Table 1 Patient characteristics in different AdvSM subtypes.

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 (%)

71 (100)

30 (42)

28 (39)

13 (18)

Age in years at Dx; median (range)

58 (21–84)

57 (27–67)

59 (21–84)

51 (27–74)

Age in years at alloHCT; median (range)

59 (21–84)

59 (31–68)

60 (22–84)

55 (28–75)

Male, n (%)

49/71 (69)

21/30 (70)

19/28 (68)

9/13 (68)

Disease characteristics before alloHCT

 Karnofsky performance score ≥90%, n (%)

37/63 (59)

18/27 (67)

12/25 (48)

7/11 (64)

 Serum tryptase ≥125 µg/L, n (%)

18/44 (41)

6/16 (38)

6/16 (38)

6/12 (50)

 Bone marrow mast cell infiltration ≥20%, n (%)

27/40 (68)

10/17 (59)

7/15 (47)

11/12 (92)

 KIT D816V positivity, n (%)

52/62 (84)

22/24 (92)

23/25 (92)

7/13 (54)

 SRSF2/ASXL1/RUNX1 positivity, n (%)

25/48 (52)

12/19 (63)

11/19 (58)

2/10 (20)

 Cytogenetics, aberrant, n (%)

23/62 (37)

5/23 (22)

15/27 (56)

3/12 (25)

 Cytogenetics, complex aberrant, n (%)

9/62 (15)

2/23 (9)

4/27 (15)

3/12 (25)

Disease course before alloHCT

 Lines of therapies; median (range)

2 (0–5)

1 (0–4)

2 (0–5)

3.5 (1–5)

 Involvement of tyrosine kinase inhibitors, n (%)

35/71 (49)

14/30 (47)

9/28 (32)

12/13 (92)

 Involvement of midostaurin/avapritinib, n (%)

27/71 (38)

10/30 (33)

5/27 (19)

11/13 (85)

 Years to alloHCT since AdvSM Dx; median (range)

1.2 (0.1–16.7)

1.5 (0.3.16.7)

1.0 (0.0–12.7)

1.2 (0.2–12.7)

 Years to alloHCT since AHN/AML Dx; median (range)

1.1 (0.1–16.7)

1.6 (0.2-16.7)

0.3 (0.1–3.1)

1.0 (0.2–12.7)

  1. AHN associated hematologic neoplasm; alloHCT allogeneic hematopoietic stem-cell transplantation, ASM aggressive systemic mastocytosis, Dx diagnosis, n number, SM-AHN systemic mastocytosis with an associated hematologic neoplasm, SM-AML systemic mastocytosis with an acute myeloid leukemia, MCL±AHN mast cell leukemia with/without an associated hematologic neoplasm.