Citation: Park J, Jeon H, Choi HY. Myeloid sarcoma of the eyelid
mimicking pre-septal cellulitis in acute myeloid leukemia. Int J Ophthalmol
2019;12(8):1359-1361. DOI:10.18240/ijo.2019.08.21
·Letter to the Editor·
Myeloid
sarcoma of the eyelid mimicking pre-septal cellulitis in acute myeloid leukemia
Jungyul Park1, Hyeshin Jeon1,2,
Hee-Young Choi1,2
1Department
of Ophthalmology, Pusan National University Hospital, Busan 602-739, Korea
2Biomedical
Research Institute of Pusan National University Hospital, Busan 602-739, Korea
Correspondence
to: Hee-Young
Choi. Department of Ophthalmology, School of Medicine, Pusan National
University, 1-10, Ami-dong, Seo-gu, Busan 602-739, Korea. hychoi@pusan.ac.kr.
Received:
DOI:10.18240/ijo.2019.08.21
Citation: Park
J, Jeon H, Choi HY. Myeloid sarcoma of the eyelid mimicking pre-septal
cellulitis in acute myeloid leukemia. Int J Ophthalmol
2019;12(8):1359-1361
Dear Editor,
I am Dr.
Jungyul Park from the Division of oculoplasty, Department of Ophthalmology,
Pusan National University Hospital, Busan, Korea. I write to discuss a case of
myeloid sarcoma of the eyelid mimicking pre-septal cellulitis in acute myeloid leukemia
(AML). This article
does not contain any studies with human participants or animals performed by
any of the authors and informed consent for publication was obtained from the family of the patient
in this case report, as potentially identifying information may be included in
this article.
Myeloid
sarcoma is a rare extramedullary manifestation of leukemia, which can occur in
isolation or concurrently with a myelodysplastic syndrome, myeloproliferative
disease, or AML. Ocular myeloid sarcomas may appear at any time during the
course of AML, and often develop simultaneously during onset or relapse of
systemic symptoms of leukemia[1-3].
Ocular involvement of myeloid sarcoma is uncommon, few cases of eyelid
involvement have been reported[4]. According to
previous reports, myeloid sarcoma on the eyelid forms a mass-like lesion[5-7]. In this case report, we present a
case of a patient, who has a history of lymphadenopathy for several months,
with an eyelid myeloid sarcoma, which mimicked pre-septal cellulitis without a
palpable mass, as the presenting sign of AML without systemic symptoms.
A
68-year-old Asian man with a history of lymphadenopathy for several months
presented to our clinic with a right upper eyelid swelling, which had been
recalcitrant to treatment (at another tertiary medical center) for orbital
cellulitis of the eyelid. The painful swelling was reddish, relatively hard,
and partially necrotic with blackish discoloration on the eyelid crease area.
No palpable mass, madarosis, or proptosis were found (Figure 1). Orbital
computed tomography revealed diffuse skin thickening and subcutaneous
enhancement of the right periorbital space (Figure 2). We diagnosed the lesion
as pre-septal cellulitis; thus, empirical antibiotics were initiated and
laboratory blood tests were performed. Despite treatment with broad-spectrum
antibiotics for 5d, the lesion did not subside and necrosis increased. Eyelid
biopsy was performed, which showed myeloblasts with limited cytoplasm,
round-to-oval nuclei with fine chromatic and prominent nucleoli. Numerous
mitotic figures were identified. Immunostaining was positive for
myeloperoxidase and negative for cluster of differentiation (CD) 3, CD15, CD10,
CD20, and CD34 (Figure 3). The pathological diagnosis was myeloid sarcoma.
