High Frequency and Clinical Prognostic Value of MYD88 L265P Mutation in Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg-Type

Abstract

This study confirms the high prevalence of MYD88 L265P mutation in PCLBCL-LT 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.

Cutaneous B‐cell lymphomas: 2016 update on diagnosis, risk‐stratification, and management

“…Perhaps not Division of Hematology/Oncology University of Michigan Cancer Center, 1500 E. Medical Center Drive Room 4310 CC, Ann Arbor, Michigan 48109-5948 surprisingly, the genetic landscape observed in PCDLBCL, LT is similar to that observed in activated B-cell-type diffuse large B-cell lymphoma (ABC-DLBCL), with NF-jB-activating mutations being observed in CD79B, CARD11, and MYD88 [12][13][14]. Of these, somatic MYD88 L265P mutations appear most common with a prevalence of 75% [13,14].…”

“…Bcl-2 expression among PCFCL with a diffuse large B-cell histology may be a notable exception [23]. In contrast, PCDLBCL, LT is associated with a 5-year disease-specific survival of approximately 50% and with cytogenetic changes, including translocations involving c-myc, that confer a poor prognosis among systemic DLBCLs [1,13,24]. The presence of a somatic MYD88 L265P mutation is also associated with inferior disease-specific and overall survival [13].…”

“…In contrast, PCDLBCL, LT is associated with a 5-year disease-specific survival of approximately 50% and with cytogenetic changes, including translocations involving c-myc, that confer a poor prognosis among systemic DLBCLs [1,13,24]. The presence of a somatic MYD88 L265P mutation is also associated with inferior disease-specific and overall survival [13]. In contrast to patients presenting with only a single tumor, involvement of multiple sites, on one or both legs, is associated with a significantly inferior disease-specific survival [25].…”

Pathologic Diagnosis of Cutaneous Lymphomas

“…115,116 A high prevalence of MYD88L265P mutation shows an association with a shorter survival. 117 Differential diagnosis Secondary cutaneous involvement has to be excluded by staging procedures. The most important differential diagnosis of PCDLBCL-LT is PCFCL with a diffuse growth pattern.…”

New developments in the pathology of malignant lymphoma. A review of the literature published from August 2014 to October 2014

“…Pham-Ledard et al [34] performed a retrospective multicenter study using the medical records of patients from the French Study Group for Cutaneous Lymphomas. They had 61 patients with a diagnosis of PCDLCL-LT between 1988 and 2010 from which in 58 they could generate MYD88 mutation status.…”