Table 2.

Incidence and Outcome of ATRA Syndrome in APL Treated With ATRA

Authors No. of PatientsProphylaxis of ATRA Syndrome No. of ATRA Syndrome (%) Treatment of ATRA SyndromeOutcome of ATRA Syndrome Overall Incidence of Fatal ATRA Syndrome (%)
CR (%) Death (%)
Fenaux15 (APL 91 trial)  54  Chemotherapy added if —WBC >5,000/μL at diagnosis —WBC >6,000, 10,000, 15,000/μL by days 5, 10 and 15 of ATRA, respectively  6 (11)  Symptomatic  100   0  
Kanamaru18  109  Chemotherapy added if —WBC >3,000/μL at diagnosis —Circulating blasts >1,000/μL during treatment 7 (6)  DXM: 3 cases  ND  14  
Vahdat30  78  No prophylaxis 21 (27)  DXM: leukaphereses chemotherapy  72  28  
Wiley31  22  —if WBC <1,000/μL initially: oral prednisone —if WBC >1,000/μL —if WBC >1,000/μL initially: chemotherapy  2 (9)   ½ ½  4  
Avvisatti32  20  Chemotherapy in all patients  2 (10)  DXM ½  ½  
Tallmann33  172  Hydroxyurea if —WBC >1,000/μL at diagnosis —WBC >30,000/μL with ATRA  47 (27)  DXM ND  7  1  
Cortes36  17  Chemotherapy added if circulating blasts + promyelocytes >10,000/μL at diagnosis or increase with ATRA  4 (24)  Chemotherapy leukaphereses ¾  ¼  5  
Present study (APL93 Trial)  413  Same as APL 91 trial  64 (15)  DXM 89   8  
Authors No. of PatientsProphylaxis of ATRA Syndrome No. of ATRA Syndrome (%) Treatment of ATRA SyndromeOutcome of ATRA Syndrome Overall Incidence of Fatal ATRA Syndrome (%)
CR (%) Death (%)
Fenaux15 (APL 91 trial)  54  Chemotherapy added if —WBC >5,000/μL at diagnosis —WBC >6,000, 10,000, 15,000/μL by days 5, 10 and 15 of ATRA, respectively  6 (11)  Symptomatic  100   0  
Kanamaru18  109  Chemotherapy added if —WBC >3,000/μL at diagnosis —Circulating blasts >1,000/μL during treatment 7 (6)  DXM: 3 cases  ND  14  
Vahdat30  78  No prophylaxis 21 (27)  DXM: leukaphereses chemotherapy  72  28  
Wiley31  22  —if WBC <1,000/μL initially: oral prednisone —if WBC >1,000/μL —if WBC >1,000/μL initially: chemotherapy  2 (9)   ½ ½  4  
Avvisatti32  20  Chemotherapy in all patients  2 (10)  DXM ½  ½  
Tallmann33  172  Hydroxyurea if —WBC >1,000/μL at diagnosis —WBC >30,000/μL with ATRA  47 (27)  DXM ND  7  1  
Cortes36  17  Chemotherapy added if circulating blasts + promyelocytes >10,000/μL at diagnosis or increase with ATRA  4 (24)  Chemotherapy leukaphereses ¾  ¼  5  
Present study (APL93 Trial)  413  Same as APL 91 trial  64 (15)  DXM 89   8  

Abbreviation: ND, not done.

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