Yanamandra Uday, Khadwal Alka, Saikia Uma Nahar, Malhotra Pankaj
Department of Internal Medicine, Armed Forces Medical College, Pune, Maharashtra, India Department of Internal Medicine (Clinical Hematology Division), Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Internal Medicine (Clinical Hematology Division), Post Graduate Institute of Medical Education and Research, Chandigarh, India.
BMJ Case Rep. 2016 Jan 20;2016:bcr2015212205. doi: 10.1136/bcr-2015-212205.
Acute promyelocytic leukaemia is among the most curable haematological malignancies after the introduction of differentiating agents (arsenic trioxide (ATO) and all-trans-retinoic-acid (ATRA)). Despite excellent cure rates, approaching 85-95% in various series, APL is associated with significant early mortality and morbidity. ATRA-related side effects partly contribute to this morbidity, which commonly presents as differentiation syndrome, pseudo tumour cerebri, dermatitis, gastrointestinal disorders, liver dysfunction (raised transaminases) and dryness of skin/eyes. Rarely, ATRA can lead to hypercalcaemia, genital vasculitis, erythaema nodosum and Sweet's syndrome. We present two cases of ATRA-associated genital vasculitis while being managed with a combination of ATO with ATRA therapy. Both patients developed these lesions despite being on prophylactic steroids (0.5 mg/kg). We also discuss the pathophysiology, clinical manifestations, differential diagnosis and treatment of genital vasculitis as a rare adverse event of ATRA.