Table 1 Patient and disease characteristics of the study population.

From: Central nervous system involvement in patients with Richter transformation of chronic lymphocytic leukemia (CLL): the Mayo Clinic experience

Phase of disease

Number (N = 36)

%

Chronic lymphocytic leukemia

Sex

Male

21

58.3

Female

15

41.7

Age at diagnosis (years)

Median (range)

63 (34–78)

 

IGHV

Mutated

7

46.7

Unmutated

8

53.3

Missing

21

 

CLL FISH by Dohner hierarchy

Del(17p)

9

34.6

Del(11q)

0

0

Trisomy 12

6

23.1

Negative

7

26.9

Del(13q)

4

15.4

Missing

10

 

TP53 somatic mutation

Negative

7

63.6

Positive

4

36.4

Missing

25

 

TP53 alteration (Del(17p) and/or TP53 somatic mutation)

Negative

13

52.0

Positive

12

48.0

Missing

11

 

CLL treatment status prior to RT

Untreated

24

66.7

Treated

12

33.3

Lines of CLL treatment prior to RT

Median (range) in all

0 (0–5)

 

Median (range) in treated (n = 12)

1.5 (1–5)

 

Richter transformation

Time to transformation (months)

Median (range)

17.3 (0–277.4)

 

Age at RT diagnosis (years)

Median (range)

69 (46–79)

 

Pathology at RT

DLBCL

33

91.7

HGBCL

3

8.3

Cell of origin

GCB

13

61.9

Non-GCB

8

38.1

Missing

15

 

MYC IHC

Negative

4

20.0

Positive (≥40%)

16

80.0

Missing

16

 

BCL2 IHC

Negative

5

20.0

Positive (≥50%)

20

80.0

Missing

11

 

MYC/BCL2 double expressor

Positive

10

27.8

MYC FISH

Negative

13

61.9

Positive

8

38.1

Missing

15

 

BCL2 FISH

Negative

13

81.2

Positive

3

18.8

Missing

20

 

BCL6 FISH

  

Negative

16

100.0

Positive

0

0

Missing

20

 

Double-/triple-hit

MYC/BCL2 Double-hit

2

5.6

PD-1 IHC

Negative

9

52.9

Positive

8

47.1

Missing

19

 

EBV IHC

Negative

15

93.7

Positive

1

6.3

Missing

20

 

MYD88 mutation

Negative

4

66.6

Positive

2

33.3

Missing

30

 

Ki-67 (n = 16)

Median (range)

90% (25–100%)

 

LDH

Elevated

33

91.7

Normal

3

8.3

Lines of treatment for RT before CNS involvement

Median (range) in all

0 (1–5)

 

Median (range) in treated (n = 15)

1 (1–5)

 

Clonal relatedness with CLL

Related

12

75.0

Unrelated

4

25.0

Missing

20

 

CNS involvement of Richter transformation

Time from RT diagnosis to CNS involvement of RT (months)

Median (range) in all

0 (0–41.6)

 

Median (range) in those with CNS involvement at RT progression (n = 14)

11.1 (1–41.6)

 

Age at CNS RT diagnosis

Median (range)

69 (46–80)

 

CNS biopsy or imaging confirmation

Tissue biopsy

16

44.4

CSF

5

13.9

Vitreous fluid

3

8.3

Imaging

12

33.3

Timing of CNS involvement

At RT diagnosis

22

61.1

At RT progression

14

38.9

Extent of CNS involvement

Isolated CNS involvement

19

52.8

CNS and systemic involvement

17

47.2

Site of CNS involvement

Parenchymal only

12

33.3

Parenchymal + othersa

11

30.6

Othersb

13

36.1

  1. aParenchymal + others: parenchymal + leptomeningeal, cranial nerve roots, or ocular disease.
  2. bOthers: leptomeningeal, ocular, cranial or spinal nerve roots, and skull base lesions.