All-trans-retinoic acid (ATRA) is typically used as a first line agent in the treatment of acute promyelocytic leukaemia (APL), achieving complete remission (CR) rates (incombination with chemotherapy) of about 90%. One of the drawbacks of the use of ATRA is that up to 30% of patients can present with retinoic acid syndrome (RAS), which can be fatal in some patients. We describe a case of RAS after only one dose of ATRA, which to our knowledge has not previously been identified in the literature. The pathophysiology and treatment of APL is presented. A clinical description of RAS is outlined, and an evaluation of risk factors for developing RAS is reviewed.
Stone RM, Mayer RJ.The unique aspects of acute promyelocytic leukemia. J Clin Oncol1990; 8: 1913-1921.
3.
Warrell RP, De The H, Wang ZY, Degops L.Acute promyelocytic leukemia. N Engl J Med1993; 329: 177-189.
4.
Grignani F, Fagioli M, Alcalay M, Longo L, Pandolfi PP, Donti E, Biondi A, Lo Coco F, Grignani F, Pelicci PG.Acute promyelocytic leukemia: from genetics to treatment. Blood1994; 83: 10-25.
Evans G, Grimwade D, Prentice HG, Simpson N.Central nervous system relapse in acute promyelocytic leukemia in patients treated with all-trans retinoic acid. Br J Haematol1997; 98: 437-439.
8.
Tallman MS, Kwaan HC.Reassessing the hemostatic disorder associated with acute promyelocytic leukemia. Blood1992; 79: 543-553.
9.
Chen Z, Brand NJ, Chen A, Chen SJ, Tong JH, Wang ZY, Waxman S, Zelent A.Fusion between a novel Kruppel-like zinc finger gene and the retinoic acid receptor a locus due to a variant t(11;17) translocation associated with acute promyelocytic leukemia. EMBO J1993; 12: 1161-1167.
10.
Melnick A, Licht JD.Deconstructing a disease: RARa, its fusion partners and their roles in the pathogenesis of acute promyelocytic leukemia. Blood1999; 93: 3167-3215.
11.
Chen SJ, Zelent A, Tong JH, Yu HQ, Wang ZY, Derre J, Berger R, Waxman S, Chen Z.Rearrangement of the retinoic acid receptor alpha and PLFZ genes resulting from t(11;17) (q23;q21) in a patient with acute promyelocytic leukemia. J Clin Invest1993; 91: 2260-2267.
12.
Huang M, Ye Y, Chen S, Chai JR, Lu JX, Zhoa L, Gu LJ, Wang ZY.Use of all-trans retinoic acid in the treatment of acute promyelocytic leukemia. Blood1988; 72: 567-572.
13.
Warrell R, Frankel SR, Miller WH, Scheinberg DA, Itri LM, Hittelman WN, Vyas R, Andreeff M, Tafuri A, Jakubowski A, et al.Differentiation therapy of acute promyelocytic leukemia with tretinoin (all-trans-retinoic acid). N Engl J Med1991; 324: 1385-1393.
14.
Castaigne S, Chomienne C, Daniel MT, Berger R, Fenaux P, Degos L.All trans retinoic acid as a differentiating therapy for acute promyelocytic leukemias. I Clinical results. Blood1990; 76: 1704-1709.
15.
Degos L, Chomienne C, Daniel MT, Berger R, Dombert H, Fenaux P, Castaigne S.Treatment of first relapse in acute promyelocytic leukemia with all transretinoic acid. Lancet1990; 2: 1440-1441.
16.
Sun G, Ouyang R, Chen S, Juang GS, Zhang T.Follow up of 481 patients with APL after CR using ATRA. Chin J Hematol1994; 15: 411-413.
17.
Fenaux P, Le Deley MC, Castaigne S, Archimbaud E, Chomienne C, Link H, Guerci A, Duarte M, Daniel MT, Bowen D.Effect of all transretinoic acid in newly diagnosed acute promyelocytic leukemia. Results of a multicenter randomized trial. Blood1993; 82: 3241-3249.
18.
Fenaux P, Chastang C, Chevret S, Sanz M, Dombret H, Archimbaud E, Fey M, Rayon C, Huguet F, Sotto J-J, Gardin C, Cony Makhoul P, Travade P, Solary E, Fegueux N, Bordessoule D, San Miguel J, Link H, Desablens B, Stamatoullas A, Deconinck E, Maloisel F, Castaigne S, Preudhomme C, Degos L.A randomized comparison of all transretinoic acid (ATRA) followed by chemotherapy and ATRA plus chemotherapy and the role of maintenance therapy in newly diagnosed acute promyelocytic leukemia. Blood1999; 94: 1192-1200.
19.
Sanz MA, Martín G, Rayón C, Esteve J, González M, Díaz-Mediavilla J, Bolufer P, Barragán E, Terol MJ, González JD, Colomer D, Chillón C, Rivas C, Gómez T, Ribera JM, Bornstein R, Román J, Calasanz MJ, Arias J, álvarez C, Ramos F, Debén G.A modified AIDA protocol with anthracycline-based consolidation results in high antileukemic efficacy and reduced toxicity in newly diagnosed PML/RARa-positive acute promyelocytic leukemia. Blood1999; 94: 3015-3021.
20.
Tallman MS, Andersen JW, Schiffer CA, Appelbaum FR, Feusner JH, Wood WG, Ogden A, Weinstein H, Shepherd L, Willman C, Bloomfield KD, Rowe JM, Wiernik PH.Alltrans retinoic acid in acute promyelocytic leukemia: long-term outcome results and prognostic factor analysis from Intergroup protocol 0129 [abstract]. Blood1999; 94: 3086a-3086a.
21.
