Table 4 Post-alloHCT treatment in patients with refractory/relapse disease.

From: Allogeneic Hematopoietic Cell Transplantation in Advanced Systemic Mastocytosis: A retrospective analysis of the DRST and GREM registries

 

All

ASM/SM-AHN

SM-AML

MCL ± AHN

Number of patients, n (%)

30/71 (42)

9/30 (30)

10/28 (36)

11/13 (85)

Refractory disease, n (%)

9/30 (30)

0/9 (0)

1/10 (10)

8/11 (73)

 SM, n (%)

8/9 (89)

–

0/1 (0)

8/8 (100)

 AHN, n (%)

0/9 (0)

–

0/1 (0)

0/8 (0)

 SM + AHN, n (%)

1/9 (11)

 

1/1 (100)

0/8 (0)

Relapse disease, n (%)

21/30 (70)

9/9 (100)

9/10 (90)

3/11 (27)

 SM, n (%)

8/21 (38)

4/9 (44)

2/9 (22)

2/3 (67)

 AHN, n (%)

12/21 (57)

5/9 (56)

6/9 (67)

1/3 (33)

 SM + AHN, n (%)

1/21 (5)

0/9 (0)

1/10 (10)

0/3 (0)

Years to relapse, median (range)

0.6 (0.1-4.5)

0.6 (0.3-4.5)

0.4 (0.1-4.1)

1 (0.4-1.4)

Post-alloHCT treatment

n (%)

Response, n (%)

n (%)

Response, n (%)

n (%)

Response, n (%)

n (%)

Response, n (%)

Lines of therapy, median (range)

2 (0–4)

–

1 (1–4)

–

2 (0–4)

–

2 (0–4)

–

Tyrosine kinase inhibitors

 Midostaurin

8/30 (27)

4/8 (50)

1/9 (11)

1/1 (100)

2/10 (20)

2/2 (100)

5/11 (45)

1/5 (20)

 Avapritinib

4/30 (13)

3/4 (75)

0/9 (0)

–

0/10 (0)

–

4/11 (36)

3/4 (75)

 Other TKIa

3/30 (10)

0/3 (0)

1/9 (11)

0/1 (0)

1/10 (10)

0/1 (0)

1/11 (9)

0/1 (0)

Cytoreductive therapy

        

 Cladribine

3/30 (10)

0/3 (0)

1/9 (11)

0/1 (0)

1/10 (10)

0/1 (0)

1/11 (9)

0/1 (0)

 Othersb

14/30 (47)

5/14 (36)

5/9 (56)

2/5 (40)

5/10 (50)

3/5 (60)

4/11 (36)

0/4 (0)

Donor lymphocyte infusion

18/30 (60)

5/18 (28)

7/9 (78)

1/7 (14)

7/10 (70)

3/7 (43)

4/11 (36)

1/4 (25)

Second alloHCT

2/30 (7)

2/2 (100)

0/9 (0)

–

2/10 (20)

2/2 (100)

0/11

–

Response to post-alloHCT treatmentc

Response, n (%)

13/30 (43)

3/9 (33)

5/10 (50)

5/11 (45)

No response or progression, n (%)

10/30 (33)

3/9 (33)

3/10 (30)

4/11 (36)

  1. alloHCT allogeneic stem cell transplantation, ASM aggressive systemic mastocytosis, DLI donor lymphocyte infusion, MCL ± AHN mast cell leukemia with/without an associated hematologic neoplasm, r/r relapsed/refractory, SM-AHN systemic mastocytosis with an associated hematologic neoplasm, TKI tyrosine kinase inhibitor.
  2. adasatinib, imatinib, nilotinib.
  3. bazacitidine, bortezomib, decitabine, hydroxyurea, induction chemotherapy, nivolumab, venetoclax.
  4. cOverall response independent of response in either the SM and/or the AHN compartment.
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