מאפיינים קליניים, טיפולים ותוצאים בחולי לימפומה עורית ראשונית מסוג גאמא-דלתא:
ניתוח רב-מרכזי של ממאירות נדירה

Background
PCGTCL is a rare disorder, accounting for <1% of all lymphomas and typically characterized by highly aggressive course.

Objective
We conducted a multi-institutional, retrospective analysis to delineateclinical and pathological characteristics, treatment patterns and outcomes in a series of individuals with PCGDTCL.

Methods
We collected detailed information on patients with PCGDTCL diagnosed between 2000-2017 across 10 centers. Pathologic data on de-identified pathology reports were reviewed centrally by an expert dermatopathologist (MP). Univariate associations were derived for associations with survival.

Results
Collectively, all centers submitted a total of 80 cases that were diagnosed and treated locally as PCGDTCL. 48 (60%) cases met pre-defined criteria for inclusion of bona fide diagnosis of PCGTCL. 26 (33%) cases had insufficient scores and were grouped in a 2nd tier. The 2-year progression free survival (PFS) for the bona fide patients was 39% and 2-year overall survival (OS) was 36%. The cases in the 2nd tier had overall similar 2-year PFS of 41% and OS of 37%. Use of consolidative stem cell transplantation in 1st remission was associated with improved survival (P=0.02). Performance status (P=0.006) and LDH (P=0.05) were significantly associated with OS.

Conclusions
This series represents one of the largest reported to date of PCGDTCL. Accurate diagnosis and classification of PCGDTCL need ongoing analysis and delineation. Using strict criteria, only 60% of cases across 10 academic centers were confirmed as bona fide PCGDTCL. Analysis of these patients treated in the modern era demonstrated modest survival. Advanced treatment options and continued collaboration are critically needed in this rare disease.