Presenter: Beltran-Gárate, B. Presenter's Affiliation: Hospital Rebagliati, Peru Type of Session: Scientific
It has been demonstrated that geographic variations can predict for the clinical behavior and pathologic characteristics of malignant lymphomas. Adult T -cell leukemia/lymphoma (ATLL) is an aggressive lymphoma associated with the human T-cell lymphotropic virus type-I (HTLV-I). This disease is commonly seen among the population of Southern Japan, the Caribbean, and parts of South America, and can have a very heterogeneous manifestation. The Shimoyama system describes four types of ATLL: acute, lymphomatous, chronic, and smoldering. More recently, the authors have described a primary cutaneous subtype in Perú (EHA 2001: abstract 129). The WHO-EORTC has a classification system for lymphomas that provides some assistance in developing a unified nomenclature. The authors initiated this study to establish the relative frequency of cutaneous lymphomas and to examine the clinical relevance of the new WHO/EORTC classification in a “General Hospital” in Lima-Perú
Materials and Methods
Retrospective study of 78 primary cutaneous lymphomas diagnosed from 1997 to 2004 in our General Hospital.
All patients were older than 18 years of age.
Clinical records, haematoxylin & eosin-stained slides and immunohistochemical stains from 78 patients were reviewed.
HTLV-1 serology was performed using ELISA and Western Blot method.
The statistical method was descriptive and survival was calculated using the Kaplan-Meier method.
Mean age at time of presentation was 62 years
Female:male ratio was 1.5:1
88.6% were T-cell lymphomas and 11.4% were B-cell lymphomas
86% (67/78) were primary cutaneous lymphomas and 14% (11/78) were secondary cutaneous lymphomas
Most frequent primary cutaneous lymphoma was mycosis fungoides (MF): 44.7% (30/67)
one case each of the following lymphomas: anaplastic large cell, Sézary syndrome, nasal type extranodal NK/T-cell lymphoma, marginal zone B-cell lymphoma, follicle center lymphoma, intravascular lymphoma, and unclassifiable lymphomas 5/67 (7.4%).
Most frequent secondary cutaneous lymphomas were acute and lymphomatous subtypes of ATLL (72%).
5-year overall survival for MF was 77%
5-year overall survival for primary cutaneous ATLL lymphomas was 18%
5-year overall survival was 0% for the secondary cutaneous ATLL group
In this retrospective analysis, MF is the most frequent type of primary cutaneous lymphoma seen at General Hospital, Peru, and has a relatively favorable overall survival at five years. Although ATLL is the second most common form seen, it is associated with a relatively poor prognosis.
This retrospective, single institution report offers a very interesting perspective of primary and secondary cutaneous lymphoma. By describing the characteristics of a Peruvian patient population, the authors provide an account of the expected distribution and survival outcomes of this relatively rare disease in an endemic population. This information can then potentially serve as a basis for future epidemiologic analyses of both primary and secondary cutaneous lymphomas.