Author(s): Scarisbrick JJ, Hodak E, Bagot M, Stranzenbach R, Stadler R, et al.
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.
Author(s): Scarisbrick JJ, Whittaker S, Evans AV, Fraser-Andrews EA, Child FJ, et al.
Author(s): Vonderheid EC, Pena J, Nowell P
Author(s): Fraser-Andrews EA, Russell-Jones R, Woolford AJ, Wolstencroft RA, Dean AJ, et al.
Author(s): Brüggemann M, White H, Gaulard P, Garcia-Sanz R, Gameiro P, et al.
Author(s): Gibson JF, Huang J, Liu KJ, Carlson KR, Foss F, et al.
Author(s): Novelli M, Fava P, Sarda C, Ponti R, Osella-Abate S, et al.
Author(s): Hurabielle C, Thonnart N, Ram-Wolff C, Sicard H, Bensussan A, et al.
Author(s): Mahe E, Pugh T, Kamel-Reid S