The "Retinoic Acid Syndrome" in Acute Promyelocytic Leukemia

Introducing biological features at diagnosis improves the relapse risk stratification in patients with acute promyelocytic leukemia treated with ATRA and chemotherapy

“…This concern is reflected by the fact that although bone marrow involvement according to BMB has been established as an important adverse prognostic factor in DLBCL [7], this is not the case for FDG-PET/CT-based bone marrow involvement. Of the five studies that reported the prognostic value of bone marrow involvement at FDG-PET/CT in DLBCL [2,[8][9][10][11], four showed that FDG-PET/CTbased bone marrow status did not have any prognostic value at all [2,[8][9][10]. These observations suggest that false-positive FDG-PET/CT results occur in a considerable proportion of patients with DLBCL and that increased FDG uptake of the marrow is not sufficient evidence to designate advanced-stage disease.…”

“…Molecular relapse was defined as a positive PML/RARA test detected in two successive marrow samples collected at any time after consolidation therapy by RT-PCR and in the absence of morphologically detectable blasts in both marrow and peripheral blood [10]. Differentiation syndrome (DS) was defined as definitely present when at least three major signs were recorded according to the criteria of Frankel [11]. Screening for FLT3-ITD mutations was carried out as previously reported [12].…”

How to manage acute promyelocytic leukemia

“…53,78 The common clinical symptoms were dyspnea with interstitial pulmonary infiltrates, peripheral edema, unexplained fever, weight gain, hypotension and acute renal failure. 79 When these happen, early use of chemotherapy and high dose of dexamethasone can decrease the mortality down to 1% or lower. 42 It has been described that prophylactic treatment of APL DS by prednisolone can reduce the incidence of DS significantly, 80 but it is risky to use the steroids routinely as prophylaxis.…”

True

“…ATRA syndrome is histologically characterized by the extensive organ infiltration of myeloid cells, 1 and the histological findings of this case was different from those of ATRA syndrome. We therefore concluded that the patient had vasculitis, and the vasculitis was induced by ATRA, because no other findings to explain the occurrence of vasculitis were observed.…”