Allogeneic stem cell transplantation for blastic plasmacytoid dendritic cell neoplasm: single-centre experience of 64257; ve cases

Ref ID: 18601

Author:

F. Michelis, A. Balta, M. Vagia, I. Grigoriou, I. Baltadakis,
D. Karakasis, J. Apostolidis, N. Harhalakis, E. Nikiforakis

Author address:

Evangelismos Hospital (Athens, GR)

Full conference title:

Annual Meeting of the EBMT, 36th

Abstract:

Background: Blastic plasmacytoid dendritic cell neoplasm
(BPDCN), formerly known as blastic NK cell lymphoma, is a
rare CD4 + CD56 + hematopoietic malignancy usually presenting with cutaneous lesions associated with or without a leukemic component. The disease irrespective of the initial pattern
has an aggressive clinical course, demonstrating only a brief
response to acute leukemia (AL)-type chemotherapy. Long
term survival has been documented in sporadic cases following
allogeneic stem cell transplantation (alloSCT) and represents
the only treatment with the potential for cure.
Aim: To present the outcome of fi ve patients with BPDCN who
underwent alloSCT with a myeloablative conditioning regimen.
Patients: Five patients (M = 3/F = 2), with a median age at the
time of transplantation of 27 years (range 15-33 years). Initial presentation of disease involved skin and bone marrow in
three patients, isolated cutaneous nodules in one patient, and
peripheral lymph nodes and bone marrow in one patient. Four
patients were treated with an AL-type induction regimen, while
the patient with only primary cutaneous disease was initially
treated with involved radiation therapy + CHOP followed by ALtype therapy at relapse.
Results: Median time from diagnosis to transplantation was
7 months (range, 5-21). Three patients underwent alloSCT in
CR1, one in CR2 and one in fi rst chemosensitive relapse. All S401
patients received a myeloablative conditioning regimen (4 = TBI/
Cy, 1 = BU/Cy), followed by infusion of PBSCs from a matched
unrelated donor. Four patients developed grade II aGvHD. Two
patients died of treatment related causes (graft failure/aspergillosis at 6 months, cGvHD/multi-organ failure and sepsis at 16
months) with disease in remission. Two patients died of disease relapse at 5 and 21 months. One patient survives at + 41
months in remission without GvHD.
Discussion: BPDCN is an aggressive malignancy with poor
long-term response to conventional chemotherapy alone.
Although alloSCT seems to offer the only prospect of long-term
survival and cure, our limited experience demonstrates that
transplant-related mortality and disease relapse remain signifi –
cant adverse factors for outcome.

Abstract Number: R1291

Conference Year: 2010

Link to conference website: NULL

New link: NULL

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