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