|Year : 2018 | Volume
| Issue : 1 | Page : 65-67
Chronic lymphocytic leukemia presenting as unilateral extraocular muscle enlargement and proptosis
Amun Sachdev1, Nigel Timothy O'Connor2, Suresh Reddy Sagili1
1 Department of Ophthalmology, Royal Shrewsbury Hospital, Shrewsbury, SY3 8XQ, United Kingdom
2 Department of Haematology, Royal Shrewsbury Hospital, Shrewsbury, SY3 8XQ, United Kingdom
|Date of Web Publication||5-Mar-2018|
Department of Ophthalmology, Royal Shrewsbury Hospital, Mytton Oak Road, Shrewsbury, SY3 8XQ
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Orbital involvement in chronic lymphocytic leukemia (CLL) is rare with very few published cases. We describe a case of unilateral isolated extraocular muscle enlargement in a patient with CLL. An incisional biopsy was performed from the left medial rectus muscle and histology revealed a lymphocytic infiltrate suggestive of CLL. Complete resolution of signs and symptoms was subsequently achieved with chemotherapy. We would suggest that in patients presenting with atypical clinical features, it is important to consider nonthyroid-related causes of extraocular muscle enlargement and a muscle biopsy should be considered to exclude neoplastic causes.
Keywords: Chronic lymphocytic leukemia, extraocular muscle enlargement, proptosis
|How to cite this article:|
Sachdev A, O'Connor NT, Sagili SR. Chronic lymphocytic leukemia presenting as unilateral extraocular muscle enlargement and proptosis. Oman J Ophthalmol 2018;11:65-7
|How to cite this URL:|
Sachdev A, O'Connor NT, Sagili SR. Chronic lymphocytic leukemia presenting as unilateral extraocular muscle enlargement and proptosis. Oman J Ophthalmol [serial online] 2018 [cited 2020 Jan 25];11:65-7. Available from: http://www.ojoonline.org/text.asp?2018/11/1/65/226353
| Introduction|| |
Extraocular muscle enlargement is a common feature of thyroid orbitopathy in Graves' disease. In the literature, the most common nonthyroid causes of muscle enlargement include inflammatory, vascular, and neoplastic processes.
In neoplastic cases, the underlying cause is usually either metastatic or orbital lymphoma involving the extraocular muscles. Leukemic infiltration of orbital tissues is rare and comprises <1% of all orbital tumors. Although all types of leukemia may involve the orbit, this is more common in acute leukemias compared to chronic leukemias. A literature search revealed that there are very few published cases of extraocular muscle enlargement due to chronic lymphocytic leukemia (CLL).,, Orbital leukemic tumors may clinically manifest as proptosis, eyelid edema, pain, motility disturbances, and chemosis.
The authors describe a rare case of unilateral isolated extraocular muscle enlargement in a patient with CLL who responded well to chemotherapy.
| Case Report|| |
A 55-year-old Caucasian female presented to the hematologist and was subsequently referred to the oculoplastic clinic, with a 3-month history of worsening left-sided headache associated with pain and swelling of the left upper and lower eyelids. Her medical history included a 4-year history of CLL, which was being managed conservatively without any active treatment and a parathyroidectomy 1 year previously for the treatment of hyperparathyroidism.
On examination, best-corrected visual acuity was 6/6 in her right eye and reduced to 6/18 in her affected left eye. Color vision was mildly affected in her left eye; however, there was no relative afferent pupillary defect. The left upper and lower eyelids were swollen, associated with 3 mm of left-sided axial proptosis and restriction of extraocular movements. Intraocular pressures were 18 mmHg bilaterally. Posterior segment examination was normal with bilateral healthy optic discs and retinas. There was no lymphadenopathy, hepatomegaly, or splenomegaly.
Baseline blood tests revealed normal thyroid function tests, renal function tests, liver function tests, and bone profile. In accordance with her CLL, the patient's white blood cell count was 56 × 109/L, lymphocyte count was 49.6 × 109/L, platelet count was 229 × 109/L, and hemoglobin was 130 g/L.
