ABSTRACT
Background and purpose:T-cell non-Hodgkin’s lymphoma(T-NHL) has relatively high incidence in Asian countries, and the incidence rate showed an upward tendency. It has a dual characteristic of both ethnic and regional. We conducted this study in order to analyze the clinical characteristics, pathological type and survival of T-NHL in our hospital. Methods:Records of 50 cases with T-NHL treated from Jan. 2002 to Dec. 2012, were analyzed in terms of clinical characteristics, distribution of pathological type and survival. Results:Patients with T-NHL account for 24%of NHL cases in our study. Of the 50 cases, 37 were Han, 13 were Uygur;The most frequent type was NK/T cell lymphoma(NK/TCL)(16/50, 32%). We performed pathological type for each age group:4 cases with children’s group were all T-lymphoblastic leukemia/lymphoma(T-LBL)(4/4,100%), the most frequent type of youth and middle age group was NK/TCL(8/20, 40%;7/13, 53.8%), as the senile group was angioimmunoblastic T-cell lymphoma(AITL)(6/13, 46.2%). There was also a signiifcant difference in pathological type between the stage, with stageⅠ-Ⅱwere all NK/TCL(12/12, 100%), stageⅢ-Ⅳwere observed with peripheral T-cell lymphomas-unspeciifed(PTCL-U)(9/38, 23.7%) and AITL(9/38, 23.7%). The frequent type of Han was NK/TCL(14/37, 37.8%), as the Uygur was T-LBL(5/13, 38.5%). The median overall survival(OS) time was 12 (ranged 0-112) months. The 5 year OS rate was 39%. Both pathological type and age group were important factors influencing survival. The difference in outcome for the pathological type(P<0.05): NK/TCL and anaplastic large cell lymphoma(ALCL) had the better 3 year OS rates(71%, 61%), but PTCL-U and T-LBL had poor prognosis(19%, 7%). Age did a signiifcant effect on OS(P<0.05):children’s group had the worse 5-year OS rate (0%), middle age group had a better prognosis (67%), the OS of the youth group was 35%, for the senile group was 21%. Conclusion:T-NHL in Xinjiang region have their unique clinical characteristics:The overall incidence rate was similar with the domestic report, however, higher than the reports abroad. The incidence of Han was higher than Uygur. Pathological type showed in this study was different from that in European and American countries. There was a significant difference in distribution of pathological type in different age group, stage and nation. The long-term survival and prognosis of patients in Xinjiang region was poor. Both pathological type and age group were important factors inlfuencing survival.
ABSTRACT
La micosis fungoides y el síndrome de Sezary están constituidos por un grupo de linfomas no Hodgkin indolentes, extranodales, con origen en linfocitos T; afectan la piel de manera primaria; son generalmente incurables y los pacientes tienen mal pronóstico porque la enfermedad es habitualmente refractaria a diversas modalidades terapéuticas. El trasplante de médula ósea alogénico es una opción terapéutica más. La alta mortalidad del acondicionamiento pretrasplante convencional ha hecho que se considere el trasplante alogénico con acondicionamiento de intensidad reducida como una opción viable para esta enfermedad. Se presenta el caso de una paciente con micosis fungoides a quien se hizo un trasplante hematopoyético con un esquema no mieloablativo y en quien se logró remisión completa sostenida de la enfermedad.
Sezary syndrome (SS) and mycosis fungoides (MF) are a group of non Hodgkin lymphomas that originate from T-lymphocytes and involve mostly the skin. These entities are generally non treatable and patient prognosis remains poor even with the advent of current treatment schedules. Complete remissions are seldom observed. For this reason, bone marrow transplant has been used as a treatment option. The high mortality associated with this procedure has turned reduced intensity conditioning stem cell transplant into a treatment option. This case study illustrates how stem cell transplant offers complete remission of this type of lymphomas.