Prognosis of Mycosis Fungoides in Children and Adolescents:
Now open: Certificate Course in Management of Covid-19 by Govt. Of Gujarat and PlexusMDKnow more...Now open: Certificate Course in Management of Covid-19 by Govt. Of Gujarat and PlexusMDKnow more...
Comprehensive data on childhood mycosis fungoides (MF) is scarce. A Study was conducted to describe the clinical features, immunophenotypes, various treatment options, and prognosis of MF in children and adolescents.

Case series and case reports were included if data on childhood MF were extractable. Patients were treated from January 1, 1990, to July 31, 2019, and were younger than 20 years at the time of diagnosis. The methodologic quality of the included studies was assessed with items from the Newcastle-Ottawa scale. A total of 571 unique patients were included. The mean (SD) age at diagnosis was 12.2 years; at onset, 8.6 years. The female-to-male ratio was 1:1.6 (350 male patients [61.3%]).

Results:
--Among 522 patients with data available at diagnosis, stage 1 disease constituted 478 cases, followed by stage 2 and stage 4.

--Among the 567 patients with data available, the most common variant of MF was the hypopigmented form (309), followed by classic MF (187).

--The MF lesions were predominantly the CD4+ and CD8+ immunophenotype in 99 and 79 of 200 patients, respectively.

--Among the treatments, narrowband UV-B was the most frequently used (150 of 426). Most patients were alive with the disease (185 of 279); 83 of 279 were in complete remission; and 11 of 279 had died by the last follow-up.

--A longer time from onset to diagnosis (hazard ratio [HR], 1.24), granulomatous slack skin (HR, 12.25), granulomatous MF (HR, 14.59), a history of organ transplant (HR, 10.15), and stage 2 disease at the time of diagnosis (HR, 10.22) were associated with worse outcomes.

In conclusion, the results of this analysis indicate that there is often a substantial delay until a proper diagnosis of childhood MF is made, which may be harmful to the prognosis.

Source: https://jamanetwork.com/journals/jamadermatology/article-abstract/2777012
Like
Comment
Share