Optic neuropathy due to chronic lymphocytic leukemia: The first manifestation of the disease

Maleita, Diogo; Pereira, Rita Gonçalves; Hipólito-Fernandes, Diogo; Moura-Coelho, Nuno; Cunha, João Paulo; Ferreira, Joana Tavares

doi:10.1016/j.ajoc.2020.100603

Cited by 3 publications

(5 citation statements)

References 12 publications

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“…However, this is not uniformly the case. Other reports indicate CNS involvement may occur in early stages of CLL and may be the presenting sign of CLL development beyond monoclonal peripheral blood lymphocytosis (6,9). Although optic nerve infiltration has been reported with multiple neoplastic entities, including lymphoma and leukemia (20,30,32), CLL presenting as optic neuropathy is rare (7,9).…”

Section: Discussionmentioning

confidence: 99%

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Optic Perineuritis Presenting as the Initial Manifestation of Central Nervous System Involvement in Rai Stage 0 Chronic Lymphocytic Leukemia

Parker¹,

Mays²,

Pratibhu³

et al. 2021

Journal of Neuro-Ophthalmology

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Background:To describe the first case of optic perineuritis because of meningeal involvement of early stage chronic lymphocytic leukemia (CLL).Methods:A case report and review of the literature.Results:A case of unilateral optic neuropathy associated with enhancement of the optic nerve sheath is described in a patient with a prior 2-year history of Rai Stage 0 CLL. Lumbar puncture revealed a lymphocytic pleocytosis. Cerebrospinal fluid flow cytometry revealed a monoclonal expansion of CD5+ B cells compatible with CLL, matching the flow cytometry characteristics of his peripheral blood.Conclusions:Optic perineuritis is often initially diagnosed as optic neuritis, yet the 2 have different etiologies and follow a different clinical course. Orbital MRI with contrast structurally separates the 2, revealing a characteristic pattern of peripheral optic nerve sheath rather than primary optic nerve enhancement. Etiologies of optic perineuritis are varied and include inflammatory, infectious, neoplastic, and toxic entities. Central nervous system (CNS) involvement by chronic lymphocytic leukemia is unusual, but cranial nerve and meningeal involvement have been reported. This case adds central nervous system chronic lymphocytic leukemia to the list of differential diagnostic possibilities for optic perineuritis. It also alerts clinicians to consider optic perineuritis as a potential presenting feature of CNS involvement in otherwise asymptomatic and stable CLL.

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Section: Discussionmentioning

confidence: 99%

“…Extranodal involvement can also occur in the lymphoid tissue of any organ, most commonly the liver, skin, pleura, kidney, and gastrointestinal tract (3,5). Rarely, CLL has been reported to involve the central nervous system (CNS) and even more rarely the optic nerve or optic nerve sheath (6)(7)(8)(9).…”

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confidence: 99%

Optic Perineuritis Presenting as the Initial Manifestation of Central Nervous System Involvement in Rai Stage 0 Chronic Lymphocytic Leukemia

Parker¹,

Mays²,

Pratibhu³

et al. 2021

Journal of Neuro-Ophthalmology

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“…It is well established that the optic nerve can be the site of CNS relapse in leukemia and lymphoma, despite presumed systemic and medullary remission (5,(10)(11)(12). Although most optic neuropathy cases occur in the setting of preexisting CNS disease, there are cases in which leukemic optic neuropathy is the first manifestation of CNS involvement (2)(3)(4)(13)(14)(15)(16). Leukemic infiltration into the optic nerve is postulated to occur through the leptomeninges through infiltration of the perivascular spaces within the pial septa of the nerve (15).…”

Section: Discussionmentioning

confidence: 99%

“…O ptic neuropathy in the context of leukemia and lymphoma can be attributed to infectious, inflammatory, infiltrative, ischemic, or toxic-metabolic etiolo-gies (1). Because the optic nerve is a direct extension of the central nervous system (CNS), the finding of optic nerve dysfunction in patients with leukemia or lymphoma raises concern for CNS involvement or relapse, thus warranting prompt evaluation and treatment (2)(3)(4)(5)(6)(7)(8). Even in the setting of prompt management, leukemic optic neuropathy carries a poor visual prognosis and many patients ultimately progress to optic atrophy and death (9).…”

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confidence: 99%

Leukemic and Lymphomatous Optic Neuropathy: A Case Series

Lee¹,

Farooq²,

Shah³

2021

Journal of Neuro-Ophthalmology

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Background: Optic neuropathy in the context of leukemia and lymphoma raises concern for central nervous system involvement or relapse and warrants prompt evaluation and treatment. To date, a gold standard for the diagnosis and management of leukemic optic neuropathy has yet to be established. Methods: Case series and review of the literature. Two illustrative cases were selected to discuss their treatment course and outcome. Results: We report 7 cases of patients with leukemia or lymphoma presenting with optic nerve infiltration. All patients received steroid therapy for presumed infiltrative optic neuropathy, and 4 patients underwent radiation therapy. Along with systemic chemotherapy, all patients received intrathecal chemotherapy except one. Three patients received chimeric antigen receptor T-cell therapy. Conclusions: Leukemic and lymphomatous optic neuropathy is difficult to diagnose and treat, and there is no gold standard for diagnosis or treatment in the current literature. We help clarify how this disease should be approached in a multidisciplinary fashion and on an individual basis to correctly diagnose and treat the vision loss, while considering the patient's long-term prognosis based on their systemic disease.

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“…Central nervous system (CNS) involvement is a rare complication of chronic lymphocytic leukemia (CLL) with an incidence of 0.8%−2% and includes manifestations such as cognitive impairment, cerebellar dysfunction, and cranial nerve palsies [1] , [2] , [3] . Ophthalmic disease in CLL is uncommon with 15 cases of optic nerve infiltration reported [1] , [2] , [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] . We contribute one additional case of infiltrative optic neuritis in a CLL along with a comprehensive literature review.…”

Section: Introductionmentioning

confidence: 99%

Leukemic infiltration of the optic nerve in chronic lymphocytic leukemia: A case report and review of literature

Liu,

Hadyah,

Park

et al. 2023

Leukemia Research Reports

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Optic neuropathy due to chronic lymphocytic leukemia: The first manifestation of the disease

Cited by 3 publications

References 12 publications

Optic Perineuritis Presenting as the Initial Manifestation of Central Nervous System Involvement in Rai Stage 0 Chronic Lymphocytic Leukemia

Optic Perineuritis Presenting as the Initial Manifestation of Central Nervous System Involvement in Rai Stage 0 Chronic Lymphocytic Leukemia

Leukemic and Lymphomatous Optic Neuropathy: A Case Series

Leukemic infiltration of the optic nerve in chronic lymphocytic leukemia: A case report and review of literature

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