Oman Journal of Ophthalmology

: 2021  |  Volume : 14  |  Issue : 3  |  Page : 134--135

Chronic myeloid leukemia versus acute myeloid leukemia in patients with retinoblastoma

Ann-Marie Leahey1, Carol L Shields2,  
1 Division of Pediatric Oncology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
2 Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA

Correspondence Address:
Ann-Marie Leahey
Division of PediatricOncology, Children'sHospital of Philadelphia, Philadelphia, PA 19104

How to cite this article:
Leahey AM, Shields CL. Chronic myeloid leukemia versus acute myeloid leukemia in patients with retinoblastoma.Oman J Ophthalmol 2021;14:134-135

How to cite this URL:
Leahey AM, Shields CL. Chronic myeloid leukemia versus acute myeloid leukemia in patients with retinoblastoma. Oman J Ophthalmol [serial online] 2021 [cited 2021 Dec 8 ];14:134-135
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Full Text

We read with interest the case report of chronic myeloid leukemia (CML) in chronic phase in a patient with heritable retinoblastoma (RB) who had received treatment with high-dose carboplatin, etoposide, and vincristine.[1] CML is divided into chronic, accelerated, and blast phases, resulting from the t (9;22) (q34; q11) translocation. In addition to their case and another referenced by the authors,[2] there is a third case in the literature described by Gombos et al.[3] In that series, case #4 of acute myeloid leukemia (AML) appears actually to describe a case of CML in myeloid blast crisis 22 years after the diagnosis of RB. Of note, this patient did not receive etoposide.

In contradistinction, secondary AML following treatment with topoisomerase inhibitors such as etoposide has an average latency of 1–2 years. This subtype of AML is defined by KMT2A gene (or chromosome) rearrangement. It has been shown to fuse to 20 other gene partners. Another type of secondary AML can occur after exposure to alkylating agents and presents 5–10 years following exposure. It is associated with monosomies or deletions of chromosomes 5 and 17.

Ionizing radiation is the only environmental factor implicated in the etiology of CML.[3] Based on data from survivors of atomic bombs, the average time from radiation exposure to clinical symptoms of CML is 8 years. Two of the three known patients with RB who developed CML, referenced in this report, had received external beam radiotherapy (EBRT) and the time interval to CML was over 20 years in both cases.

The patient in this case report did not share the environmental exposure to EBRT, but all three reports share the fact that there was bilateral disease and all share the common genetic milieu of heritable RB. Thus, therein may be the causal connection. Abnormalities in the RB gene, protein, and signaling pathway have been associated with CML cell survival as well as conversion to accelerated and blast phases.[5],[6],[7]

Fortunately, CML is a rare leukemia that is highly treatable with currently available tyrosine kinase inhibitors. We agree that long-term monitoring of patients with heritable RB is mandatory, but there is no evidence to suggest that treatment with etoposide leads to CML.

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Conflicts of interest

There are no conflicts of interest.


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