initials: J
authors.lastname: Said
authors.forename: Scott
authors.initials: S
authors.lastname: Binder
authors.forename: Nancy Lee
authors.initials: NL
authors.lastname: Harris
authors.forename: Andrew L
authors.initials: AL
authors.lastname: Feldman
authors.forename: Jinah
authors.initials: J
authors.lastname: Kim
authors.forename: Youn H
authors.initials: YH
authors.lastname: Kim
authors.forename: Dita
authors.initials: D
authors.lastname: Gratzinger
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000077548:Anaplastic Lymphoma Kinase; D014408:Biomarkers, Tumor / Q000032:analysis*; D001706:Biopsy; D001808:Blood Vessels / Q000276:immunology / Q000473:pathology; D049109:Cell Proliferation; D054637:Dual-Specificity Phosphatases / Q000235:genetics; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007150:Immunohistochemistry; D050835:Interferon Regulatory Factors / Q000235:genetics; D017730:Ki-1 Antigen / Q000032:analysis*; D042601:Lymphatic Vessels / Q000276:immunology* / Q000473:pathology; D054446:Lymphoma, Primary Cutaneous Anaplastic Large Cell / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D017731:Lymphomatoid Papulosis / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D054639:Mitogen-Activated Protein Kinase Phosphatases / Q000235:genetics; D010641:Phenotype; D011379:Prognosis; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D012878:Skin Neoplasms / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology* / Q000473:pathology; D014178:Translocation, Genetic; D014481:United States; D000099218:Interferon Regulatory Factor-4
chemical_list: D000077548:Anaplastic Lymphoma Kinase; D014408:Biomarkers, Tumor; D054637:Dual-Specificity Phosphatases; D050835:Interferon Regulatory Factors; D017730:Ki-1 Antigen; D054639:Mitogen-Activated Protein Kinase Phosphatases; D020794:Receptor Protein-Tyrosine Kinases; D000099218:Interferon Regulatory Factor-4; C000626173:ALK protein, human; C442824:DUSP22 protein, human