Tallman MS.Therapy of acute promyelocytic leukemia: all-trans retinoic acid and beyond. Leukemia1998; 12: S37-S40.
22.
Avvisati G, LoCoco F, Diverio D, Falda M, Ferrara F, Lazzarino M, Russo D, Petti MC, Mandelli F.AIDA (all-trans retinoic acid+idarubicin) in newly diagnosed acute promyelocytic leukemia: a Gruppo Italiano Malattie Ematologiche Maligne dell’Adulto (GEMEMA) Pilot Study. Blood1996; 88: 1390-1398.
23.
Frankel SR, Eardly A, Lauwers G, Weiss M, Warrell RP.The‘retinoic acid syndrome’ in acute promyelocytic leukemia. Ann Intern Med1992; 117: 292-296.
24.
Frankel SR, Eardley A, Heller G, Berman E, Miller WH, Dmitrovsky E, Warrell RP.All-trans retinoic acid for acute promyelocytic leukemia: result of the New York Study. Ann Intern Med1994; 120: 278-286.
25.
Tallman MS, Andersen JW, Schiffer CA, Appelbaum FR, Feusner JH, Ogden A, Shepherd L, Willman C, Bloomfield CD, Rowe JM, Wiernik PH.All-trans retinoic acid in acute promyelocytic leukemia. N Engl J Med1997; 337: 1021-1028.
26.
De Botton S, Dombret H, Sanz M, San Miguel J, Caillot D, Zittoun R, Gardembas M, Stamatoulas A, Condé E, Guerci A, Gardin C, Geiser K, Cony Makhoul D, Reman O, de la Serna J, Lefrere F, Chomienne C, Chastang C, Degos L, Fenaux P, and the European APL Group. Incidence, clinical features, and outcomes of all trans retinoic acid syndrome in 413 cases of newly diagnosed acute promyelocytic leukemia. Blood1998; 92: 2712-2718.
27.
De Gentile A, Toubert ME, Dubois C, Krawice J, Schlagetes MH, Balitrand N, Castaigne S, Degos L, Rain JD.Induction of high-affinity GM-CSF receptors during alltrans retinoic acid in acute promyelocytic leukemia. Leukemia1994; 8: 1758-1762.
28.
Dubois C, Schlageter MH, De Gentile A, et al.Modulation of II-8 and II-1b and G-CSF secretion by all-trans retinoic acid in acute promyelocytic leukemia. Leukemia1994; 8: 1750-1757.
29.
Dubois C, Schlageter MH, De Gentile A, Guidez F, Balitrand N, Toubert ME, Krawice I, Fenaux P, Castaigne S, Najean Y.Hematopoietic growth factor expression and ATRA sensitivity in acute promyelocytic blast cells. Blood1994; 83: 3264-3270.
30.
Larson R, Brown D, Sklar L.Retinoic acid induces aggregation of the acute promyelocytic leukemia cell line NB-4 by utilization of LFA-1 and ICAM-2. Blood1997; 90: 2747-2756.
31.
Stadler M, Ganser A, Hoelzer K.Acute promyelocytic leukemia. N Engl J Med1994; 330: 140-141.
Mandelli F, Diverio D, Avvisati G, Luciano A, Barbui T, Bernasconi C, Broccia G, Cerri R, Falda M, Fioritoni G, Leoni F, Liso V, Petti MC, Rodeghiero F, Saglio G, Luce Vegna M, Visani G, Jehn U, Willemze R, Muus P, Pelicci PG, Biondi A, Lo Coco F.Molecular remission in PML/RAR alpha-positive acute promyelocytic leukemia by combined all-trans retinoic acid and idarubicin (AIDA) therapy. Gruppo Italiano-malattie Ematologiche Maligne dell’Adulto and Associazione Italiana di Ematologia ed Oncologia Pediatrica Cooperative Groups. Blood1997; 90: 1014-1021.
34.
Avvisati G.AIDA Protocol: the Italian way of treating APL [abstract]. Br J Haematol1998; 102: 593a-593a.
35.
Tallman MS, Andersen JW, Schiffer CA, Appelbaum FR, Feusner JH, Ogden A, Shepherd L, Rowe JM, François C, Larson RS, Wiernik PH.Clinical description of 44 patients with acute promyelocytic leukemia who developed the retinoic acid syndrome. Blood2000; 95: 90-95.
36.
Vahdat L, Maslak P, Miller WH, Eardley A, Heller G, Scheinberg DA, Warrell RP.Early mortality and the retinoic acid syndrome in acute promyelocytic leukemia: impact of leukocytosis, low-dose chemotherapy, PMN/RARa isoform, and CD13 expression in patients treated with all-trans retinoic acid. Blood1994; 84: 3843-3849.
37.
Warrell RP, Maslak P, Eardley A, Heller G, Miller WH, Frankel SR.Treatment of acute promyelocytic leukemia with all-trans retinoic acid: An update of the New York experience. Leukemia1994; 8: 926-933.
38.
Wiley JS, Firkin FC.Reduction of pulmonary toxicity by prednisolone prophylaxis during all-trans retinoic acid treatment of acute promyelocytic leukemia. Australian Leukemia Study Group. Leukemia1995; 9: 774-778.
39.
Firkin FC, Mathews JP, Bradstock KF, Wiley JS.A phase II study of all-trans retinoic acid (ATRA) with prednisone prophylaxis in the treatment of acute promyelocytic leukemia (APL)[abstract]Proc Am Soc Clin Oncol1999; 94: 4225a-4225a.
40.
Naranjo CA, Busto U, Sellers EM, Sandor P, Ruiz I, Roberts EA, Janecek E, Domecq C, Greenblatt DJ.A method for estimating the probability of adverse drug reactions. Clin Pharmacol Ther1981; 30: 239-245.
