Case Report

PML-RARA Fusion Transcripts Detectable 8 Months prior to Promyelocytic Blast Crisis in Chronic Myeloid Leukemia

Table 3

Summary of 21 reported cases of promyelocytic blast crisis in CML.

SexAge (years)Treatment during CML chronic phaseInterval between CML diagnosis and blast crisisTreatment during promyelocytic blast phaseConventional cytogenetic resultsSurvival (after blast crisis onset)/outcomeReference

F82Imatinib2 yearsNDNDND[9]

M22N/AConcurrentATRA, chemotherapy, allogeneic SCT46, XY, t(9;22)(q34;q11) [5], 47, XY, t(9;22), +8, t(15;17)(q22;q11-21) [4]118 days after allogeneic SCT[10]

M31Cytosine arabinoside, 6-thioguanine#27 monthsND46, XY, t(9;22) [40%], 46, XY, t(9;22), t(15;17)(q15;q21) [60%]3 months[11]

F69Imatinib13 monthsATRA, idarubicin, Ara-C, imatinib, ATO46, XX, t(9;22)(q34;q11.2), t(15;17)(q22;q12) [19], 46, XX [1]§‡Complete molecular response[12]

F85None#10 monthsNone46, XX, t(9;22), t(15;17)(q22;q21) [100%]2 days[13]

M78Imatinib, dasatinib7 yearsATRA, ATO46, XY, t(9;22)(q34;q11.2), t(15;17)(q24;q21) [100%]§‡2 months[14]

M32Imatinib6 monthsATRA, imatinib46, XY, t(9;22)(q34;q11), t(15;?;17)(q22;?;q21) [100%]§‡Complete remission[15]

M37Misulban#10 monthsDaunorubicin, cytosine arabinoside46,(9;22), t(15;17) [26], 46, t(9;22) [5]ND[16]

F52Hydroxyurea#3 yearsMitoxantrone, etoposidet(9;22)(q34;q11.2), t(15;17)(q24;q11.2–12) [9]6 weeks[17]

M55Hydroxyurea#2 yearsATRA, mitoxantrone, cytosine arabinoside, etoposidet(9;22)(q34;q11.2), t(15;17)(q22;q11.2–12) [2]ND[17]

M60ND#3 yearsCytarabine, mitoxantrone, etoposide, idarubicine, 6-thioguanine46, XY, t(9;22)(q34;q11)[40%], 46, XY, t(9;22)(q34;q11), t(15;17)(q22;q21) [60%]§3 weeks[18]

M50ND#3 yearsND46, XY, t(9;12;22)(q34;q22;q11) [43.8%], 46, XY, t(9;12;22)(q34;q22;q11), t(15;17)(q22;q12–21) [56.2%]ND[19]

M30Busulfan#10 monthsN4-Behenoyl-1-β-D-arabinofuranosyl-cytosine, daunorubicin, 6-mercaptopurine, prednisone46, XY, t(9;22)(q34;q11) [25%], 46, XY, t(9;22)(q34;q11), t(3;21)(q12;q22) [75%]5 months[20]

F32Busulfan#8 yearsDoxorubicin74, XX, t(9;22), t(9;22)(3n±)1 month[21]

M3Busulfan, alpha-2a interferon, hydroxyurea, cytarabine#3.5 years“Chemotherapy”Hypodiploidy, t(9;22), iso(17q)2 months[22]

M38ND#25 monthsAziridinyl-benzoquinone46, XY, t(9;22)(q34;q11) [4], 46, XY, t(15;17), t(9;22) [4], 45, X, -Y, t(15;17), t(9;22) [2]1 month[23]

M26N/AConcurrentCytarabine, arabinoside, daunorubicin, dasatinib, allogeneic SCTt(9;22)Complete molecular remission[24]

M31Hydroxy-carbamide, imatinib4 monthsATRA, ATO, dasatinib, idarubicin, cytarabine, allogeneic SCTND§‡Complete molecular remission[25]

M27Natural interferon-α4 yearsND51, XY, +der(1)t(1;17)(p11;q11), +7, +8, +8, t(9;22)(q34;q11), 22q-ND[26]

M38Allopurinol, busulfan25 monthsND46, XY, t(9;22)(q34:q11) [4], 46, XY, t(15;17)(q22;q12?), t(9;22) (q34;q11) [6]47 days[27]

M48ND#6 yearsATRA46, XY [6]/48, XY, +17, +der(1)t(1;?)(p13;?), t(9;22)(q34;q11) [14]>87 days[28]

Full karyotype information not provided. #Publication date prior to 2001. FISH studies negative for PML-RARA fusion. §FISH studies positive for PML-RARA fusion. FISH and RT-PCR testing not performed. RT-PCR positive for PML/RARA fusion. ND, no data provided; N/A, not applicable; ATRA, all-trans retinoic acid; ATO, arsenic trioxide; SCT, stem-cell transplantation.