title: High frequency of primary cutaneous lymphomas associated with lymphoproliferative disorders of different lineage.
abstract: In patients suffering from primary cutaneous lymphomas, secondary malignancies of various origin may develop. However, the frequency of a second neoplasm deriving from another lymphoid lineage is still unclear and may be underestimated. We screened all our patients with primary cutaneous lymphomas from a 4-year recruitment period for a coexisting secondary lymphoproliferative disorder. The cohort comprised of a total of 82 patients with primary cutaneous lymphomas, 62 with primary cutaneous T-cell lymphoma (CTCL), 18 with primary cutaneous B-cell lymphomas, and two with CD4+/CD56+ hematodermic neoplasm/blastic lymphomas. Seven patients (8.5%) were identified with a coexisting lymphoma of a different lymphoid lineage. Four patients with Sézary syndrome (SS) suffered from systemic B-cell lymphoma. Two of these developed SS after chemotherapy of their B-cell lymphoma. The other three patients with various types of skin lymphomas (SS, Mycosis fungoides [MF], primary cutaneous marginal zone lymphoma) developed Hodgkin's disease (hairy cell leukemia). Our data indicate that patients with primary cutaneous lymphomas have an elevated risk for the development of a secondary lymphoproliferative disorder even without previous chemotherapy. Possible explanations for this association include a genetic predisposition, alterations in early progenitor cells, underlying viral infections, and/or stimulation of a B-cell clone by the malignant helper T cells of the primary CTCL and vice versa.
authors.affiliation: Department of Dermatology, University of Goettingen, Goettingen, Germany. christian.hallermann@fachklinik-hornheide.de
authors.forename: Christian
authors.initials: C
authors.lastname: Hallermann
authors.forename: Kjell Matthias
authors.initials: KM
authors.lastname: Kaune
authors.forename: Matthias Kjell
authors.initials: MK
authors.lastname: Kaune
authors.forename: Markus
authors.initials: M
authors.lastname: Tiemann
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Kunze
authors.forename: Frank
authors.initials: F
authors.lastname: Griesinger
authors.forename: Christina
authors.initials: C
authors.lastname: Mitteldorf
authors.forename: Hans-Peter
authors.initials: HP
authors.lastname: Bertsch
authors.forename: Christine
authors.initials: C
authors.lastname: Neumann
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D005260:Female; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000150:complications* / Q000188:drug therapy; D008232:Lymphoproliferative Disorders / Q000150:complications*; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000473:pathology*; D012307:Risk Factors