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Table 5 Outcomes of patients with a TMA/aHUS score ≥ 5 (N = 185)

From: Application of a scoring system in Japanese patients diagnosed with atypical hemolytic uremic syndrome to assess the relationship between the score and clinical responses to eculizumab

Item

Survivors

n (%)

Non-survivors

n (%)

p

N

148 (80)

37 (20)

 

TMA score items

 Hb < 10.0 g/dL*

145 (98.0)

36 (97.3)

1.000

 PLT < 150 × 109/L

146 (98.6)

37 (100.0)

1.000

 Renal failure

137 (92.6)

32 (86.5)

0.322

 Extrarenal organ failure

92 (62.2)

30 (81.1)

0.033

Positive aHUS score items

Past history of TMA

15 (10.1)

4 (10.8)

1.000

 1 time

15 (10.1)

4 (10.8)

 

 ≥ 2 times

0 (0.0)

0 (0.0)

 

Family history of TMA, n/N reported (%)

9/141 (6.1)

1/35 (2.7)

0.689

 1 family member

7/141 (4.7)

1/35 (2.7)

 

 ≥ 2 family members

2/141 (1.4)

0/35 (0.0)

 

Age at onset of TMA

 < 10 years

37 (25.0)

12 (32.4)

0.673

 ≥ 10 to < 63 years

77 (52.0)

18 (48.6)

 

 ≥ 63 years

34 (23.0)

7 (18.9)

 

Trigger

36 (24.3)

10 (27.0)

0.831

Exclusion aHUS score items

Bloody stool

17 (11.5)

6 (16.2)

0.414

ADAMTS13 < 10%

2 (1.4)

0 (0.0)

1.000

Underlying diseases

15 (10.1)

15 (40.5)

 < 0.001

 Malignant tumor

11 (7.4)

11 (29.7)

 

 HSCT

11 (7.4)

10 (27.0)

 

 Chemotherapy

1 (0.7)

4 (10.8)

 

 Acute pancreatitis

0 (0.0)

0 (0.0)

 

D-dimer > 20 μg/mL

0 (0.0)

0 (0.0)

-

WBC > 16,000/mL

1 (0.7)

1 (2.7)

0.360

Total score, median (range)

10 (3–16)

9 (4–13)

0.055

  1. *Description used in post-marketing surveillance: microangiopathic hemolytic anemia (defined from following items: Hb < 10 g/dL, LDH increase, haptoglobin decrease, presence of schistocyte)
  2. Wilcoxon’s rank sum test and Fisher’s test were used, as appropriate
  3. Abbreviations: aHUS atypical hemolytic uremic syndrome, Hb hemoglobin, PLT platelet count, sCr serum creatinine, TMA thrombotic microangiopathy, ULN upper limit of normal, WBC white blood cell count