1. Optic nerve involvement in Acute Lymbhobastic Leukaemia , more than what meets the eye

    Acute Lymphoblastic Leukaemia can affect the eye in various ways . It may present with an ischaemic optic nerve involvement (1) or optic nerve swelling due to direct involvement . But sometimes it may also present as an optic nerve involvement as an effect of a drug like Linezolid which has been used for treatment of infections which coexist (2) or it may present as an infiltration of the nerve in combination with herpes virus (3)

    Though papillodema is the most frequent sign of optic nerve involvement it can be absent in direct infiltration of the nerve by leukemic cells, or present just due to increased intracranial pressure , or swelling because of retrolaminar leukemic invasion.

    The involvement in invasion of the optic nerve usually occurs during the evolution of acute lymphoblastic leukemia, but that , when as is elucidated in the present case and also by Mess et al earlier in 2003, is the first sign of acute lymphoblastic leukemia or of extramedullary relapse after remission , it usually means a poor prognosis for the patient, especially if it happens when the patient is still receiving treatment, rather than after it. And so earlier it is picked up, the better .Hence the value of a routine check even in absence of symptoms .

    Optic nerve swelling may mean many things and one needs to differentiate the causes of the optic nerve swelling clinically and with non invasive tests like Ocular coherence tomography ( OCT ) so as to institute proper therapy . Optic nerve involvement, as one needs to emphasise again , is not always a result of leukaemia infiltration as a surprise in remission . Sometimes Peripapillary retinal leukemic infiltration may be associated with papilledema in a patient without cranial or optic nerve involvement.(4) . Optic nerve swelling may be seen due to optic neuritis (5) which can be seen sometimes in cases of leukaemia without leukaemic infiltration .Sometimes the peri neuritis may be transient (6) Papillodema due to central nervous system involvement may be seen .Medications used for treatment may sometimes cause secondary optic nerve affectation and may include but be not limited to toxicity of chemotherapy, antibiotics or radiotherapy, ischemia after anaemia or hyperviscosity, and opportunistic infections in immunocompromised patients. In such cases to figure out whether the optic nerve involvement is indeed direct infiltration one may look for other layers of eye being involved . (7) So if there are other layers involved there is a clue and an eye surgeon will pick it up because conjunctival , scleral and trabecular involvement is characteristic

    But in a case where optic nerve alone is involved one can use OCT to figure out what's hapenning at the optic nerve level. The swelling begins not at superior or inferior edge of the optic nerve as in papillodema due to raised intra cranial pressure and "doesn't follow ISNT rule " ( that's a rule in glaucoma where loss occurs in that order but sometimes swellings occur in similar way ) and also one doesn't see a typical swelling of optic neuritis then it's obvious that a lot of clues are waiting to be picked up

    The present case report helped us manage a case locally and so we are thankful to the authors and the journal for the same

    The optic nerve is relatively unaffected by systemic chemotherapy and serves as a sanctuary of ALL.(8) So it is probably essential that an eye surgeon opinion is sought regularly since there's a lot they can tell , lots more than meets the non ophthalmologist's eye .

    Reference :-

    1) Chaudhuri T, Roy S, Roy P. Ischaemic optic neuropathy induced sudden blindness as an initial presentation of acute lymphoblastic leukemia. Indian J Med Paediatr Oncol 2013;34:335-6

    2) Joshi L, Taylor SRJ, Large O, Yacoub S, Lightman S (2009). A case of optic neuropathy after short-term linezolid use in a patient with acute lymphocytic leukemia. Clin Infect Dis 48:73-74. ISSN 1537-6591.

    3) Bhatt UK, Gregory ME, Madi MS, Fraser M, Woodruff HA (2008). Sequential leukemic infiltration and human herpervirus optic neuropathy in acute lymphoblastic leukemia. Journal of AAPOS 12:200-202. ISSN 1528-3933.

    4) ?eki? O, Biberoglu E, Esen F. Peripapillary retinal leukemic infiltration associated with papilledema in a T-ALL patient without cranial or optic nerve involvement. Tumori. 2016 Nov 11;102(Suppl. 2). doi: 10.5301/tj.5000490

    5) Yo Han Ra, Sun Young Park, Soo Ah Im, Jee Young Kim, Nak Gyun Chung, Bin Cho. Enhancement of Optic Nerve in Leukemic Patients: Leukemic Infiltration of Optic Nerve versus Optic Neuritis.iMRI 2016;20:167-174.

    6) Townsend JH, Dubovy SR, Pasol J, Lam BL. Transient optic perineuritis as the initial presentation of central nervous system involvement by pre-B cell lymphocytic leukemia. J Neuroophthalmol 2013;33:162-164

    7) Rosenthal AR. Ocular manifestations of leukemia. A review. Ophthalmology 1983;90:899-905

    8) Ninane J, Taylor D, Day S. The eye as a sanctuary in acute lymphoblastic leukemia. Lancet. 1980;1:452-3.

    Conflict of Interest:

    None declared

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  2. Interesting Case

    Editor Madam, it is very interesting case where the Lymphoblasts appeared to cross over the blood brain barrier to appear in the CSF or a leak from adjoining bone or capillary contributed it, and more interestingly blast cells in CSF spared rest of the brain and nerves while preferentially deposited in retinal layers formed by optic nerve, like of the previous cases cited in the report. If any other significant reason appeared than per chance juxta papillary deposit of lymphoblasts in recurred leukaemia, authors may better comment.

    Conflict of Interest:

    None declared

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