Magnetic resonance imaging of the orbits revealed the enlargement of all extraocular muscles on the left side [Figure 1]. An incisional biopsy was performed from the left medial rectus muscle and subsequent histological examination revealed a lymphocytic infiltrate between the muscle fibers that was compatible with CLL (CD20 and CD5 positive) [Figure 2].
|Figure 1: Magnetic resonance imaging scan, coronal section, T2 image, showing enlargement of all extraocular muscles in the left orbit|
Click here to view
|Figure 2: Histopathology of the left medial rectus muscle revealing chronic lymphocytic leukemia. (a) H and E, ×40 revealing an infiltrate with multiple lymphocytes. (b) Positive immunohistochemistry with CD20 (a B cell marker). (c) Positive immunohistochemistry with CD5 (a chronic lymphocytic leukemia marker). (d) Positive immunohistochemistry with Ki-67 (suggestive of low-grade lymphoma)|
Click here to view
The patient was treated with four cycles of chemotherapy (fludarabine, cyclophosphamide, rituximab) at 6-week interval. On subsequent clinic visits, the patient was found to have made a rapid recovery and reported a complete resolution of all her symptoms within 4 weeks of starting treatment. She remained free of symptoms at her 6-month follow-up.
| Discussion|| |
Isolated extraocular muscle involvement secondary to CLL is extremely rare. There have been only three published cases of extraocular muscle enlargement due to CLL.,, One case described a 72-year-old male who presented with bilateral enlargement of all extraocular muscles due to CLL and responded well to orbital radiotherapy. The second case described a 51-year-old male who developed unilateral leukemic infiltration of the lateral rectus muscle 10 years after a systemic diagnosis of CLL and died 10 months later. The third case described a 53-year-old male with unilateral enlargement of the extraocular muscles; elevated thyroid-stimulating hormone was suggestive of thyroid eye disease, but biopsy of the lateral rectus revealed lymphocytic infiltration suggestive of CLL; he responded well to orbital radiotherapy.
While all previously reported cases were in male patients, our case was in a female patient. This article presents the rare case of a 55-year-old female who developed unilateral enlargement of all the extraocular muscles 4 years after she was diagnosed with CLL. The patient underwent incisional biopsy and subsequent histological analysis confirmed leukemic infiltration. She then made a rapid recovery with chemotherapy.
| Conclusion|| |
Although extraocular muscle enlargement is often associated with thyroid-related orbitopathy, it is important to consider other causes, such as inflammatory, vascular, or neoplastic processes. A diagnostic extraocular muscle biopsy should be considered in patients presenting with atypical clinical features.
We would like to thank Dr. George R Powell and Dr. Nicholas J Green, Consultant Histopathologists, for interpreting and providing photos of the histological findings.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Lacey B, Chang W, Rootman J. Nonthyroid causes of extraocular muscle disease. Surv Ophthalmol 1999;44:187-213.
Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111:997-1008.
Bidar M, Wilson MW, Laquis SJ, Wilson TD, Fleming JC, Wesley RE, et al.
Clinical and imaging characteristics of orbital leukemic tumors. Ophthal Plast Reconstr Surg 2007;23:87-93.
Ramkissoon YD, Lee RW, Malik R, Hsuan JD, Potts MJ. Bilateral infiltrative disease of the extraocular muscles: A rare clinical presentation of early stage chronic lymphocytic leukemia. Orbit 2008;27:293-5.
Kiratli H, Balci KE, Himmetoglu C, Uner A. Isolated extraocular muscle involvement as the ophthalmic manifestation of leukaemia. Clin Exp Ophthalmol 2009;37:609-13.
Wilson ME, Thornton S, Murchison AP, Bilyk JR. Clinical challenge: An orbital Hickam's dictum. Surv Ophthalmol 2016;61:799-805.
[Figure 1], [Figure 2]