Page 132 - Haematologica3

M.L. Nijland et al.
Table 4. Distribution of histologic subtypes in cases reported in the literature.
HIV Post tx AID HM Other ID Total
ALCL 52 16 12 3 1 84 ALK+ 2422 10 ALK– 27 3 1 1 32 ALKNR 23127 42
AITL 2 31 6 ATLL116 17 CTCL 48 48 34 39 4 173 EATL 1 1 ENKL 1 8 3 1 13 HSTCL 1 23 47 1 1 73 IVL 1 1 NKL 6 6 NKTCL104 14 PrecT-LBL 8 8 PTCL,NOS 76 53 5 10 2 146 SPTCL 3519
T-LGL
Other T-NHL Total
201
9
11 34
197 143
55
9 45 9 605
492
AID: autoimmune disease; AITL: angioimmunoblastic T-cell lymphoma; ALCL: anaplastic large cell lymphoma, systemic; ALK: anaplastic lymphoma kinase; ATLL: adult T-cell leukemia/lymphoma; CTCL: primary cutaneous T-cell lymphoma; EATL: enteropathy-associated T-cell lymphoma; ENKL: extranodal NK/T-cell lymphoma; HM: hematologic malig- nancies; HSTCL: hepatosplenic T-cell lymphoma; ID: immunodeficiency; IVL: intravascular lymphoma; NKL: natural killer cell lymphoma(/leukemia); NKTCL: natural killer/T-cell lymphoma; NR: Not recorded or not known; PTCL, NOS: peripheral T-cell lymphoma, not otherwise specified; Prec T-LBL: precursor T-cell lymphoblastic lymphoma; SPTCL: sub- cutaneous panniculitis-like T-cell lymphoma; T-LGL: T-cell large granular lymphocytic leukemia; T-NHL: T-cell non-Hodgkin lymphoma; tx: transplantation.
those cases for which this information was available, were lymphoma progression (N=6) or sepsis (N=2). One patient developed two other malignancies and the exact cause of death remained unknown.
Discussion
In the general population, T-cell neoplasms are uncom- mon, representing about 5 to 10% of all NHL in Western countries.26 While B-cell lymphomas are known to occur more frequently in transplant recipients and in patients with IBD, HIV or autoimmune diseases,3,5,6,11 for T-NHL this data has not been elucidated as of yet. Since T-NHL are tumors of the immune system, akin to B-cell lym- phomas, it is likely that the risk of developing a T-NHL is also increased in patients with an impaired immune sys- tem. However, a higher incidence of T-NHL has only been reported for a few specific patient groups, including HIV patients,14,15 solid organ transplant recipients19 and patients with a history of coeliac disease, psoriasis or eczema.33,34 Furthermore, a higher incidence of the rare hepatosplenic T-NHL has been documented in IBD patients on thiop- urines.21
The present study of 25 patients is the largest case series of T-NHL in patients with varying causes of acquired immunodeficiencies published thus far. These 25 patients, diagnosed at our center between 1999 and 2014, consti- tute 12% of the total number of cases of T-NHL in which clinical data were available. Three of these 25 patients developed AITL, which may be accompanied by autoim-
mune features such as arthritis and synovitis, and can therefore resemble RA.35 The autoimmune diseases in these three patients were RA, polymyalgia rheumatica and sarcoidosis, which had been diagnosed 30 years, five years and three years, respectively, prior to the diagnosis of AITL. These lengthy intervals signify that it is most unlikely that they were manifestations of the AITL itself, since arthritis occurring more than six months in advance of the diagnosis is extremely uncommon.35
To determine whether T-NHL occurs more often in immunocompromised patients than in the general popula- tion, the prevalence of patients with an immunodeficiency within the cohort of patients with T-NHL should be com- pared to the prevalence of patients with an immunodefi- ciency in the general population. Since no data are avail- able on the overall prevalence of immunodeficiencies in the general population, we compared the frequencies of specific underlying disorders in the Dutch context. We did this for IBD, B-CLL and RA, the three most consistent underlying disorders which we witnessed, apart from solid organ transplantation for which an association with the occurrence of T-NHL is already known.17,19,30 Based on a prevalence of IBD in 432 patients per 100,000 inhabi- tants in 2010,36 we would expect that 0.90 of the 209 T- NHL would occur in patients with IBD, compared to the seven we actually found. For B-CLL, a prevalence of 5,061 patients (average number in 2013/2014)37 out of a total population of 16,829,28938 leads to an expectation of 0.063 patients with B-CLL in our cohort, within which we iden- tified an actual number of four patients. A similar calcula- tion for RA, which had a prevalence of 116,000 patients in
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