Table 1.

Clinical and hematologic features of patients investigated











Size of PNH-cell population*




Patients with PNH
Sex/age, y
Time since diagnosis, y
Hb, g/L
WBC, x10–9/L
ANC, x10–9/L
Lymph, x10–9/L
Platelets, x10–9/L
Retics, %
RBCs, %
PMN, %
Hemoglobinuria
Thrombosis
Blood transfusion
Other treatment
1   M/53   1   102   4.4   3.2   0.9   249   6.0   21   81   Yes   No   Occasional   Supportive  
2   M/45   2   94   2.9   1.6   1.1   97   4.8   20   55   Yes   No   Never   Supportive  
3   M/34   2   101   4.7   2.5   1.7   178   10.2   50   80   Yes   No   Occasional   Supportive 
4   F/54   10   81   3.3   2.4   0.7   57   7.1   36   98   Yes   Hepato-portal   Never   Supportive  
5   F/54   10   77   3.8   1.8   1.5   193   6.4   30   93   Yes   No  Never   Supportive  
6   M/33   5   123   2.1   0.9   0.9   87   2.2   22   69   Yes   No   Never   Supportive  
7   F/25   8   67   2.5   1.1   1.2   83   14.5   23   82   Yes   No   Regular   Supportive§ 
8   F/53   7   121   3.6   1.6   1.7   64   2.1   51   96   Yes   Budd-Chiari   Occasional   Supportive  
9   F/25   1   109   4.5   2.4   1.8   323   9.8   26   80   Yes   No  Never   Supportive  
10   F/45   5   86   8.7   7.5   1.0   143   17.0   75   99   Yes   No   Occasional   Supportive  
11   M/33   5   101   5.6   3.3   1.8   262   6.8   37   97   Yes   No   Occasional   Supportive§ 
12   F/55   23   84   3.9   2.5   1.0   185   10.1   54   90   Yes   No   Regular   Supportive  
13
 
F/32
 
3
 
78
 
4.0
 
2.2
 
1.3
 
193
 
2.4
 
91
 
69
 
Yes
 
No
 
Occasional
 
Supportive§
 










Size of PNH-cell population*




Patients with PNH
Sex/age, y
Time since diagnosis, y
Hb, g/L
WBC, x10–9/L
ANC, x10–9/L
Lymph, x10–9/L
Platelets, x10–9/L
Retics, %
RBCs, %
PMN, %
Hemoglobinuria
Thrombosis
Blood transfusion
Other treatment
1   M/53   1   102   4.4   3.2   0.9   249   6.0   21   81   Yes   No   Occasional   Supportive  
2   M/45   2   94   2.9   1.6   1.1   97   4.8   20   55   Yes   No   Never   Supportive  
3   M/34   2   101   4.7   2.5   1.7   178   10.2   50   80   Yes   No   Occasional   Supportive 
4   F/54   10   81   3.3   2.4   0.7   57   7.1   36   98   Yes   Hepato-portal   Never   Supportive  
5   F/54   10   77   3.8   1.8   1.5   193   6.4   30   93   Yes   No  Never   Supportive  
6   M/33   5   123   2.1   0.9   0.9   87   2.2   22   69   Yes   No   Never   Supportive  
7   F/25   8   67   2.5   1.1   1.2   83   14.5   23   82   Yes   No   Regular   Supportive§ 
8   F/53   7   121   3.6   1.6   1.7   64   2.1   51   96   Yes   Budd-Chiari   Occasional   Supportive  
9   F/25   1   109   4.5   2.4   1.8   323   9.8   26   80   Yes   No  Never   Supportive  
10   F/45   5   86   8.7   7.5   1.0   143   17.0   75   99   Yes   No   Occasional   Supportive  
11   M/33   5   101   5.6   3.3   1.8   262   6.8   37   97   Yes   No   Occasional   Supportive§ 
12   F/55   23   84   3.9   2.5   1.0   185   10.1   54   90   Yes   No   Regular   Supportive  
13
 
F/32
 
3
 
78
 
4.0
 
2.2
 
1.3
 
193
 
2.4
 
91
 
69
 
Yes
 
No
 
Occasional
 
Supportive§
 

Hb indicates hemoglobin; PMN, granulocytes; Lymph, lymphocytes; Retics, reticulocytes; RBCs, red blood cells; Yes, recurrent episodes of macroscopic hemoglobinuria throughout several years of clinical history.

*

PMN reflects the relative size of the PNH-cell population more accurately than the percentage of PNH RBCs, because the latter will be grossly underestimated as a consequence of selective hemolysis; this may be further complicated by blood transfusion

Previous prednisone treatment

Recurrent attacks of severe abdominal pain but no documented thrombosis

§

Previous antithymocyte globulin treatment

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