الفهرس 
Differentiation syndrome in adult patients with acute promyelocytic leukemia at national cancer institute Cairo university (2015-2019
LIST OF ABBREVIATIONSOnly 14 pages are availabe for public view
 |  | from | 100 |  |  |
| | | from | 100 | | |
Abstract
Background
Acute promyelocytic leukemia (APL) is a distinguished subset of acute
myeloid leukemia which is characterized by fusion gene transcript
PML-RAR-alpha. It is a medical emergency with a very high pre-treatment
mortality. All-Trans Retinoic Acid (ATRA) is the mainstay in the treatment
of APL and used in all modern regimens. Differentiation Syndrome (DS) is an
inflammatory reaction with increased capillary permeability that occurs in up to
25% of patients with APL treated with ATRA. The aim of the study is to review
the risk factors, presentation, management and clinical outcome of DS in APL
at the National Cancer Institute, Cairo Egypt.
Methods
This is a retrospective study that includes all adult patients with APL
diagnosed and treated at National Cancer Institute, Cairo Egypt from January
2015 to December 2019.
Results
During this study, 97 adult patients with APL were assessed. The median
age at presentation was 37 years with females slightly more prominent than
males (56.7% vs 43.3%) . Bleeding was the predominant presenting symptom in
50 (51.5%) patients with gum bleeding occurring in 25 (25.5%) patients. Most
patients were low risk APL 68 (70.1%) patients and 29 (29.9%) patients were
high risk. The results of our study concluded that during induction, 70 (72.2%)
patients developed complete remission while 27 (27.8%) patients died. DS was
reported in 29 (29.9%) patients. 22 (75.9%) patients developed DS in the first
seven days of ATRA administration. Only one patient (3.7%) died due to DS
with respiratory failure and acute kidney injury. After a median follow up period
of 31.8 months, three year survival rate was 58.8% and mean OS was 86.2
months. There was no association between OS or RFS and the incidence of DS
with (P >0.05). High risk APL patients had a significantly shorter OS and RFS
when compared to the low-risk group with (P <0.001) and (P=0.034)
respectively. 24 (24.7%) patients required ICU admission during the treatment
course and it was associated with a shorter OS and a shorter RFS with (P
<0.001). Also patients who developed DIC had a significantly shorter OS and
RFS with (P =0.019) and (P=0.017) respectively.
Conclusion
Differentiation syndrome can be a life-threatening complication in
patients with APL undergoing induction therapy with ATRA or arsenic trioxide.
Clinical manifestations of DS greatly overlap with other disorders, such as
infection or sepsis, heart failure, pulmonary thromboembolism and diffuse
alveolar hemorrhage. There are no universally accepted diagnostic criteria for
APL DS but the presence of any of the following factors justifies a presumptive
diagnosis of APL DS and the empiric start of steroid therapy : dyspnoea,
unexplained fever, weight gain >5 kg, unexplained hypotension, acute renal
failure, a chest radiograph demonstrating pulmonary infiltrates or pleural or
pericardial effusion. Prompt steroid initiation is crucial along with investigating
other disorders. In the current study, we found no predictive factors for the
development of differentiation syndrome. There was no association between OS
or RFS and the incidence of DS with (P >0.05) .