Discussion
Diagnosis With Brief Discussion
- Diagnosis
- ATRA syndrome (Differentiation syndrome)
- Radiologic Findings
- Fig 1. Chest PA shows central ground glass opacity (GGO) at the right lung and septal lines at both lungs.
Fig 2-4. CT scans reveal central GGO at the right upper lobe and interlobular septal thickening at the right middle lobe, right lower lobe, and left upper lobe. A small amount of pericardial effusion and bilateral pleural effusion are present.
Our patient underwent ATRA therapy due to APL 10 days before taking the chest radiograph in Figure 1.
- Brief Review
- Acute promyelocytic leukemia (APL) is one of the subtypes of acute myeloid leukemia (AML) and is a rare disease that occurs in 10-15% of patients with AML. ATRA is used as an accepted therapy for APL. Formerly known as ATRA syndrome, differentiation syndrome (DS) is a potentially life-threatening complication of ATRA that usually emerges during the first days or weeks of ATRA therapy. Dyspnea, pulmonary infiltrates, pleural effusion, fever, weight gain, peripheral edema, hypotension, and acute renal failure are the hallmarks of DS. DS occurs in approximately 20-25% of all APL patients undergoing ATRA and is treated with intravenous dexamethasone or by considering discontinuation of ATRA. The mortality rate of DS is known to be 5-30%.
Radiologic features of DS is not specific and similar to congestive heart failure with pulmonary edema: these include cardiomegaly, widening of the vascular pedicle width, increased pulmonary blood volume, peribronchial cuffing, ground-glass opacity, septal lines, and pleural effusion.
- References
- 1. Jung JI, Choi JE, Hahn ST, Min CK, Kim CC, Park SH. Radiologic features of all-trans-retinoic acid syndrome. AJR Am J Roentgenol. 2002 Feb;178(2):475-80. doi: 10.2214/ajr.178.2.1780475. PMID: 11804921.
2. Yilmaz, M., Kantarjian, H. & Ravandi, F. Acute promyelocytic leukemia current treatment algorithms. Blood Cancer J. 11, 123 (2021). https://doi.org/10.1038/s41408-021-00514-3
- Keywords