Table 1.

Clinical and biologic data

Patient 1Patient 2Patient 3
Age (y) 29 62 51 
Sex Female Female Male 
Indication of SU5416 therapy Retinal Hb Cervical Hb Cerebellar Hb  
Other VHL manifestations Quiescent cerebellar Hb Quiescent cerebellar Hb Small renal tumor 
 Renal and pancreatic cysts Renal and pancreatic cysts Renal and pancreatic cysts  
Hematocrit (%)/hemoglobin (g/L)    
 Before SU5416 therapy 39/12.3 45/14.9 50/16.6  
 After SU5416 therapy* 51/16.3 53/17.2 56/18.4 
Red blood cell mass (mL/kg) 32.2 36.4 37.7 
Variation of globular volume + 26% + 28% + 28% 
Erythropoietin (N: 5-25 mU/mL)    
 Before SU5416 therapy 2.0 2.0 10.0  
 After 12 weeks of drug 1.9 2.6 8.0  
Assessment of VHL tumors after 12 weeks of drug Stable Stable Stable  
Interruption of drug No No No  
Treatment of polycythemia Bloodletting (1 × 300 mL) Bloodletting (3 × 300 mL) Bloodletting (2 × 300 mL) 
Recurrence of polycythemia No No No 
Patient 1Patient 2Patient 3
Age (y) 29 62 51 
Sex Female Female Male 
Indication of SU5416 therapy Retinal Hb Cervical Hb Cerebellar Hb  
Other VHL manifestations Quiescent cerebellar Hb Quiescent cerebellar Hb Small renal tumor 
 Renal and pancreatic cysts Renal and pancreatic cysts Renal and pancreatic cysts  
Hematocrit (%)/hemoglobin (g/L)    
 Before SU5416 therapy 39/12.3 45/14.9 50/16.6  
 After SU5416 therapy* 51/16.3 53/17.2 56/18.4 
Red blood cell mass (mL/kg) 32.2 36.4 37.7 
Variation of globular volume + 26% + 28% + 28% 
Erythropoietin (N: 5-25 mU/mL)    
 Before SU5416 therapy 2.0 2.0 10.0  
 After 12 weeks of drug 1.9 2.6 8.0  
Assessment of VHL tumors after 12 weeks of drug Stable Stable Stable  
Interruption of drug No No No  
Treatment of polycythemia Bloodletting (1 × 300 mL) Bloodletting (3 × 300 mL) Bloodletting (2 × 300 mL) 
Recurrence of polycythemia No No No 

Hb, hemangioblastoma.

*

Maximal value of hematocrit;

after 16 weeks of drug;

after 12 weeks of drug.