Page 129 - Haematologica3

T-cell non-Hodgkin lymphoma arising in patients with immunodeficiencies
or in combination with tumor necrosis factor (TNF)-α inhibitors.21,22 Even less is known about the development of T-NHL in patients with other immunodeficiencies, as only case reports and some small case series have been published.
Herein, we present a relatively large series of 25 immunodeficient patients in whom T-NHL was diag- nosed in a single referral center in the period 1999 to 2014. In this cohort study, we describe the clinical charac- teristics of these cases and correlate them to the patholog- ical features of T-NHL. Furthermore, we present a review of the literature on T-NHL in immunocompromised patients. To the best of our knowledge, this is the largest series of T-NHL in patients with varying causes of immunodeficiency reported so far.
Methods
Histopathological material and reports from patients treated in or referred to the Academic Medical Center in Amsterdam are stored prospectively in a database. This database was queried for samples on which a T-cell receptor (TCR) gene rearrangement analysis was performed between 1999 and 2014. In our center, analysis of TCR gene rearrangement on tumor tissue is standard practice in the workup if T-NHL is suspected. In cases where the diagnosis of T-NHL was confirmed by histology, molecular testing and clinical features, the corresponding clinical data were searched for the presence of immunodeficiency prior to the diagnosis of T- NHL. For all cases, the biopsies were reviewed and immunohisto- chemical stains for CD2, CD3, CD4, CD5, CD8, granzyme B, PD1, CD30, ALK1, CD21, CD20, TdT, CD56 and in situ hybridiza- tion for Epstein-Barr virus (EBV)-encoded coded ribonucleic acid (RNA; EBER) were analyzed. If these were not performed in the routine diagnostic work-up (especially in older cases), they were additionally performed for this purpose. The lymphomas were (re)classified according to the World Health Organization (WHO)
tion for all patients was collected in an anonymized database. The survival status and the cause of death were determined at the cut- off date of April 1, 2015. For staging, the Ann Arbor system was used for all patients, with the exception of those with cutaneous lymphomas, for whom the Mycosis Fungoides Cooperative Group (MFCG) tumor-node-metastasis (TNM) staging system of cutaneous T-NHL was used.24 Statistical analyses of the data were performed using SPSS (version 23.0 for Windows).
A search in PubMed was performed to find additional cases of T-NHL in patients with varying causes of immunodeficiency using the following search terms: “Immunocompromised”, “immunod- eficiency”, “decreased immunity”, “reduced immunity”, “HIV”, “autoimmune disease”, “rheumatoid arthritis”, “IBD”, “inflamma- tory bowel disease”, “Crohn”, “ulcerative colitis”, “hematologic malignancy”, “Hodgkin”, “Waldenström”, “B-cell lymphoma”, “B- cell lymphoma”, and “leukemia”. These terms were combined using the “AND”-function with the search terms: “T-NHL”, “T-cell lymphoma”, “peripheral T-NHL”, “peripheral T-cell lymphoma” or “PTCL”. When available, Medical Subject Headings (MeSH) terms were used. In order to be included in the review herein, the cases of patients were required to have a pre-existing immunodeficien- cy due to HIV, immunosuppressive therapy, hematologic malig- nancies or a primary immunodeficiency before they developed a T-NHL. Reference lists of selected articles were used to identify additional articles. Articles published in a language other than English were excluded. Articles using earlier published cases were checked and duplicate cases were eliminated. Since more compre- hensive reviews describing case series and previously published cases are available in the literature for T-NHL occurring in solid organ transplant recipients or in HIV patients, only these papers were included.
Results
Patients’ characteristics
A total of 251 T-NHL cases were found in our histopathological database. Forty-two cases, for which no
2008 classification of lymphoid malignancies.23
continued from the previuos page
Clinical informa-
BM/Bone: 2 Skin: 1 Liver: 1
PB: 1 Spleen: 2 Lung: 1
1 (16.7%)
5 (83.3%)
7.2
Extranodal sites
BM/Bone: 4 Skin: 4
Liver: 4 Nasopharynx: 1 Bowel: 1
Lung: 1 Heart: 1 Pancreas: 1 PCO:1
7 (50%)
7 (50%)
62
BM/Bone: 1 Bowel: 2 Heart: 1 CZS: 1
-
4 (100%)
8 96
Lung: 1
BM/Bone: 7 (28%) Skin: 5 (20%) Liver: 5 (20%) Spleen: 3 (12%) Bowel: 3 (12%) Lung:3 (12%) Heart: 2 (8%) Pancreas: 2 (8%) PB:1 (4%)
PCO: 1 (4%) CZS: 1 (4%)
9 (36%)
16 (64%)
11.3
Stage* I/II III/IV
Median survival (m)
1 (100%)
-
*For cutaneous lymphomas the TNMB system was used. All other lymphomas were staged by the Ann Arbor staging system. AI: autoimmune; AITL: angioimmunoblastic T-cell lymphoma; ALCL: anaplastic large cell lymphoma; B-CLL: B-cell chronic lymphatic leukemia; BM: bone marrow; CT: chemotherapy; CD: Crohn’s disease; Dx: diagnosis; CTCL: pri- mary cutaneous T-cell lymphoma; EN: extranodal; ENKL: extranodal NK/T cell lymphoma; HIV: human immunodeficiency virus; HSTCL: hepatosplenic T-cell lymphoma; IS(T): immunosuppressive (therapy); LN: lymphnodes involved; MCL: mantle cell lymphoma; MM: multiple myeloma; NR: not recorded/retraceable; PB: peripheral blood; PCO: pelvic cavity organs; PR: polymyalgia rheumatica; Prec T-LBL: precursor T-cell lymphoblastic lymphoma; Prim. C-CD30+ T-LPD: primary cutaneous CD30+ T-cell lymphoproliferative dis- ease; PSC: primary sclerosing cholangitis; PTCL, NOS: peripheral T-cell lymphoma, not otherwise specified; RA: rheumatoid arthritis; T-NHL: T-cell non-Hodgkin lymphoma; T-PLL; T-cell prolymphocytic leukemia; Tx: transplantation.
haematologica | 2018; 103(3)
489