Primary Cutaneous Posttransplant Lymphoproliferative Disorders in Solid Organ Tranpsplant Recipients: A Multicenter European Case Series
Abstrakt
Primary cutaneous posttransplant lymphoproliferative disorders (PTLD) are rare. This retrospective, multicenter study of 35 cases aimed to better describe this entity.
Cases were (re)-classified according to the WHO-EORTC or the WHO 2008 classifications of lymphomas. Median interval between first transplantation and diagnosis was 85 months.
Fifty-seven percent of patients had a kidney transplant. Twenty-four cases (68,6%) were classified as primary cutaneous T cell lymphoma (CTCL) and 11 (31,4%) as primary cutaneous B cell PTLD.
Mycosis fungoides (MF) was the most common (50%) CTCL subtype. Ten (90,9%) cutaneous B cell PTLD cases were classified as EBV-associated B cell lymphoproliferations (including one plasmablastic lymphoma and one lymphomatoid granulomatosis) and one as diffuse large B cell lymphoma, other, that was EBV-negative.
Sixteen (45,7%) patients died after a median follow-up of 19.5 months (11 [68,8%] with CTCL [6 of whom had CD30+ lymphoproliferative disorders (LPD)] and 5 [31,2%] with cutaneous B cell PTLD. Median survival times for all patients, CTCL and cutaneous B cell PTLD subgroups were 93, 93 and 112 months, respectively.
Survival rates for MF were higher than those for CD30+ LPD. The spectrum of primary CTCL in organ transplant recipients (OTR) is similar to that in the general population.
The prognosis of posttransplant primary cutaneous CD30+ LPD is worse than postrtransplant MF and than its counterpart in the immunocompetent population. EBV-associated cutaneous B cell LPD predominates in OTR.