Two cases of differentiation syndrome with ocular manifestations in patients with acute promyelocytic leukaemia treated with all-trans retinoic acid and arsenic trioxide

Abstract

PurposeTo describe two cases of differentiation syndrome presenting with ocular manifestations including bilateral chorioretinopathy in patients with acute promyelocytic leukaemia treated with all-trans retinoic acid and arsenic trioxide differentiation therapy.ObservationsThis observational case series identifies two patients at a single tertiary institution diagnosed with differentiation syndrome with associated ophthalmic involvement. Both patients reported bilateral reduction in visual acuity at days fourteen and ten respectively following initiation of differentiation therapy in addition to developing other systemic manifestations of differentiation syndrome. Both patients received the same chemotherapeutic regimen including both all-trans retinoic acid and arsenic trioxide as well as ten days of routine differentiation syndrome prophylaxis with oral prednisolone. Case 1 presented with bilateral pale yellow sub-retinal lesions concentrated at the posterior poles with ocular coherence tomography (OCT) evidence of bilateral multifocal areas of focal RPE elevation and adhesion to the thickened outer retina with interspersed sub-retinal fluid. Fluorescein angiography revealed areas of early hyperflouresence corresponding to the yellow chorioretinal lesions with late diffuse leakage of fluid into the subretinal space. Case 2 presented with a similar characteristic retinal findings on fundoscopy and optical coherence tomography. Both patients experienced rapid improvement in the visual symptoms and marked resolution of the sub-retinal fluid within seven to fourteen days of onset with excellent long-term visual outcome. Both patients achieved molecular remission after induction and received standard consolidation and maintenance therapy without visual disturbance.Conclusion and importanceOcular manifestations of differentiation syndrome have been only recently recognised. We present a case series of two patients with differentiation syndrome with ocular involvement. Common to both presentations was the presence of bilateral reduction in visual acuity with multifocal serous retinal detachment secondary to chorioretinopathy. The visual outcome from both presentations was excellent with rapid normalisation of visual acuity and resolution of the sub-retinal fluid with only the first case having their differentiation therapy temporarily withheld during the acute phase of illness.

Incidence of Differentiation Syndrome Associated with Treatment Regimens in Acute Myeloid Leukemia: A Systematic Review of the Literature

“…We identified a total of 838 articles from PubMed; after removing duplicates and non-English studies, 591 titles and abstracts were screened, resulting in the exclusion of 434 articles ( Figure 1 ). In addition to the remaining 157 full-text articles, we further identified 57 articles [ 12 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 77 ,…”

Pseudotumor Cerebri in the Setting of Differentiation Syndrome in a Patient with Acute Promyelocytic Leukemia Treated with All-trans Retinoic Acid During Induction Therapy

“…In the literature, only three such cases are reported till date, which include chorioretinopathy with multifocal serous neurosensory retinal detachments, choroidal hyperpermeability and bilateral angle closure due to presumed anterior rotation of the iris-lens diaphragm secondary to choroidal thickening. 13,14 Our patient had bilateral disc edema along with superficial exudation in right eye. The ocular findings and the history was suggestive of PC in the setting of DS.…”

Differentiation Syndrome with Severe Abdominal Pain During Induction Treatment of Acute Promyelocytic Leukemia: A Case Report

“…Based on these pathophysiologic mechanisms, APL DS presents in diverse and nonspecific symptoms, which make the diagnosis of DS challenging. In addition to the most common signs, rare symptoms such as diffuse alveolar hemorrhage, 7 Sweet’s syndrome 8 and ocular manifestations 9 have also been reported. It is necessary to identify DS when patients present with rare symptoms.…”