Treatment of POEMS syndrome with bevacizumab

Abstract

We report a case of POEMS syndrome which relapsed six years after autologous peripheral blood stem cell transplantation. According to encouraging data published recently, we treated the patient with cyclophosphamide, dexamethasone and the VEGF-antibody bevacizumab. After an initial improvement, the subsequent course was complicated by severe adverse events leading to multiorgan failure and death. This dramatic decline highlights the need for further investigation before using bevacizumab in patients with POEMS syndrome.

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

“…Two other patients using it as "salvage" improved, but relapsed and died despite continued therapy, normal VEGF at 3.5 and 5.5 years. Six other cases of use with or after other alkylator based therapy yielded one death and 4 patients with improvement [22][23][24][25][26][27]176] High-dose chemotherapy with peripheral blood stem cell transplant can also be quite effective, but selection basis may confound these reports. Case series suggest 100% of patients achieve at least some neurologic improvement [18,54,87,140,[149][150][151][152][153][154][155][156][181][182][183][184][185].…”

“…In addition, tandem transplant has been applied in one patient, but again, no information is available regarding any added value of the second transplant [157]. Anecdotally, responses are durable, but relapses have been reported [27,186]. Of the 59 patients with POEMS syndrome treated at the Mayo Clinic Rochester, progression-free survival was 98, 94, and 75% at 1, 2, and 5 years, respectively [184].…”

“…Although an anti-VEGF strategy is appealing, the results with bevacizumab have been mixed [22][23][24][25][26][27]. Five patients who had also received either radiation or alkylator during and/or predating the bevacizumab had benefit [24][25][26]187], including three who had improvement, but was then consolidated with high-dose chemotherapy with autologous stem cell transplant [25,187].…”

“…Five patients who had also received either radiation or alkylator during and/or predating the bevacizumab had benefit [24][25][26]187], including three who had improvement, but was then consolidated with high-dose chemotherapy with autologous stem cell transplant [25,187]. Three patients receiving bevacizumab died [21,22,27].…”

“…To date, VEGF is the cytokine that correlates best with disease activity [11][12][13][14][15][16][17][18][19][20], although it may not be the driving force of the disease based on the mixed results seen with anti-VEGF therapy [21][22][23][24][25][26][27][28]. VEGF is known to target endothelial cells, induce a rapid and reversible increase in vascular permeability, and be important in angiogenesis.…”