CLINICAL REPORT
735
ActaDV ActaDV Advances in dermatology and venereology Acta Dermato-Venereologica
Morbidity and Causes of Death in Patients with Cutaneous T-cell Lymphoma in Finland
Liisa VÄKEVÄ 1 , Tuomas LIPSANEN 1 , Harri SINTONEN 2 and Annamari RANKI 1
1
Center of Inflammation , Department of Dermatology , Allergology and Venereal Diseases , University of Helsinki and Helsinki University Central Hospital , and 2 Department of Public Health , University of Helsinki , Helsinki , Finland
Cutaneous T-cell lymphomas ( CTCL ), especially mycosis fungoides , can be considered as a state of longstanding low-grade systemic inflammation . Many studies have focused on secondary cancers with CTCL , but information about comorbidities is limited . A total of 144 patients with CTCL at Helsinki University Central Hospital during 2005 to 2015 were studied to determine associated comorbidities and causes of death in this cohort . Compared with an age-standardized control population , the prevalence of type 2 diabetes mellitus was increased among patients with CTCL with no link to obesity . Patients with CTCL had a lower prevalence of hypertension , myocardial infarction and stroke than the control group . The 3 most common causes of death were CTCL , coronary artery disease and lung cancer . The increased risk of myocardial infarction or stroke reported previously was not detected in this patient group .
Key words : mycosis fungoides ; cutaneous T-cell lymphomas ; morbidity ; diabetes mellitus .
Accepted Feb 7 , 2017 ; Epub ahead of print Feb 8 , 2017 Acta Derm Venereol 2017 ; 97 : 735 – 738 .
Corr : Liisa Väkevä , Center of Inflammation , Department of Dermatology , Allergology and Venereal Diseases , Helsinki University Central Hospital , FIN-00250 Helsinki , Finland . E-mail : Liisa . vakeva @ hus . fi
Cutaneous T-cell lymphomas ( CTCL ) are a rare group of non-Hodgkin ’ s lymphomas in which neoplastic cells primarily home to the skin . The most common type of CTCL is mycosis fungoides ( MF ), the clinical course of which is usually very indolent , depending on the stage of the disease . In the early stages the prognosis is favouable , but progression to tumours or nodal involvement predicts a poor prognosis ( 1 ). Sézary syndrome ( SS ) and subcutaneous panniculitis-like T-cell lymphoma ( SPTL ) are rare forms of CTCL . SS is understood to arise from a different subset of T cells ( central memory cells ) and is currently not considered to be a leukaemic variant of MF , where the cells arise from skin resident-effector memory T cells ( 2 ).
MF is understood to arise through longstanding chronic inflammation , and can be considered as a state of longstanding low-grade systemic inflammation ( 3 ). Chronic systemic inflammation has recently been accepted to be an important contributor to metabolic diseases . Inflammatory mediators released in chronic inflammation can lead to hypertension , type 2 diabetes mellitus ( DM2 ) and atherogenesis ( 4 , 5 ). Psoriasis is an example of chronic systemic inflammation with an elevated risk of cardiovascular diseases . In addition , patients with atopic dermatitis appear to have increased risk of cardiovascular disease , heart attack and stroke ( 6 , 7 ). In advanced stages of CTCL the immunoprofile is polarized towards Th2- activated memory cells , similarly to atopic dermatitis .
Most previous studies of comorbidities in CTCL have focussed on cancer . These studies have demonstrated an increased risk of secondary cancers in patients with CTCL ( 8 – 11 ). There are only a few previous studies concerning other comorbidities in CTCL ( 12 – 14 ). In MF the recently reported increased risk of myocardial infarction may be mediated by chronic inflammation ( 14 ). Evaluation of cardiovascular risk factors in patients with MF has demonstrated an increased rate of cardiovascular risk related to higher levels of total cholesterol and low-density lipoprotein ( LDL ) cholesterol than in control patients ( 12 ). Interestingly , MF has also been linked to increased risk of anxiety and depression ( 13 ).
The prognosis and survival of patients with CTCL is dependent on the stage of the disease ( 15 – 17 ). Patients with non-advanced stages of CTCL seldom die of the disease itself , and reports of causes of death in this patient group are sparse .
The aims of this study were to examine comorbidities in the largest cohort of Finnish patients with CTCL and to assess the most common causes of death in this patient group .
PATIENTS AND METHODS
A total of 144 patients with CTCL , treated in the Skin and Allergy Hospital , Helsinki University Central Hospital ( HUCH ), Helsinki , Finland , during the period 1 January 2005 to 18 February 2015 were analysed retrospectively .
Patient search was based on a confirmed dermatological and histopathological diagnosis . The majority of patients had a diagnosis of MF ( n = 122 ). There were 8 patients with SS , 8 with SPTL , and 6 with anaplastic large cell lymphoma . All except one of the patients with SPTL had an αβ-phenotype . Clinical patient data and comorbidity data was collected from the comprehensive electronic medical patient records of HUCH . Staging of the patients was performed according to current guidelines ( 15 ). Systemic treatment information was also collected from patient charts . Demographic and survival data for the 144 CTCL patients is presented in Table I .
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2017 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2629 Acta Derm Venereol 2017 ; 97 : 735 – 738