Evaluation of the Cutaneous Lymphoma International Prognostic Index in patients with early stage mycosis fungoides

Amorim, Gustavo Moreira; Corbellini, João Paulo Niemeyer; Quintella, Danielle Carvalho; Cuzzi, Tullia; Ramos-e-Silva, Márcia

doi:10.1590/abd1806-4841.20187134

Gustavo Moreira Amorim Mailing Address: Gustavo Moreira Amorim. E-mail: gustavomoreiraamorim@hotmail.com

Program of Post-graduation in Anatomical Pathology, Hospital Universitário Clementino Fraga Filho, Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.

Outpatient clinic of Dermatology, Faculdade de Medicina, Universidade do Sul de Santa Catarina, Palhoça (SC), Brazil.

https://orcid.org/0000-0001-6067-9463

João Paulo Niemeyer Corbellini

Dermatology Division, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.

https://orcid.org/0000-0001-8104-3915

Danielle Carvalho Quintella

Pathology Division, Hospital Federal de Bonsucesso, Rio de Janeiro (RJ), Brazil.

Department of Pathology, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.

https://orcid.org/0000-0001-9013-9417

Tullia Cuzzi

Department of Pathology, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.

Instituto Nacional de Infectologia Evandro Chagas, Fundação Oswaldo Cruz, Rio de Janeiro (RJ), Brazil.

https://orcid.org/0000-0002-3331-5290

Márcia Ramos-e-Silva

Dermatology Division, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.

Discipline of Dermatology, Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.

https://orcid.org/0000-0003-1625-0760

Mailing Address: Gustavo Moreira Amorim. E-mail: gustavomoreiraamorim@hotmail.com

Conflict of interest: None.

AUTHORS'CONTRIBUTIONS

Gustavo Moreira Amorim

0000-0001-6067-9463

Critical review of the literature; Design and planning of the study; Collecting, analysis and interpretation of data; Statistical analysis; Preparation and writing of the manuscript

João Paulo Niemeyer Corbellini

0000-0001-8104-3915

Critical review of the literature; Intellectual participation in propaedeutic and/or therapeutic conduct of studied cases; Statistical analysis

Danielle Carvalho Quintella

0000-0001-9013-9417

Critical review of the literature; Intellectual participation in propaedeutic and/or therapeutic conduct of studied cases; Critical review of the manuscript

Tullia Cuzzi

0000-0002-3331-5290

Effective participation in research orientation; Design and planning of the study; Critical review of the manuscript; Approval of the final version of the manuscript

Márcia Ramos-e-Silva

0000-0003-1625-0760

Effective participation in research orientation; Design and planning of the study; Critical review of the manuscript; Approval of the final version of the manuscript

Cutaneous involvement
T1	Patches, papules and/or plaques limited to 10% of the body surface area T1a (Patches)/T1b (Patches and plaques)
T2	Patches, papules and/or plaques involving more than 10% of the body surface area T2a (Patches)/T2b (Patches and plaques)
T3	One or more tumors (equal to or larger than 1cm)
T4	Confluence of erythema involving at least 80% of the body surface area
Lymph node involvement
N0	No abnormalities in the peripheral lymph nodes
N1	Presence of abnormal lymph node. Dutch grade I on histopathology. N1a (negative clone)/ N1b (positive clone)
N2	Presence of abnormal lymph node. Dutch grade II on histopathology. N2a (negative clone)/ N2b (positive clone)
N3	Presence of abnormal lymph node. Dutch grade III or IV on histopathology. Negative or positive clone. Dutch histopathology grading system for the classification of lymph node involvement is recognized by ISCL/EORTC
Nx	Presence of abnormal lymph node, with no histological evaluation/confirmation
Visceral involvement
M0	No visceral involvement
M1	Presence of visceral involvement, confirmed by histopathology. The organ involved must be specified
Peripheral blood involvement
B0	Absence of peripheral blood involvement (equal to or less than 5% of atypical lymphocytes in the peripheral blood - Sézary cells). B0a (negative clone)/B0b (positive clone)
B1	Presence of more than 5% of atypical lymphocytes, not fulfilling criteria for B2. B1a (negative clone)/B1b (positive clone)
B2	Presence of 1,000 Sézary cells per cubic millimeter or more in the peripheral blood, with positive clone

Staging	T	N	M	B
IA	1	0	0	0 or 1
IB	2	0	0	0 or 1
IIA	1 or 2	1 or 2	0	0 or 1
IIB	3	0 or 2	0	0 or 1
III	4	0 or 2	0	0 or 1
IIIA	4	0 or 2	0	0
IIIB	4	0 or 2	0	1
IVA1	1 or 4	0 or 2	0	2
IVA2	1 or 4	3	0	0 or 2
IVB	1 or 4	0 or 3	1	0 or 2

Early stage MF:
Male gender
Age > 60 years
Plaques
Folliculotropism
N1/NX (TNMB staging)
Interpretation: 0 - 1 (low risk)/2 (intermediate risk)/3 - 5 (high risk)
Late stage MF:
Male gender
Age > 60 years
B1/B2 (TNMB staging)
N1/NX (TNMB staging)
Visceral involvement
Interpretation: 0 – 1 (low risk)/2 (intermediate risk)/3 – 5 (high risk) Adapted: Benton EC et al., 2013.¹²12 Benton EC, Crichton S, Talpur R, Agar NS, Fields PA, Wedgeworth E, et al. A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. Eur J Cancer. 2013;49:2859-68.

Variables independent	Dependent variables
Variables independent	Progression						Related death
	N°	Freq (%)	N°	Freq (%)	PR	P	CI 95%	N°	Freq (%)	PR	P	95% CI
Male	55	53.9	19	34.5	1.4	0.1	0.7 - 2.7	4	7.3	0.8	0.5	0.2 - 3.2
> 60 years	50	49.0	16	32.0	1.1	0.3	0.6 - 2.1	3	6.0	0.6	0.3	0.1 - 2.4
Plaques	56	54.9	22	39.3	1.3	0.01	1.061 - 1.745	6	10.7	1.0	0.2	0.9 - 1.1
Folliculotropism	6	5.9	1	16.7	0.5	0.4	0.1 - 3.3	0	0	-	-	-
N1/Nx	0	0	-	-	-	-	-	-	-	-	-	-

			Progression			Related death			5-year survival
CLIPi score:	N	Freq (%)	N	Freq (%)	P	N	Freq (%)	P	(%)
1. Low risk	43	42.2	8	18.6	0.1	3	7	0.7	93
2. Intermediate risk	39	38.2	14	35.9		4	10.3		89.7
3. High risk	20	19.6	8	40		1	5		95

Progression						Related death
CLIPi score	N°	Freq (%)	PR	P	95% CI	N°	Freq (%)	PR	P	95% CI
1. Low risk	8	18.6	0.4	0.04	0.2 - 1.0	3	7	0.8	0.7	0.2 - 3.2
2. Interm. and high	22	37.3	1.2		1.018 - 1.655	5	8.6	1.0		0.9 - 1.1

[1] Mailing Address: Gustavo Moreira Amorim. E-mail: gustavomoreiraamorim@hotmail.com

Brasil

Brasil

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