Lymphoma development and survival in refractory coeliac disease type II: Histological response as prognostic factor.
BACKGROUND
Refractory coeliac disease type II (RCDII) frequently transforms into an enteropathy-associated T-cell lymphoma (EATL) and therefore requires intensive treatment. Current evaluated treatment strategies for RCDII include cladribine (2-CdA) and autologous stem cell transplantation (auSCT).


OBJECTIVE
The purpose of this study was to evaluate long-term survival and define clear prognostic criteria for EATL development comparing two treatment strategies.


METHODS
A total of 45 patients were retrospectively analysed. All patients received 2-CdA, after which they were either closely monitored (monotherapy,n= 30) or a step-up approach was used including auSCT (step-up therapy,n= 15).


RESULTS
Ten patients (22%) ultimately developed EATL; nine of these had received monotherapy. Absence of histological remission after monotherapy was associated with EATL development (p= 0.010). Overall, 20 patients (44%) died with a median survival of 84 months. Overall survival (OS) within the monotherapy group was significantly worse in those without histological remission compared to those with complete histological remission(p= 0.030). The monotherapy group who achieved complete histological remission showed comparable EATL occurrence and OS as compared to the step-up therapy group (p= 0.80 andp= 0.14 respectively).


CONCLUSIONS
Histological response is an accurate parameter to evaluate the effect of 2-CdA therapy and this parameter should be leading in the decisions whether or not to perform a step-up treatment approach in RCDII.
Department of Gastroenterology, VU University Medical Centre, Amsterdam, The Netherlands.
P
P
Nijeboer
Department of Gastroenterology, VU University Medical Centre, Amsterdam, The Netherlands.
Rlj
R
van Wanrooij
Department of Gastroenterology, VU University Medical Centre, Amsterdam, The Netherlands.
T
T
van Gils
Department of Nutrition and Dietetics, VU University Medical Centre, Amsterdam, The Netherlands.
N J
NJ
Wierdsma
Department of Gastroenterology, VU University Medical Centre, Amsterdam, The Netherlands.
G J
GJ
Tack
Department of Epidemiology and Biostatistics, VU University Medical Centre, Amsterdam, The Netherlands.
B I
BI
Witte
Department of Pathology, VU University Medical Centre, Amsterdam, The Netherlands.
H J
HJ
Bontkes
Department of Haematology, VU University Medical Centre, Amsterdam, The Netherlands.
O
O
Visser
Department of Gastroenterology, VU University Medical Centre, Amsterdam, The Netherlands.
Cjj
C
Mulder
Department of Gastroenterology, VU University Medical Centre, Amsterdam, The Netherlands.
G
G
Bouma
Refractory coeliac disease type II; autologous stem cell transplantation; cladribine; coeliac disease; complicated coeliac disease; enteropathy-associated T-cell lymphoma