Table 2.

Sites of thrombosis in 29 out of 163 cases of PNH


UPN

Site of thrombosis

Years after diagnosis

Granulocyte PNH clone size, %

Red cell PNH clone size, %*
  Liver-related     
5   Portal vein (probable cause of death)   5   Not known   64  
6   Hepatic vein   4   99.4   33.4  
8   Hepatic vein   0   97.96   30.88  
10   Portal vein + sagittal sinus (pregnant)   15   Not known   52  
17   Hepatic + portal veins   0   Not known   100  
27   Portal vein   8   99.6   89.59  
33   Hepatic vein, shunt thrombosis   14   97.8   64  
40   Portal vein   5   97   57  
53   `Liver' (cause of death)   4   78   23  
80   Portal vein (cause of death)   0   98   15.1  
95   Hepatic vein—2 events   0   80   22.99  
98   Hepatic vein   Not known   68.1   67.66  
104   Hepatic vein   Not known  Not known  Not known 
107   Hepatic vein (cause of death)   0   99.5   49.81  
109   Portal vein   0   99.9   5.73  
149   Hepatic vein   0   98.3   83.4  
153   Portal vein, pulmonary embolus   0.25   96.3   67.8  
165   Hepatic vein—2 events   2.25   98.9   21.1  
  Other     
3   Deep venous thrombosis—2 events   7   9.7   4.4  
22   Pulmonary embolus (Factor V Leiden heterozygote)   2   90.82   20.9  
26   Superficial thrombophlebitis   1   55   24  
34   Deep venous thrombosis   5   73   45  
48   Cerebral vein thrombosis   8.75   92.9   77.4  
61   Line thrombosis   1   67.62   21  
70   Retinal vein   4   76.04   11.66  
119   Thrombosis right cerebellum (Factor V Leiden heterozygote)   0   15.3   7.21  
144   Deep venous thrombosis   0   96.6   43.2  
146   Pulmonary embolus   0.8   84   57.4  
177
 
Pulmonary embolus
 
7
 
97.1
 
81.5
 

UPN

Site of thrombosis

Years after diagnosis

Granulocyte PNH clone size, %

Red cell PNH clone size, %*
  Liver-related     
5   Portal vein (probable cause of death)   5   Not known   64  
6   Hepatic vein   4   99.4   33.4  
8   Hepatic vein   0   97.96   30.88  
10   Portal vein + sagittal sinus (pregnant)   15   Not known   52  
17   Hepatic + portal veins   0   Not known   100  
27   Portal vein   8   99.6   89.59  
33   Hepatic vein, shunt thrombosis   14   97.8   64  
40   Portal vein   5   97   57  
53   `Liver' (cause of death)   4   78   23  
80   Portal vein (cause of death)   0   98   15.1  
95   Hepatic vein—2 events   0   80   22.99  
98   Hepatic vein   Not known   68.1   67.66  
104   Hepatic vein   Not known  Not known  Not known 
107   Hepatic vein (cause of death)   0   99.5   49.81  
109   Portal vein   0   99.9   5.73  
149   Hepatic vein   0   98.3   83.4  
153   Portal vein, pulmonary embolus   0.25   96.3   67.8  
165   Hepatic vein—2 events   2.25   98.9   21.1  
  Other     
3   Deep venous thrombosis—2 events   7   9.7   4.4  
22   Pulmonary embolus (Factor V Leiden heterozygote)   2   90.82   20.9  
26   Superficial thrombophlebitis   1   55   24  
34   Deep venous thrombosis   5   73   45  
48   Cerebral vein thrombosis   8.75   92.9   77.4  
61   Line thrombosis   1   67.62   21  
70   Retinal vein   4   76.04   11.66  
119   Thrombosis right cerebellum (Factor V Leiden heterozygote)   0   15.3   7.21  
144   Deep venous thrombosis   0   96.6   43.2  
146   Pulmonary embolus   0.8   84   57.4  
177
 
Pulmonary embolus
 
7
 
97.1
 
81.5
 
*

Includes both type II (partial deficiency) and type III (complete deficiency) cells.

Diagnosed in the 1960s.

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