Leptomeningeal Relapse of Acute Promyelocytic Leukemia

Abstract

Extramedullary relapse (EMR) of acute promyelocytic leukemia (APL) is a rare entity, with predilection to involve the central nervous system (CNS). Risk factors include leukocytosis of > 10 × 10⁹/L, bcr3 isoform, microgranular variant, age > 45 years and development of subarachnoid hemorrhage (SAH) during induction therapy. We report a case of APL who completed induction and consolidation therapy but subsequently relapsed with leptomeningeal involvement. Retrospectively, we identified several risk factors for EMR in our patient. Interestingly, the use of all-trans retinoic acid has recently been associated with higher risk of EMR possibly due to up-regulation of adhesion molecules on the surface of the leukemic cell, resulting in their passage through the endothelium to extramedullary tissues. However, data remain conflicting in that regard. Although universal CNS prophylaxis has been suggested, the low incidence of EMR among APL patients renders this strategy less attractive. Nonetheless, active surveillance and CNS prophylaxis may be considered in patients at high risk for EMR, particularly in those of SAH during induction therapy. Further research is needed to evaluate the effectiveness and safety of this strategy.

Keywords: Acute promyelocytic leukemia; Extramedullary relapse; Leptomeningeal relapse.

Figure 1

T1-weighted MRI image showing leptomeningeal enhancement (arrows).

Figure 2

Cerebrospinal fluid is hypercelluar with numerous hypogranular promyelocytes.