Table 2 Clinical characteristics of patients divided according to PNH clone size on granulocytes at baseline.

From: Clinical and prognostic significance of small paroxysmal nocturnal hemoglobinuria clones in myelodysplastic syndrome and aplastic anemia

Clone size

Neg N = 1740

0.01–1% N = 215

1–10% N = 108

10–50% N = 59

>50% N = 128

Males, N (%)

945 (54)

114 (53)

60 (56)

30 (51)

56 (44)

Females, N (%)

795 (46)

101 (47)

48 (44)

29 (49)

72 (56)

Median age, years (IQR)

55 (39–66)

49 (34–63)

48 (30–65)

48 (28–60)

44 (31–85)

MDS, N (%)

616 (35)

71 (33)

32 (30)

13 (22)

7 (6)*

AA, N (%)

175 (10)

98 (46)

72 (67)

40 (68)

47 (37)

MDS/AA, N (%)

92 (5)

8 (4)

2 (2)

0 (0)

0 (0)

Acute leukemia, N (%)

157 (9)

11 (5)*

0 (0)

1 (2)

0 (0)

Hemolytic PNH, N (%)

0 (0)

1 (0.5)a

1 (0.5)a

3 (5)

74 (58)*

MPN, N (%)

0 (0)

8 (4)

0 (0)

0 (0)

0 (0)

MDS/MPN, N (%)

0 (0)

4 (2)

0 (0)

0 (0)

0 (0)

Isolated cytopenia, N (%)

433 (25)

19 (9)

1 (1)

1 (2)

0 (0)

Isolated thrombosis, N (%)

245 (14)

6 (3)*

1 (1)

0 (0)

0 (0)

Others, N (%)

22 (2)

0 (0)

0 (0)

0 (0)

0 (0)

 

N = 1740

N = 215

N = 108

N = 59

N = 128

Treated, N (%)

425 (56)

130 (68)

81 (78)

46 (78)

114 (89)*

Thrombosis, N (%)

315 (18)

16 (7)

6 (6)

3 (5)

27 (21)*

Death, N (%)

536 (31)

33 (15)

12 (11)

12 (20)

9(7)*

 

N = 771

N = 215

N = 108

N = 59

N = 128

Hb < 100 g/L, N (%)

299 (39)

102 (47)

58 (54)

30 (51)

67 (52)

PLT < 10 × 109/L, N (%)

351 (45)

133 (62)

88 (82)

45 (76)*

43 (34)

ANC < 1.5 × 109/L, N (%)

220 (29)

65 (30)

57 (53)

29 (49)

17 (13)

Median LDH U/L (range)

197 (73–1520)

222 (83–1200)

198 (108–1158)

266 (70–984)

880 (97–4403)*

  1. Values are shown only for patients with evaluable granulocyte PNH clone size at baseline by FLAER and with available clinical and laboratory data (Negative, N = 1740, positive, N = 510).
  2. MDS myelodysplastic syndromes, AA aplastic anemia, MDS/AA hypoplastic MDS, MPN myeloproliferative neoplasms, Hb hemoglobin, PLT platelets, ANC absolute neutrophil counts.
  3. *p < 0.0005.
  4. aTwo patients showed small and very small clones at baseline and then developed classic hemolytic PNH, with 15% and 20% clone size, respectively.
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