Engraftment Syndrome Is Common in Patients with POEMS Syndrome Undergoing PBSCT.

Background: POEMS syndrome is a devastating syndrome, characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells, skin changes, papilledema, volume overload, sclerotic bone lesions, thrombocytosis, & high VEGF. We noted an unexpectedly high transplant related morbidity, which we have since postulated to be ES. Methods: 30 patients with POEMS were treated with PBSCT at Mayo Clinic Rochester. We retrospectively studied outcomes, with an emphasis on treatment related morbidity. Two definitions of ES were used: Spitzer (BMT 2001) and Maiolino (BMT 2003). Results: Two-thirds were male. Median age was 48, range 20–70. Time from first symptoms and diagnosis was 26 and 4 months, respectively. To mobilize stem cells, CTX/G-CSF was used in 5 & G-CSF in the remainder. Conditioning was Mel200 (n=19), Mel140 (n=10), and BEAM (n=1). Post-transplant 15 had GM-CSF begun day+6. Only 10% remained outpatient, and median time to discharge from hospital was transplant day 17 (range 0–175). Factors predicting for later dismissal included age (p=0.04), abnormal CXR 7 to 17 days post transplant (p<0.0001), & bolus corticosteroids (CS) beyond day 12 post-transplant (p=0.006). Ninety-three percent had fever, although only 9 had bacteremia. Eight satisfied criteria for ES according to Spitzer and 14 according to Maiolino. Another 3 patients received steroid bolus days 8, 12, and 11 for presumed ES but did not meet criteria because of delayed ANC recovery (days 16, 18, and 20, respectively). Of the 5 patients requiring endotracheal intubation, 3 satisfied Maiolino’s criteria for ES. Although these 3 received bolus CS during their course, administration was delayed at 18, 14, and 18 days. The patient whose CS bolus antedated intubation only received prednisone 30 mg/day. In toto, fourteen patients received bolus CS (daily doses between 20 and 1200 mg prednisone equivalents) commencing day 8 to 59. Those 7 patients who received CS before day 13 did better than the 7 who received them day 13 or later (Table). Conclusions: It is essential to recognize that nearly 50% of patients satisfied formal criteria for ES as defined by Maiolino. In these patients ES may run a self-limited course or lead to catastrophe. | | No Steroid (n=16) | Steroid ≤ D12 (n=7) | Steroid > D12 (n=7) | P | |:-------------------------------------------------------------------------------- | ----------------- | ------------------- | ------------------- | ------ | | *Engraftment syndrome according to definition of Maiolino (M) or of Spitzer (S). | | ES M / S, n* | 5 / 2 | 4 / 4 | 5 / 2 | NA | | Wt change, % | 0.6 (−.4.2–6.7) | 6.7 (3.6–27.2) | 11.2 (–2.1–23.2) | 0.005 | | Rash, % | 27 | 71 | 43 | 0.13 | | Diarrhea, % | 73 | 86 | 86 | 0.71 | | Tmax, C | 39 (37.8–41) | 40.1 (39–41.1) | 38.9 (38.7–40.8) | 0.08 | | 1st fever, day | 10 (6–15) | 8 (7–9) | 12 (8–146) | 0.007 | | Abn CXR1, % | 13 | 71 | 71 | 0.03 | | Ventilator, % | | 14 | 71 | 0.004 | | First WBC, day | 12 (8–21) | 14 (12–14) | 14 (12–17) | 0.03 | | ANC500, day | 15 (12–29) | 16 (14–115) | 18 (15–45) | 0.08 | | PLT20, day | 12 (8–41) | 20 (11–115) | 24 (9–170) | 0.05 | | PLT50, day | 15 (11–192) | 32 (16–115) | 56 (13–551) | 0.03 | | RBCs, units | 3 (2–8) | 6 (4–31) | 11 (6–64) | 0.0008 | | PLTS, aph. units | 2 (1–9) | 9 (4–51) | 18 (4–60) | 0.0004 | | Hosp dsm, day | 15 (13–36) | 21 (15–69) | 41 (16–175) | 0.009 |