Laboratory test results were as follows: white blood cell count 50 000/µL with
neutrophil 12% (band neutrophils 0 and segmented neutrophils 12%), lymphocytes
11%, monocytes 6%, eosinophils 0, basophils 0, metamyelocytes 0, and blasts
70%; red blood cell count 3.99×106/µL; hemoglobin
Figure 1
Clinical photographs of the patient demonstrate a mild right sided ptosis with
indurated eyelid, swelling, erythema and blackish skin color change The conjunctiva on right eye is
injected and mild chemosis was found. No palpable mass was found.
Figure 2
Orbital computed tomography (non-enhanced) demonstrate
diffuse skin thickening (white arrow) and subcutaneous layer enhancement (white
arrow head) of right periorbital, right zygomatic and right preseptal space.
Figure 3 The specimen is composed of
myeloblasts with limited cytoplasm, round-to-oval nuclei with fine chromatic
and prominent nucleoli (A). Numerous mitotic figures were identified. H&E
stain, ×400. Positive immunostaining for myeloperoxidase (B). These findings
suggested myeloid sarcoma.
There are
two major types of acute and chronic leukemia: lymphocytic and myelocytic.
Thus, there are four patterns of leukemia: acute lymphocytic leukemia, chronic
lymphocytic leukemia, acute myelocytic leukemia, and chronic myelocytic
leukemia[8]. Among these four, although it is less
common, acute myelocytic leukemia can be observed initially with orbital
involvement, most commonly in young children[5,8]. Soft tissue infiltrations of leukemic cells that form
a tumoral lesion have been referred to as a granulocytic sarcoma or chloroma[9-14]; however, the term “myeloid
sarcoma” is currently preferred[15]. If the
lesion resembles cellulitis and involves dermal infiltrations of leukemic
cells, it is called Sweet’s syndrome or acute febrile neutrophilic dermatosis.
Sweet’s syndrome is characterized by fever, neutrophilic leukocytosis,
erythematous, and tenderness of skin lesion that respond well to corticosteroid
therapy[16-17].
Myeloid
sarcoma is a rare disease with an incidence of 2/1 000
Infiltration
of leukemic cells into periocular tissue can cause cellulitis[8].
However, the patient in the present case exhibited progressive worsening of the
lesion, despite the application of broad-spectrum antibiotic regimens,
including antifungal agents. Therefore, we concluded that these symptoms were
due to myeloid sarcoma presenting as cellulitis. In terms of clinical symptoms
prior to histological examination, it is necessary to consider Sweet’s syndrome
as a differential diagnosis, although it is characterized by specific symptoms
that were not present in this patient. Moreover, we diagnosed our patient with
myeloid sarcoma by eyelid biopsy[16].
On the basis
of previous studies of myeloid sarcoma in the literature, infiltration of
myeloid sarcoma to the orbital and ocular adnexa, as well as the surrounding
organs and tissues, is more prevalent in pediatric patients, with 88%
experiencing exophthalmos; notably, there was no proptosis in our patient, who
did not present with characteristic symptoms or history of leukemia. In
contrast to our patient, bilateral tumor has been observed more frequently than
unilateral tumor. Nevertheless, these reports have not clearly explained
initial symptoms, features, or directionality of myeloid sarcoma arising from
the periorbital regions, including the eyelids. Based on these prior reports,
myeloid sarcoma can infiltrate the orbital and ocular adnexa in various ways.
If an elderly patient without characteristic leukemic symptoms shows lesions
suggestive of eyelid cellulitis that do not respond to adequate treatment,
clinicians should consider the possibility of myeloid sarcoma as a differential
diagnosis; these lesions may constitute the initial manifestation of AML[5,8,20,22-23].
The patient
in this case received antibiotic treatments for multiple days in multiple
hospitals; consequently, his bone marrow examination and final diagnosis at our
center was delayed. From this report, we conclude that it is essential to
consider various differential diagnoses for a patient with cellulitis and to
perform in-depth blood tests and biopsies to ensure that the patient receives
appropriate treatment early.
ACKNOWLEDGEMENTS
Conflicts of
Interest: Park J,
None; Jeon H, None; Choi HY, None.
REFERENCES