07.02.2020 | T-Cell and Other Lymphoproliferative Malignancies (J Zain, Section Editor) | Ausgabe 2/2020 Open Access

Advances and Perspectives in the Treatment of T-PLL
- Zeitschrift:
- Current Hematologic Malignancy Reports > Ausgabe 2/2020
Publisher’s Note
Introduction
Current Treatment Options
Summary of most relevant clinical studies on chemo-/immunotherapy in T-PLL
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|||||||||
---|---|---|---|---|---|---|---|---|---|
Regime
|
Study design
|
Treatment status
|
n
|
ORR
|
CR
|
PR
|
PFS
|
OS
|
Reference
|
[%]
|
[%]
|
[%]
|
[mo]
|
[mo]
|
|||||
Pentostatin
|
Single center, retrospective
|
Pretreated
|
56
|
45
|
9
|
36
|
6
|
9
|
[
9]
|
Alemtuzumab, iv
|
Single center, retrospective
|
Pretreated
|
15
|
73
|
60
|
13
|
6
|
8
|
[
34]
|
Alemtuzumab, iv
|
Multicenter, prospective
|
Pretreated
|
39
|
76
|
60
|
16
|
7
|
10
|
[
33]
|
Alemtuzumab, iv
|
Multicenter, retrospective
|
Untreated
|
4
|
75
|
75
|
0
|
5
|
8.7
|
[
30]
|
Pretreated
|
72
|
50
|
37.5
|
12.5
|
4.5
|
7.5
|
|||
Pentostatin + alemtuzumab, iv
|
Single center, prospective
|
Pretreated
|
13
|
69
|
62
|
8
|
7.8
|
10.2
|
[
39]
|
Alemtuzumab, iv
|
Single center, prospective
|
Untreated
|
32
|
91
|
81
|
10
|
12
|
48
|
[
10]
|
Pretreated
|
45
|
74
|
60
|
14
|
12
|
48
|
|||
Alemtuzumab, sc
|
Untreated
|
9
|
33
|
33
|
0
|
12
|
48
|
||
FMC + alemtuzumab, iv
|
Multicenter, prospective
|
Untreated
|
16
|
92
|
48
|
44
|
11.5
|
17.1
|
[
5]
|
Pretreated
|
9
|
||||||||
Bendamustine
|
Multicenter, retrospective
|
Untreated
|
6
|
55.3
|
20
|
33.3
|
5
|
8.7
|
[
24]
|
Pretreated
|
9
|
||||||||
Alemtuzumab, iv
|
Single center, retrospective
|
Untreated
|
13
|
n.a.
|
n.a.
|
n.a.
|
n.a.
|
40.5
|
[
32]
|
Alemtuzumab, sc
|
Single center, retrospective
|
Pretreated
|
5
|
n.a.
|
n.a.
|
n.a.
|
n.a.
|
13.7
|
[
32]
|
Alemtuzumab, iv + cladribine +/− HDAC inhibition
|
Single center, retrospective
|
Untreated
|
4
|
100
|
75
|
25
|
6.3
|
14.8
|
[
52]
|
Pretreated
|
4
|
100
|
100
|
0
|
11.35
|
23.7
|
|||
Alemtuzumab, iv
|
Single center, retrospective
|
Untreated
|
42
|
81
|
61
|
20
|
11
|
15
|
[
31•]
|
Pretreated
|
15
|
46
|
46
|
0
|
3
|
15
|
|||
Alemtuzumab, iv + pentostatin
|
Untreated
|
13
|
82
|
73
|
9
|
4.3
|
10.4
|
||
Pretreated
|
5
|
75
|
50
|
25
|
2.6
|
2.6
|
|||
FMC + alemtuzumab, sc
|
Multicenter, prospective
|
Untreated
|
13
|
68.7
|
32.1
|
36.6
|
7.5
|
11.5
|
[
26•]
|
Pretreated
|
5
|
Conventional Cytostatics
Alemtuzumab Remains the Current Benchmark
Hematopoietic Stem Cell Transplantation
Study design
|
n
|
Age [years]
|
Relapse rate [%]
|
TRM [%]
|
Pre-HSCT CR [%]
|
Post-HSCT CR [%]
|
PFS [mo]
|
OS
|
Reference
|
---|---|---|---|---|---|---|---|---|---|
Summary of clinical studies on auto-HSCT in T-PLL
|
|||||||||
Multi-center, retrospective
|
15
|
58
|
60
|
7
|
87
|
100
|
n.a.
|
52 mo
|
[
42]
|
Summary of clinical studies on allo-HSCT in T-PLL
|
|||||||||
Multicenter, retrospective
|
13
|
51
|
33
|
31
|
69
|
92
|
n.a.
|
33mo
|
[
42]
|
CIBMTR registry, retrospective
|
21
|
54
|
39*
|
28*
|
n.a.
|
n.a.
|
5.1
|
11.2mo
|
[
43]
|
EBMT registry, retrospective
|
41
|
51
|
41**
|
41**
|
27
|
n.a.
|
10
|
21%**
|
[
41]
|
Multicenter, prospective
|
5
|
n.a.
|
n.a.
|
n.a.
|
n.a.
|
n.a.
|
n.a.
|
24.8mo
|
[
5]
|
French Society of SCT, retrospective
|
27
|
53
|
47
|
31**
|
52
|
78
|
26**
|
36%**
|
[
44]
|
Single-center, retrospective
|
11
|
56
|
21***
|
34***
|
91
|
91
|
15
|
56mo
|
[
45]
|
EBMT registry, prospective (a)
|
37
|
56
|
38***
|
32***
|
62
|
n.a.
|
30***
|
42%***
|
[
47••]
|
TRUMP registry, retrospective
|
20
|
54
|
69.6**
|
20.9*
|
30
|
n.a.
|
33.5%**
|
40%**
|
[
46]
|
New Rational-based Approaches at Conceptual Stages and with First (Pre)Clinical Evidence
Novel therapeutic approaches
|
|
---|---|
Strategy
|
Reference
|
Epigenetic modulation
|
|
P53 reactivation
|
|
Inhibition of constitutive JAK/STAT signaling
|
|
Antagonists of BCL2 family molecules
|
|
Inhibitors of cyclin-dependent kinases
|
[
18••]
|
Modulation of immune cell synapses
|
[
76]
|
CAR-T cell therapy
|
[
79••]
|