High Prevalence of and Shorter Survival With MYD88 Mutation in Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg-Type
The activating mutation MYD88 L265P has been reported in approximately two-thirds of cases of primary cutaneous diffuse large B-cell lymphoma, leg-type. In a French retrospective analysis reported in JAMA Dermatology, Pham-Ledard et al confirmed the high prevalence of the mutation and documented poorer survival in disease with the mutation.
The study involved data from 58 patients diagnosed between 1988 and 2010 at dermatology departments in the French Study Group for Cutaneous Lymphomas who were available for molecular study. Median follow-up was 33 months, and 67% of patients were monitored until death.
Patient Characteristics
Patients had a median age of 79 years, 59% were female, and lesions were located on the leg in 76% of cases. The MYD88 L265P mutation was present in 34 patients (59%). Patients with the mutation and those with wild-type allele had similar clinical characteristics at presentation except for greater age (median, 84 vs 73 years, P = .006) and more frequent involvement of the leg (88% vs 58%, P = .008) among those with the mutation.
Survival Rates
Patients with vs without the MYD88 mutation had lower 3-year (65.7% vs 85.4%) and 5-year (60.2% vs 71.7%, P = .003) disease-specific survival rates and lower 3-year (55.8% vs 81.3%) and 5-year (38.4% vs 62.6%, P = .002) overall survival rates.
On multivariate analysis systematically adjusting for age, MYD88 mutation (odds ratio [OR] = 3.01, P = .04) and multiple skin lesions (OR = 5.12, P = .03) were significantly associated with poorer disease-specific survival, but location on the leg and age were not. MYD88 mutation (OR = 2.94, P = .02) and multiple skin lesions (OR = 2.94, P = .04) were independent predictors of poorer overall survival, but location on the leg and age were not.
The investigators concluded, “This study confirms the high prevalence of MYD88 L265P mutation in [primary cutaneous diffuse large B-cell lymphoma, leg-type] and shows its association with shorter survival. The clinical effect of MYD88 mutation activating the nuclear factor-κB pathway supports the use of targeted therapies at the time of relapse after conventional therapies.”
Jean-Philippe Merlio, MD, PhD, of University Bordeaux, is the corresponding author for the JAMA Dermatology article.
The study was supported by the REV-LEG Programme Hospitalier de la Recherche Clinique, Celgene, and the SIRIC BRIO program. The study authors reported no potential conflicts of interest.
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