Histopathological and immunophenotypical criteria for the diagnosis of Sézary syndrome in differentiation from other erythrodermic skin diseases
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Histopathological and immunophenotypical criteria for the diagnosis of Sézary syndrome in differentiation from other erythrodermic skin diseases : a European Organisation for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Task Force Study of 97 cases. / Klemke, C D; Booken, N; Weiss, C; Nicolay, J P; Goerdt, S; Felcht, M; Géraud, C; Kempf, W; Assaf, C; Ortonne, N; Battistella, M; Bagot, M; Knobler, R; Quaglino, P; Arheiliger, B; Santucci, M; Jansen, P; Vermeer, M H; Willemze, R.
In: BRIT J DERMATOL, Vol. 173, No. 1, 07.2015, p. 93-105.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Histopathological and immunophenotypical criteria for the diagnosis of Sézary syndrome in differentiation from other erythrodermic skin diseases
T2 - a European Organisation for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Task Force Study of 97 cases
AU - Klemke, C D
AU - Booken, N
AU - Weiss, C
AU - Nicolay, J P
AU - Goerdt, S
AU - Felcht, M
AU - Géraud, C
AU - Kempf, W
AU - Assaf, C
AU - Ortonne, N
AU - Battistella, M
AU - Bagot, M
AU - Knobler, R
AU - Quaglino, P
AU - Arheiliger, B
AU - Santucci, M
AU - Jansen, P
AU - Vermeer, M H
AU - Willemze, R
N1 - © 2015 British Association of Dermatologists.
PY - 2015/7
Y1 - 2015/7
N2 - BACKGROUND: Patients with erythrodermic disease are a diagnostic challenge regarding the clinical and histological differential diagnosis.OBJECTIVES: To evaluate histopathological and immunohistochemical diagnostic markers for Sézary syndrome.METHODS: Ninety-seven erythrodermic cases [Sézary syndrome (SS), n = 57; erythrodermic inflammatory dermatoses (EIDs), n = 40] were collected by the EORTC Cutaneous Lymphoma Task Force histopathology group. Evaluation criteria were (i) epidermal and dermal changes; (ii) morphology of the infiltrate; (iii) immunohistochemical analysis of marker loss (CD2, CD3, CD4, CD5 and CD7); (iv) bystander infiltrate by staining for CD8, FOXP3 and CD25; and (v) expression of Ki-67, CD30, PD-1 and MUM-1.RESULTS: The workshop panel made a correct diagnosis of SS in 51% of cases (cutaneous T-cell lymphoma 81%) and of EID in 80% without clinical or laboratory data. Histology revealed a significantly increased degree of epidermotropism (P < 0.001) and more intraepidermal atypical lymphocytes (P = 0.0014) in SS biopsies compared with EID. Pautrier microabscesses were seen only in SS (23%) and not in EID (P = 0.0012). SS showed significantly more dermal cerebriform and blastic lymphocytes than EID. Immunohistochemistry revealed a significant loss of CD7 expression (< 50%) in 33 of 51 (65%) cases of SS compared with two of 35 (6%) EID (P < 0.001). The lymphocytic infiltrate in SS skin samples was found significantly to express PD-1 (P = 0.0053), MUM-1 (P = 0.0017) and Ki-67 (P < 0.001), and showed less infiltration of CD8(+) lymphocytes (P < 0.001). A multivariate analysis identified CD7 loss, increased numbers of small cerebriform lymphocytes, low numbers of CD8(+) lymphocytes and increased proliferation (Ki-67(+) lymphocytes) as the strongest indicators for the diagnosis of SS.CONCLUSIONS: A number of different histological and immunophenotypical criteria are required to differentiate between SS and EIDs.
AB - BACKGROUND: Patients with erythrodermic disease are a diagnostic challenge regarding the clinical and histological differential diagnosis.OBJECTIVES: To evaluate histopathological and immunohistochemical diagnostic markers for Sézary syndrome.METHODS: Ninety-seven erythrodermic cases [Sézary syndrome (SS), n = 57; erythrodermic inflammatory dermatoses (EIDs), n = 40] were collected by the EORTC Cutaneous Lymphoma Task Force histopathology group. Evaluation criteria were (i) epidermal and dermal changes; (ii) morphology of the infiltrate; (iii) immunohistochemical analysis of marker loss (CD2, CD3, CD4, CD5 and CD7); (iv) bystander infiltrate by staining for CD8, FOXP3 and CD25; and (v) expression of Ki-67, CD30, PD-1 and MUM-1.RESULTS: The workshop panel made a correct diagnosis of SS in 51% of cases (cutaneous T-cell lymphoma 81%) and of EID in 80% without clinical or laboratory data. Histology revealed a significantly increased degree of epidermotropism (P < 0.001) and more intraepidermal atypical lymphocytes (P = 0.0014) in SS biopsies compared with EID. Pautrier microabscesses were seen only in SS (23%) and not in EID (P = 0.0012). SS showed significantly more dermal cerebriform and blastic lymphocytes than EID. Immunohistochemistry revealed a significant loss of CD7 expression (< 50%) in 33 of 51 (65%) cases of SS compared with two of 35 (6%) EID (P < 0.001). The lymphocytic infiltrate in SS skin samples was found significantly to express PD-1 (P = 0.0053), MUM-1 (P = 0.0017) and Ki-67 (P < 0.001), and showed less infiltration of CD8(+) lymphocytes (P < 0.001). A multivariate analysis identified CD7 loss, increased numbers of small cerebriform lymphocytes, low numbers of CD8(+) lymphocytes and increased proliferation (Ki-67(+) lymphocytes) as the strongest indicators for the diagnosis of SS.CONCLUSIONS: A number of different histological and immunophenotypical criteria are required to differentiate between SS and EIDs.
KW - Adult
KW - Aged
KW - Aged, 80 and over
KW - Antigens, CD/metabolism
KW - Biomarkers, Tumor/metabolism
KW - Biopsy/methods
KW - Diagnosis, Differential
KW - Female
KW - Follow-Up Studies
KW - Forkhead Transcription Factors/metabolism
KW - Humans
KW - Immunohistochemistry
KW - Lymphocytes/pathology
KW - Male
KW - Middle Aged
KW - Phenotype
KW - Prognosis
KW - Programmed Cell Death 1 Receptor/metabolism
KW - Sezary Syndrome/immunology
KW - Skin/pathology
KW - Skin Neoplasms/immunology
U2 - 10.1111/bjd.13832
DO - 10.1111/bjd.13832
M3 - SCORING: Journal article
C2 - 25864856
VL - 173
SP - 93
EP - 105
JO - BRIT J DERMATOL
JF - BRIT J DERMATOL
SN - 0007-0963
IS - 1
ER -