Table 1. Clinical, pathological, and... | American Society of Hematology

Table 1.

Clinical, pathological, and genetic features of the patients reported in this study

Patient	Age, y	Immune profile	Sample at diagnosis	Karyotype*	FISH pattern	Mutation status	Stage†	Treatment/ response	Status	Survival, m
1	70	CD4⁺, CD5⁺, CD7⁺, CD10⁺, BCL6⁺, PD1 (ICOS and CXCL13: NA), EBER⁺	Lymph node	46,XX,der(1)(5q35.3→5q33.3::5q21.3→5q33.3::1p34→1q44),der(5)t(1;5)(p34;q21.3)	SYK BA: 2F	NF	II	2007: rituximab; CR; 2010/Rl: rituximab and mini-CHOP; NR; RT/PR; 2011/progression chlorambucil	Dead	60
					FER BA: 1F1R1G
					ITK BA: NI
2	61	CD4⁺, CD5⁺, CD7⁺, CD10⁺, BCL6⁺, PD1⁺, ICOS⁺, CXCL13⁺, EBER⁺	Lymph node	47,XX,+X,+2,del(6)(q11.1q22.3),+7,der(8)t(8;18)(p11.2;q12.1),der(15) t(15;16)(q26.1;q22.1),der(16)(20q13.3→20p13::16p13.3→p13.1::16p11.1→p13.1::16p13.1→q22.1::15q26.1→q26.3),–18,–20	SYK BA: 2F	TET2^H1380Y	II	CHOP, remains PET positive	Dead	8
					ITK BA: 2F	TET2^S1449fs
					FER BA: 2F
					FES BA: 1F1R1G
3	83	CD4⁺, CD2^–, CD7^–, CD10⁺, BCL6^–, PD1⁺, ICOS⁺, CXCL13⁺, EBER⁺	Spleen	46,X,–X,der(2)dup(2)(q33.1q33.3)t(2;11)(q37.3;q23.3),t(3;14)(p13;q32.2),del(5)(q14.2q23.2),del(6)(p21.31p24.1),der(8)t(8;8)(p11.21;q22.3),der(11)t(X;11)(q21.1;p15.5),+19	SYK BA: 2F	RHOA^G17V	IVBS	Splenectomy plus chlorambucil; progressive disease	Dead	1
					ITK BA: 2F	IDH2^R172K
					FER BA: 1F	TET2^T1499fs
					FES BA: 2F	TET2^Q1687X
					LSI IGH: 14F, der(3)F
4	71	CD3⁺, CD4⁺, CD5⁺, CD23⁺/ FDC, PD1⁺, CXCL13⁺, ICOS⁺, CD10p⁺, BCL6w⁺, EBER⁺	Lymph node	46,XX	SYK BA: 2F	RHOA^G17V	III	CHOP; CR	Alive	160
					ITK BA: 2F	IDH2^R172S
					FER BA: 2F	TET2^Q769X
					FES BA: 2F	TET2^W1219fs
						DNMT3A^R771P
5	58	CD3⁺, CD4⁺, CD5⁺, CD7^–, CD10^–, PD1⁺, ICOS⁺, BCL6⁺, CXCL13⁺, CD21⁺/FDC, EBER⁺	Lymph node	46,XX	SYK BA: 2F	RHOA^G17V	IIIS	CHOP; CR	Alive	111
					ITK BA: 2F	IDH2^R172S
					FER BA: 2F	TET2^N1714fs
					FES BA: 2F	TET2^K1752fs
6	76	CD3⁺, CD4⁺, PD1⁺, ICOS⁺, CXCL13⁺, CD10⁺, BCL6⁺, CD23⁺/FDC, EBER⁺	Lymph node	47,XX,+5	SYK BA: 2F	RHOA^G17V	IIIS	CHOP; CR	Alive	20
					ITK BA: 3F	IDH2^R172M
					FER BA: 3F	TET2^Y1294C
					FES BA: 2F

