Table 5

Activity of VEGF inhibition for the treatment of POEMS syndrome

TreatmentOutcome
Bev alone21  Death within 6 wks 
Bev + mycophenolate + Dex97  One mo after starting therapy, patient deteriorated further with worsening ascites and shortness of breath; Bev and Dex were discontinued; Mel and Pred were begun; patient died 1 mo later 
Bev alone22  Worsening PN, anasarca, multiorgan failure; died of pneumonia 5 wks after therapy 
Bev alone23  Improved pain, breathing, and walking 
Bev + Mel-Dex24  Improved effusions/ascites 
Prior VAD/CTX25  Improved edema, pain, weakness, and VEGF 
Bev + CTX-Dex26  Initial worsening; repeat with Bev → improved pulmonary HTN, anasarca, skin changes 
Bev + CTX-CS27  Initial improvement, but multiorgan failure and death 
Bev + CTX radiation29  Two patients: (1) first patient treated with radiation and CTX and then Bev; clinical improvement started before Bev; at radiologic relapse, Bev no use, so Len plus Dex used with benefit; and (2) second patient treated with same sequence, but course complicated by sepsis; biochemical and early neurologic response before Bev started 
Bev + CTX28  Clinical and biochemical relapse; no response to CTX, so bevacizumab added; death 
TreatmentOutcome
Bev alone21  Death within 6 wks 
Bev + mycophenolate + Dex97  One mo after starting therapy, patient deteriorated further with worsening ascites and shortness of breath; Bev and Dex were discontinued; Mel and Pred were begun; patient died 1 mo later 
Bev alone22  Worsening PN, anasarca, multiorgan failure; died of pneumonia 5 wks after therapy 
Bev alone23  Improved pain, breathing, and walking 
Bev + Mel-Dex24  Improved effusions/ascites 
Prior VAD/CTX25  Improved edema, pain, weakness, and VEGF 
Bev + CTX-Dex26  Initial worsening; repeat with Bev → improved pulmonary HTN, anasarca, skin changes 
Bev + CTX-CS27  Initial improvement, but multiorgan failure and death 
Bev + CTX radiation29  Two patients: (1) first patient treated with radiation and CTX and then Bev; clinical improvement started before Bev; at radiologic relapse, Bev no use, so Len plus Dex used with benefit; and (2) second patient treated with same sequence, but course complicated by sepsis; biochemical and early neurologic response before Bev started 
Bev + CTX28  Clinical and biochemical relapse; no response to CTX, so bevacizumab added; death 

Bev indicates bevacizumab; Dex, dexamethasone; Mel, mephalan; Pred, prednisone; PN, polyneuropathy; VAD, vincristine, doxorubicin, dexamethasone; CTX, cyclophosphamide; HTN, hypertension; CS, corticosteroids; and Len, lenalidomide.

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