Clinical features of the retinoic acid syndrome:
(1) leukocytosis
(2) fever
(3) increase in body weight
(4) respiratory distress, dyspnea
(5) pulmonary infiltrate on chest X-ray
(6) episodic hypotension
(7) effusions (pleural, pericardial)
(8) cardiac failure with edema of the lower extremities
(9) renal failure
De Botton (1998) noted that most patients had 3 or more of these findings.
Risk of the syndrome is increased in patients with:
(1) a high initial white blood cell count (some use > 5,000 per µL, others > 10,000 per µL)
(2) rising white blood cell count after therapy is started (De Botton used > 6,000 per µL at day 5; > 10,000 per µL at day 10; > 15,000 per µL on day 15)
(3) blast cells expressing CD13
Therapy may include:
(1) administration of high dose corticosteroid therapy
(2) antineoplastic therapy (with rapidly increasing WBC counts)
