title: Predictive value of RT-PCR PML-RARA transcript monitoring for extramedullary relapse of acute promyelocytic leukemia in the pleura, heart and pericardium after allogeneic SCT.
abstract: BACKGROUND
  We report a patient with acute promyelocytic leukemia (APL) relapse in extremely rare sites--the pleura, heart and pericardium without evidence of bone marrow infiltration and with molecular evidence of disease after allogeneic stem cell transplantation (alloSCT).
  
  
  METHODS
  Presented patient underwent alloSCT in second complete hematological and cytogenetic remission with presence of promyelocytic leukemia-retinoic acid receptor A (PML-RARA) detected in reverse transcription-polymerase chain reaction (RT-PCR) with sensitivity of 10(-2). After transplant, this patient remained in complete hematological and cytogenetic remission but nested RT-PCR assays with detection thresholds of 10(-3)/10(-4) were positive for PML-RARA rearranged gene even chimerism tests showed 100% of donor profile. He was in a very good clinical condition and presented symptoms of transient limited chronic graft vs. host disease. Twenty one months after transplant, the leukemic relapse in the pleura, heart and pericardium was diagnosed. At that time, PML-RARA transcript detected in RT-PCR assay (10(-2)) was positive for the first time after transplant. During salvage chemotherapy he died because of cardiogenic shock.
  
  
  CONCLUSIONS
  We conclude that detection of PML-RARA after alloSCT should be indication insightful diagnosis of medullary or extramedullary (EM) relapse. The imaging techniques of all possible sites of APL EM relapse have to be included.
authors.affiliation: Bone Marrow Transplantation Unit, Institute of Hematology and Blood Transfusion, Warsaw, Poland. barbaramail@poczta.onet.pl
authors.forename: Barbara
authors.initials: B
authors.lastname: Nasilowska-Adamska
authors.forename: Mirosław
authors.initials: M
authors.lastname: Majewski
authors.forename: Ilona
authors.initials: I
authors.lastname: Seferynska
authors.forename: Andrzej
authors.initials: A
authors.lastname: Szczepinski
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Tomaszewska
authors.forename: Monika
authors.initials: M
authors.lastname: Prochorec-Sobieszek
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Guz
authors.forename: Adam
authors.initials: A
authors.lastname: Torbicki
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Warzocha
authors.forename: Bozena
authors.initials: B
authors.lastname: Marianska
mesh_terms: D000328:Adult; D006338:Heart Neoplasms / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D010496:Pericardium; D010997:Pleural Neoplasms / Q000175:diagnosis* / Q000235:genetics; D011237:Predictive Value of Tests; D020133:Reverse Transcriptase Polymerase Chain Reaction; D023981:Sarcoma, Myeloid / Q000175:diagnosis* / Q000235:genetics; D033581:Stem Cell Transplantation
chemical_list: D015514:Oncogene Proteins, Fusion; C093476:promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein