CLINICAL REPORT
835 ActaDV ActaDV Advances in dermatology and venereology Acta Dermato-Venereologica
Aggressive Rare T-cell Lymphomas with Manifestation in the Skin : A Monocentric Cross-sectional Case Study
Marie-Charlotte BRÜGGEN 1 , 2 , Katrin KERL 1 , 2 , Eugenia HARALAMBIEVA 1 , 3 , Urs SCHANZ 1 , 4 , Yun-Tsan CHANG 1 , 2 , Desislava IGNATOVA 1 , 2 , Reinhard DUMMER 1 , 2 , Antonio COZZIO 5 , Wolfram HOETZENECKER 1 , 2 , 6 , Lars E . FRENCH 1 , 2 and Emmanuella
GUENOVA 1 , 2 , 5 1
Faculty of Medicine , 2 Department of Dermatology , 3 Institute of Pathology , 4 Center of Oncology and Hematology , University Hospital Zurich , Zurich , 5 Department of Dermatology , Kantonsspital St Gallen , St Gallen , Switzerland , and 6 Department of Dermatology , Kepler University Hospital , Linz , Austria
Rare T- or NK-cell lymphomas with cutaneous manifestation may display a highly aggressive clinical course and major diagnostic / therapeutic challenges . This report describes our experiences with different lymphomas of this rare category and the therapeutic options used . This retrospective , descriptive , monocentric , cross-sectional case study , identified 4 rare aggressive T- / NK-cell lymphomas with manifestation in the skin , which were diagnosed in a tertiary care centre over a period of 4 years . Two patients had an Epstein-Barr virus-associated extranodal NK / T-cell lymphoma and 2 patients had a primary cutaneous CD8 + aggressive epidermotropic cytotoxic T-cell lymphoma . Concomitant extracutaneous involvement was observed in 2 of all 4 patients . Two patients had fulminant disease progression and resistance to chemotherapy . Two patients underwent allogeneic haematopoietic stem cell transplantation , which resulted in one complete remission and one partial remission . This report emphasizes the importance of an early diagnostic work-up and a prompt aggressive therapeutic approach .
Key words : lymphoma ; manifestation in the skin ; ENKTL ; PC- AETCL ; allogeneic hematopoietic stem cell transplantation .
Accepted Apr 24 , 2018 ; Epub ahead of print Apr 24 , 2018 Acta Derm Venereol 2018 ; 98 : 835 – 841 .
Corr : Emmanuella Guenova , Department of Dermatology , University Hospital Zurich , Gloriastrasse 31 , CH – 8091 Zurich , Switzerland . E-mail : emmanuella . guenova @ usz . ch
Mature T- and NK-cell malignancies are rare .
However , in the skin , approximately 75 % of all cutaneous lymphoma are of T-cell ( approximately 65 %) and NK-cell ( approximately 10 %) origin ( 1 – 5 ). Most of the T-cell lymphomas manifesting in the skin are primary cutaneous , i . e . originating from cells with skin-homing capacity that either permanently resides in the skin or recirculates through the blood ( 6 , 7 ). Skin manifestation occurs in rare cases as the first clinical appearance of peripheral T-cell lymphomas ( 8 , 9 ).
The 2008 WHO classification , revised in 2016 , recognizes extranodal NK / T-cell lymphoma ( ENKTL ) as one of the prototypes of virally associated Epstein-Barr virus ( EBV ) -positive T-cell or NK-cell lymphoma ( 9 – 12 ). ENKTL , nasal type affects adult individuals and is more
SIGNIFICANCE
Aggressive T-cell lymphomas with primary manifestation in the skin are rare and often harbour major diagnostic / therapeutic challenges . In this retrospective descriptive monocentric cross-sectional case study over a period of 4 years , we identified a total of 4 patients suffering from aggressive T-cell lymphomas with primary manifestation in the skin . This corresponded to < 1 % of all newly diagnosed cutaneous lymphoma cases . We observed an aggressive disease course in all our patients and disease progression and resistance to chemotherapy in two of them . Our report emphasizes the importance of an early extensive diagnostic work-up and a prompt aggressive therapeutic approach .
frequent in Asia , Mexico and South America , where it accounts for up to 6 % of all cases of non-Hodgkin ’ s lymphomas ( 13 ). Males are affected 2 – 3 times more often than females ( 14 ). ENKTL nasal type manifests mostly in the nasal / paranasal area and can further involve the skin , gastrointestinal tract and , in rare cases , the bone marrow ( 15 ). An extranasal manifestation of ENKTL is less common , resulting in the suggestion of some authors that , in most cases of extranasal ENKTL , an occult nasal involvement was missed on initial evaluation / staging ( 9 , 16 – 18 ). The prototypic immunophenotype of ENKTL is CD2 + CD56 + . Surface CD3 and other common T-cell antigens ( CD4 , CD8 , CD5 ) are usually negative with positivity for cytoplasmic CD3ε . Some tumours express α / β-T-cell receptor ( α / β-TCR ). γ / δ TCR expression has rarely been reported and molecular analysis only rarely detects a monoclonal rearrangement of the TCR gene ( 19 ). Overall patient survival and clinical features seem to be similar in T- and NK-cell phenotype ENKTL ( 20 ). Characteristically , EBV can be detected in almost all cases of ENKTL ( 21 ).
The prognosis of ENKTL depends on the disease stage . Disease manifestation outside the site of initial tumour manifestation is associated with poor prognosis ( 15 ). Elevated levels of lactate dehydrogenase and systemic symptoms of fever , malaise and weight loss are additional , independent poor prognostic factors ( 22 ).
Primary cutaneous CD8 + aggressive epidermotropic cytotoxic T-cell lymphoma ( PC-AETCL ) has been
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2018 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2950 Acta Derm Venereol 2018 ; 98 : 835 – 841