Blood classification and blood response criteria in mycosis fungoides and Sézary syndrome using flow cytometry: recommendations from the EORTC cutaneous lymphoma task force. (April 2018)

• Record Type: Journal Article
• Authors: Scarisbrick, Julia J.; Hodak, Emmilia; Bagot, Martine; Stranzenbach, Rene; Stadler, Rudolf; Ortiz-Romero, Pablo L.; Papadavid, Evangelia; Evison, Felicity; Knobler, Robert; Quaglino, Pietro; Vermeer, Maarten H.
• Is Part Of: European journal of cancer. Volume 93(2018)
• Page Start: 47
• Page End: 56
• Publication Date: 2018-04
• Subjects: Cutaneous T-cell lymphoma -- CD26 -- CD7 -- Staging -- Classification -- Blood -- Erythroderma; Cancer -- Periodicals; Neoplasms -- Periodicals; Cancer -- Périodiques; Cancer; Tumors; Electronic journals; Periodicals; Electronic journals; 616.994
• DOI: 10.1016/j.ejca.2018.01.076
• Languages: English
• ISSNs: 0959-8049

Abstract

Abstract: Our current mycosis fungoides (MF) and Sézary Syndrome (SS) staging system includes blood-classification from B0-B2 for patch/plaque/tumour or erythroderma based on manual Sézary counts but results from our EORTC survey confirm these are rarely performed in patch/plaque/tumour MF, and there is a trend towards using flow cytometry to measure blood-class. Accurately assigning blood-class effects overall stage and the 'global response' used to measure treatment responses in MF/SS and hence impacts management. The EORTC Cutaneous Lymphoma Task Force Committee have reviewed the literature and held a Workshop (June 2017) to agree a definition of blood-class according to flow cytometry. No large study comparing blood-class as defined by Sézary count with flow cytometry has been performed in MF/SS. The definition of blood-class by flow cytometry varies between publications. Low-level blood involvement occurs in patch/plaque/tumour much less than erythroderma (p < 0.001). The prognostic relevance of blood involvement (B1 or B2) in patch/plaque/tumour is not known. Studies have not shown a statistically worse difference in prognosis in erythrodermic MF patients with low-level blood involvement (IIIB) versus those without (IIIA), but Sezary patients who by definition have a leukaemic blood picture (staged IVA1 or higher) have a worse prognosis. For consistency flow, definition for blood-class must be an objective measurement. We propose absolute counts of either CD4+CD7-or CD4+CD26-where B0<250/μL, B1 = 250/μl–<1000/μL and B2≥1000/μL plus a T-cell blood clone. Fluctuations between B0 and B1 should not be considered in the treatment response criteria until further prognostic information is known. Highlights: Blood-class was introduced into staging of MF/SS in 2007 using manual Sezary counts which are rarely performed in all stages. Flow cytometry has largely replaced manual Sezary counts for monitoring blood involvement in MF/SS. Flow cytometry should define blood class according to absolute counts of CD4+7- or CD4+26- populations. Clinical significance of variation in blood-class between B0/B1 has not been proven and should not be considered in response criteria.