Role of Prophylactic Steroids in Differentiation Syndrome

Abstract

Acute promyelocytic leukemia (APML) is defined as a balanced chromosomal translocation between chromosomes 15 and 17 t(15;17)(q24;q21), which results in the formation of promyelocytic leukemiaretinoic acid receptor-alpha (PML-RARA) fusion protein. A widespread recommendation for APML treatment is combined all-trans retinoic acid (ATRA)/arsenic trioxide (ATO) therapy. Differentiation syndrome (DS), or retinoic acid syndrome, is one of the well-known complications of APML treated with ATRA or ATO. The presenting symptoms of APML-induced DS are diverse, and rare symptoms are easily misdiagnosed. However, unexplained fever, dyspnea, weight gain > 5 kg, leukocytosis, acute renal failure, and a chest radiograph demonstrating pleural or pericardial effusion are the most common manifestations of DS. Early recognition and prompt initiation of corticosteroids are key factors in the management of DS. As soon as ATRA/ATO therapy is started, prophylactic treatment with steroids has been recommended to minimize the severity of DS. It is proposed that ATRA/ATO should be stopped or held once the signs and symptoms of DS develop. This case report describes a 45-year-old male who was diagnosed with APML after he developed episodes of hematuria and nose bleeding at home. The patient was also given an empiric steroid along with ATRA/ATO to lessen the intensity of DS. Our study suggests that early initiation of prophylactic steroid treatment can improve the prognosis and mortality of patients with APML-induced DS.

Acute promyelocytic leukemia in children cancer hospital Egypt

“…Differentiation syndrome was discovered multiple times throughout therapy, affecting the continuation of ATRA administration, followed by omission or change of the ATRA dose, which may have an impact on the APL patient's result. The 5-year OS for patients who did not have DS was 74.2%, and for those who complained of recurrent DS was 71.0%, with no significant P-value; this finding was also mentioned by Sultana et al [ 31 ]. On the other hand, age, obesity, starting promyelocytic count, hydroxyurea treatment, and risk stratification did not increase the prevalence of DS.…”

“…ATRA started once suspicion of APL diagnosis, the most common manifestations of DS are unexplained fever, dyspnea, water retention resulting in weight gain of more than 5 kg, laboratory findings showing elevated WBC, acute renal insufficiency, and radiological diagnosis as pleural or pericardial effusion detected by chest x-ray [ 31 ].…”

“…Early detection, examination, and therapy with corticosteroids can save lives in the management of the life-threatening illness DS [ 31 ]. In the current study, 21 (46.6%) of 45 patients who received prophylactic steroids initially developed a DS.…”

A Race Against Time: Early-Onset Differentiation Syndrome Following All-Trans-Retinoic-Acid (ATRA) Therapy in Acute Promyelocytic Leukemia (AML-M3)

“…DS is associated with severe complications if not treated promptly. One of the most crucial aspects of treating DS is early detection and timely corticosteroid initiation [4,5]. Our research includes the signs, symptoms, and steps that can be followed for prompt diagnosis and treatment of DS.…”