Rationale: Extramedullary relapse of acute lymphoblastic leukemia (ALL) involving the orbit is uncommon, and lacrimal gland infiltration is particularly rare in adults. Bilateral lacrimal gland enlargement can be clinically nonspecific and may be misinterpreted as inflammatory or autoimmune disease, potentially delaying diagnosis. This case report describes a rare adult case of extramedullary relapse of B-cell ALL presenting as bilateral lacrimal gland enlargement following allogeneic hematopoietic stem cell transplantation (HSCT). Patient concerns: A 55-year-old woman presented with a 4-day history of a painless, palpable mass in the right upper eyelid. The patient had a history of Philadelphia chromosome-negative B-cell ALL and had previously undergone chemotherapy followed by allogeneic HSCT. Diagnoses: Orbital computed tomography demonstrated diffuse, heterogeneous enlargement of both lacrimal glands, more prominent on the right side. Incisional biopsy of the right lacrimal gland with immunohistochemical staining (terminal deoxynucleotidyl transferase positive and B-lineage markers positive) confirmed extramedullary relapse of B-cell ALL. Interventions: The patient received salvage chemotherapy followed by a 2nd allogeneic HSCT. Outcomes: Follow-up orbital computed tomography performed 6 months after treatment showed complete resolution of bilateral lacrimal gland enlargement without evidence of local progression. Lessons: Bilateral lacrimal gland enlargement in patients with a history of ALL may represent isolated extramedullary relapse, even in the absence of hematologic abnormalities. Early histopathologic confirmation is essential to prevent diagnostic delay and to enable timely systemic management in ALL patients.
Jang et al. (Fri,) studied this question.