Acute Promyelocytic Leukemia (APL), a distinct subtype of acute myeloid leukemia is a relatively rare disease, characterized by a severe coagulopathy which is often present at the time of diagnosis. Mortality due to bleeding complications during induction is more common in this subtype than in other FAB classifications. The number of newly diagnosed cases in the US is estimated to be 600 to 800 cases a year. The introduction of all-trans retinoic acid (ATRA) into the therapy of APL has completely revolutionized the management and outcome of this disease. The treatment and cure of patients with APL depend not only on the effective use of combination therapy but also involves critical supportive care measures.
The aim of this study was to analyze the number of red cells, platelets, plasma and cryoprecipitate transfused during the induction phase of treatment until time to response.
Patient and transfusion data was retrospectively collected from the Leukemia Department files and Blood bank records at the UT MD Anderson Cancer Center from 2010 to 2011.
There were 28 newly diagnosed APL patients ([16F: 12 M]; 2 AA/6 Hispanic/20 White), median age 49 (21-84) and included patients who did not go on to clinical trials due to early complications. Karyotyping was obtained on 26 (93%) patients. Confirmation of the PML-RARα short or long transcripts was obtained in 25 (89%) patients by quantitative RT-PCR all of whom showed the PML-RARα fusion transcript. Induction therapy was started on day -1 to day 0 from the date of diagnosis in 5 (18%) patients, Day 1 in 13 (46%), Day 2 in 1 (3%), Day 3 in 3 (11%), Day 4 in 2 (7%), Day 6 in 3 (11%) and Day 7 in 1 (3%) patient. 24 (86%) patientsreceived Arsenic + ATRA, 3 (11%) received Arsenic + ATRA + Idarubicinand 1 (3%)received Arsenic + ATRA + Gemtuzumab Ozogamicin. 4 (14%) patients died early from complications of severe coagulopathy. Response to therapy was noted in 24 (86%) patients, median 25 (range 19-63) days from start of treatment. Red cells were transfused to 25 (89%) patients, median 6 (range 1-29) units, platelets to 23 (82%) patients, median 5 (1-47) units, plasma to 11 (39%) patients, median 8 (2-38) units and cryoprecipitate to 14 (50%) patients, median 10 (2-20) units.
There was 1 (3%) patient who did not require blood or blood products, 3 (11%) did not require red cell transfusions, 5 (18%) platelet transfusions, 17 (61%) plasma transfusions and 14 (50%) did not require cryoprecipitate. Of the 24 patients who responded to therapy, 22 (79%) patients are alive. One patient has been lost to follow up. The remaining 21 (75%) patients are in molecular remission with a median follow-up of 714 (256-1110) days from the date of response. Two (7%) patients died in molecular remission from unrelated non-hematologic causes (204, 283 days from their date of response). Table 1
Total Number of Patients (n) | 28 |
Transfusion Requirements | |
Patients receiving Red Cells | 25 (89%) |
Patients receiving Platelets | 23 (82%) |
Patients receiving FFP | 11 (39%) |
Patient receiving Cryoprecipitate | 14 (50%) |
Red cells transfused, median (range)units | 5.5 (0-29) |
Platelets transfused, median (range) | 4 (0-47) |
Fresh Frozen Plasma transfused, median (range) units | 0 (0-38) |
Cryoprecipitate transfused, median (range) units | 2 (0-20) |
Alive, in complete molecular remission | 22 (79%) |
Time from response to induction therapy, median (range) days | 714 (256- 1110) |
Total Number of Patients (n) | 28 |
Transfusion Requirements | |
Patients receiving Red Cells | 25 (89%) |
Patients receiving Platelets | 23 (82%) |
Patients receiving FFP | 11 (39%) |
Patient receiving Cryoprecipitate | 14 (50%) |
Red cells transfused, median (range)units | 5.5 (0-29) |
Platelets transfused, median (range) | 4 (0-47) |
Fresh Frozen Plasma transfused, median (range) units | 0 (0-38) |
Cryoprecipitate transfused, median (range) units | 2 (0-20) |
Alive, in complete molecular remission | 22 (79%) |
Time from response to induction therapy, median (range) days | 714 (256- 1110) |
The results of the laboratory studies at the time of diagnosis/ time of response are as follows; WBC median 1.2 K (0.5-17.9)/median 3.3 K/UL (1.0-5.5), Hgb median 8.39 G/Dl (5.9-12.1/median 10.3 G/Dl (8.1-12.1), platelet count median 31 K/UL (3-87)/median 180 K/UL (49-1335), BM blast median 1% (0-64), median 1% (0-4), BM progranulocytes median 59% (0-93)/median 1% (0-7), BM normoblast median 9% (1-35)/median 28% (0-72%), PT median 16.2 secs (14.7-21.0)/ median 14.3 sec (13.1-15.5), INR median 1.29 (1.12-1.76)/median 1.10 (0.97-1.20), aPTT median 29.9 secs (26.0-41.0)/ median 32.2 secs (24.1-47.4), D -Dimer median 19.83 mcg/ml (3.71->20.00)/median 0.96 mcg/ml (0.39-6.09), Fibrinogen median 172 MG/DL (77-461)/ median 399 MG/DL (164-856) and LDH median 883 IU/L (374-2561)/median 591 IU/L (444-1084).
In conclusion, our review found that the majority of cases required red cells and platelet transfusion but only 50% of the patients required plasma or cryoprecipitate transfusion support for their coagulopathy.
No relevant conflicts of interest to declare.
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