Patient	Age, y	Immune profile	Sample at diagnosis	Karyotype*	FISH pattern	Mutation status	Stage†	Treatment/ response	Status	Survival, m
1	70	CD4⁺, CD5⁺, CD7⁺, CD10⁺, BCL6⁺, PD1 (ICOS and CXCL13: NA), EBER⁺	Lymph node	46,XX,der(1)(5q35.3→5q33.3::5q21.3→5q33.3::1p34→1q44),der(5)t(1;5)(p34;q21.3)	SYK BA: 2F	NF	II	2007: rituximab; CR; 2010/Rl: rituximab and mini-CHOP; NR; RT/PR; 2011/progression chlorambucil	Dead	60
					FER BA: 1F1R1G
					ITK BA: NI
2	61	CD4⁺, CD5⁺, CD7⁺, CD10⁺, BCL6⁺, PD1⁺, ICOS⁺, CXCL13⁺, EBER⁺	Lymph node	47,XX,+X,+2,del(6)(q11.1q22.3),+7,der(8)t(8;18)(p11.2;q12.1),der(15) t(15;16)(q26.1;q22.1),der(16)(20q13.3→20p13::16p13.3→p13.1::16p11.1→p13.1::16p13.1→q22.1::15q26.1→q26.3),–18,–20	SYK BA: 2F	TET2^H1380Y	II	CHOP, remains PET positive	Dead	8
					ITK BA: 2F	TET2^S1449fs
					FER BA: 2F
					FES BA: 1F1R1G
3	83	CD4⁺, CD2^–, CD7^–, CD10⁺, BCL6^–, PD1⁺, ICOS⁺, CXCL13⁺, EBER⁺	Spleen	46,X,–X,der(2)dup(2)(q33.1q33.3)t(2;11)(q37.3;q23.3),t(3;14)(p13;q32.2),del(5)(q14.2q23.2),del(6)(p21.31p24.1),der(8)t(8;8)(p11.21;q22.3),der(11)t(X;11)(q21.1;p15.5),+19	SYK BA: 2F	RHOA^G17V	IVBS	Splenectomy plus chlorambucil; progressive disease	Dead	1
					ITK BA: 2F	IDH2^R172K
					FER BA: 1F	TET2^T1499fs
					FES BA: 2F	TET2^Q1687X
					LSI IGH: 14F, der(3)F
4	71	CD3⁺, CD4⁺, CD5⁺, CD23⁺/ FDC, PD1⁺, CXCL13⁺, ICOS⁺, CD10p⁺, BCL6w⁺, EBER⁺	Lymph node	46,XX	SYK BA: 2F	RHOA^G17V	III	CHOP; CR	Alive	160
					ITK BA: 2F	IDH2^R172S
					FER BA: 2F	TET2^Q769X
					FES BA: 2F	TET2^W1219fs
						DNMT3A^R771P
5	58	CD3⁺, CD4⁺, CD5⁺, CD7^–, CD10^–, PD1⁺, ICOS⁺, BCL6⁺, CXCL13⁺, CD21⁺/FDC, EBER⁺	Lymph node	46,XX	SYK BA: 2F	RHOA^G17V	IIIS	CHOP; CR	Alive	111
					ITK BA: 2F	IDH2^R172S
					FER BA: 2F	TET2^N1714fs
					FES BA: 2F	TET2^K1752fs
6	76	CD3⁺, CD4⁺, PD1⁺, ICOS⁺, CXCL13⁺, CD10⁺, BCL6⁺, CD23⁺/FDC, EBER⁺	Lymph node	47,XX,+5	SYK BA: 2F	RHOA^G17V	IIIS	CHOP; CR	Alive	20
					ITK BA: 3F	IDH2^R172M
					FER BA: 3F	TET2^Y1294C
					FES BA: 2F

BA, break-apart; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisone; CR, complete remission; F, fused signal; FDC, follicular dendritic cell; G, green signal; NA, not available; NF, not found; NI, not informative because of a poor resolution of signals on inv(5); NR, no response; p, partial; PET, positron emission tomography; PR, partial response; R, red signal; Rl, relapse; RT, radiotherapy; w, weak.

Corrected after FISH and array comparative genomic hybridization studies (data not shown).

†

Staging according to Ann Arbor.

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