Chronic lymphocytic leukaemia (CLL) of the lacrimal sac
Reviewed by Yamini Krishna

The authors present a case series of three patients with known CLL, two of which presented with mass and epiphora and one case with epiphora alone. All three patients had initially being diagnosed and were treated for chronic dacryocystitis. Histopathological examination of nasolacrimal sac biopsies, taken during dacryocystorhinostomy (DCR) due to clinical suspicion and / or perioperative appearances of the lacrimal sac, revealed a diagnosis of CLL of the lacrimal sac. Immunohistochemical staining confirmed the tumour cells had a classical CLL phenotype (CD5+; CD20+; CD21+; CD23+; CD79a+; BCL-2+ with synthesis of IgM). One case had molecular testing, next generation sequencing with a panel known to be relevant for CLL prognosis and treatment response, but no somatic mutations were detected. In summary all three patients had systemic CLL with secondary lacrimal sac infiltration, were elderly but one had presumed bilateral involvement (two DCRs) and one with bony destruction which is rare. One patient was already under the care of haematologists, whilst the other two were re-referred. Two of the patients had been treated with chemotherapy whilst one had low-dose local area irradiation as no further disease was detected. Lymphomas of the lacrimal sac are rare accounting for 2-6% of malignant lacrimal sac tumours; may be primary or secondary; present with symptoms typical of secondary acquired nasolacrimal duct obstruction and are thus often misdiagnosed. It should therefore be suspected in such patients with persistent nasolacrimal obstruction. Even though involvement of the lacrimal sac by CLL is usually seen in the advanced stages, the clinical course of these patients after diagnosis is very variable. Nasolacrimal sac biopsies can yield sufficient DNA for next generation sequencing and assessment of somatic mutation status, relevant for prognostication. However, whether or not lacrimal sac involvement is associated with the same somatic gene mutations is unclear and requires further evaluation in a larger case series.

Chronic lymphocytic leukaemia (CLL)/small-cell lymphocytic lymphoma (SLL) of the lacrimal sac: a case series.
Krishna Y, Irion LD, Karim S, et al.
OCULAR ONCOLOGY AND PATHOLOGY
2017;3:224-8.