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Peripheral blood stem cell transplant for POEMS syndrome is associated with high rates of engraftment syndrome.

Dispenzieri A, Lacy MQ, Hayman SR, Kumar SK, Buadi F, Dingli D, Litzow MR, Gastineau DA, Inwards DJ, Elliott MA, Micallef IN, Ansell SM, Hogan WJ, Porrata LF, Johnston PA, Afessa B, Bryce A, Kyle RA, Gertz MA - Eur. J. Haematol. (2008)

Bottom Line: Earlier and more aggressive use of corticosteroids may be associated with less complicated post-transplant courses.In addition, important clinical improvements and reductions in plasma VEGF levels can occur in the absence of significant decrease in the monoclonal protein.Unraveling the mechanisms of the syndrome both in the context of ASCT and in general are challenges for the future.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA. dispenzieri.angela@mayo.edu

ABSTRACT
Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome is a devastating syndrome, characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells, skin changes, papilledema, volume overload, sclerotic bone lesions, thrombocytosis and high vascular endothelial growth factor (VEGF). High-dose chemotherapy with autologous peripheral blood stem cell transplantation (ASCT) ultimately yields excellent clinical responses, but there can be considerable peritransplant morbidity. We have treated 30 POEMS patients with ASCT at Mayo Clinic, Rochester. During transplant period, patients had high rates of fever, diarrhea, weight gain and rash (93%, 77%, 53% and 43%, respectively). Only 13% remained outpatient, and median time to discharge from hospital was transplant day 17 (range 0-175). Splenomegaly was the baseline factor that best predicted for a complicated peritransplant course. Depending on the definition used, approximately 50% of patients satisfied criteria for engraftment syndrome. Earlier and more aggressive use of corticosteroids may be associated with less complicated post-transplant courses. Median overall survival has not been reached; the treatment-related mortality was 3%. In addition, important clinical improvements and reductions in plasma VEGF levels can occur in the absence of significant decrease in the monoclonal protein. Unraveling the mechanisms of the syndrome both in the context of ASCT and in general are challenges for the future.

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Engraftment syndrome (ES) radiograph findings. (A) Day +8 PBSCT demonstrating relatively normal CXR despite fever and diarrhea. (B) Day +11 PBSCT (same patient) demonstrating increased heart size, diffuse interstitial infiltrates throughout both lungs with alveolar infiltrates in the lower lungs.
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fig02: Engraftment syndrome (ES) radiograph findings. (A) Day +8 PBSCT demonstrating relatively normal CXR despite fever and diarrhea. (B) Day +11 PBSCT (same patient) demonstrating increased heart size, diffuse interstitial infiltrates throughout both lungs with alveolar infiltrates in the lower lungs.

Mentions: Prospectively, rash was attributed to drugs, with a resultant change in antibiotic regimen from cefepime to meropenem. Sixty-three percent of patients had infiltrates and/or effusions on CXR at a median of day +12. Of the 14 patients who initially had a normal CXR with their first fever, six patients went on to have a second CXR that was abnormal – most commonly with infiltrates, effusions or both. The findings may be subtle or dramatic (Fig. 2).


Peripheral blood stem cell transplant for POEMS syndrome is associated with high rates of engraftment syndrome.

Dispenzieri A, Lacy MQ, Hayman SR, Kumar SK, Buadi F, Dingli D, Litzow MR, Gastineau DA, Inwards DJ, Elliott MA, Micallef IN, Ansell SM, Hogan WJ, Porrata LF, Johnston PA, Afessa B, Bryce A, Kyle RA, Gertz MA - Eur. J. Haematol. (2008)

Engraftment syndrome (ES) radiograph findings. (A) Day +8 PBSCT demonstrating relatively normal CXR despite fever and diarrhea. (B) Day +11 PBSCT (same patient) demonstrating increased heart size, diffuse interstitial infiltrates throughout both lungs with alveolar infiltrates in the lower lungs.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2327207&req=5

fig02: Engraftment syndrome (ES) radiograph findings. (A) Day +8 PBSCT demonstrating relatively normal CXR despite fever and diarrhea. (B) Day +11 PBSCT (same patient) demonstrating increased heart size, diffuse interstitial infiltrates throughout both lungs with alveolar infiltrates in the lower lungs.
Mentions: Prospectively, rash was attributed to drugs, with a resultant change in antibiotic regimen from cefepime to meropenem. Sixty-three percent of patients had infiltrates and/or effusions on CXR at a median of day +12. Of the 14 patients who initially had a normal CXR with their first fever, six patients went on to have a second CXR that was abnormal – most commonly with infiltrates, effusions or both. The findings may be subtle or dramatic (Fig. 2).

Bottom Line: Earlier and more aggressive use of corticosteroids may be associated with less complicated post-transplant courses.In addition, important clinical improvements and reductions in plasma VEGF levels can occur in the absence of significant decrease in the monoclonal protein.Unraveling the mechanisms of the syndrome both in the context of ASCT and in general are challenges for the future.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA. dispenzieri.angela@mayo.edu

ABSTRACT
Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome is a devastating syndrome, characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells, skin changes, papilledema, volume overload, sclerotic bone lesions, thrombocytosis and high vascular endothelial growth factor (VEGF). High-dose chemotherapy with autologous peripheral blood stem cell transplantation (ASCT) ultimately yields excellent clinical responses, but there can be considerable peritransplant morbidity. We have treated 30 POEMS patients with ASCT at Mayo Clinic, Rochester. During transplant period, patients had high rates of fever, diarrhea, weight gain and rash (93%, 77%, 53% and 43%, respectively). Only 13% remained outpatient, and median time to discharge from hospital was transplant day 17 (range 0-175). Splenomegaly was the baseline factor that best predicted for a complicated peritransplant course. Depending on the definition used, approximately 50% of patients satisfied criteria for engraftment syndrome. Earlier and more aggressive use of corticosteroids may be associated with less complicated post-transplant courses. Median overall survival has not been reached; the treatment-related mortality was 3%. In addition, important clinical improvements and reductions in plasma VEGF levels can occur in the absence of significant decrease in the monoclonal protein. Unraveling the mechanisms of the syndrome both in the context of ASCT and in general are challenges for the future.

Show MeSH
Related in: MedlinePlus