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Clinicopathologic Reports, Case Reports, and Small Case Series
December 2002

Acute Myeloid Leukemia Manifesting Initially as a Conjunctival Mass in a Patient With Acquired Immunodeficiency Syndrome

Author Affiliations
 

W. RICHARDGREENMD

Arch Ophthalmol. 2002;120(12):1741-1742. doi:

The ophthalmic manifestations of human immunodeficiency virus (HIV) infection range from molluscum contagiosum of the eyelid to cotton-wool spots of the retina.14 Additionally, HIV infection is associated with several opportunistic cancers of the eye and orbit,2 including conjunctival squamous cell carcinoma,3 Kaposi sarcoma,4 and non-Hodgkin lymphoma.4 In this report, we illustrate the rare association of HIV infection with acute myeloid leukemia that manifested initially as an epibulbar mass.

Report of a Case

A 47-year-old African American man with acquired immunodeficiency syndrome (AIDS) who was being treated with highly active antiretroviral therapy developed bilateral pink conjunctival lesions during a 2-week period. There were 3 lesions noted on the bulbar conjunctiva of each eye (Figure 1). The subconjunctival tumors measured up to 7 mm in basal diameter and 2 mm in thickness and had no apparent intrinsic vasculature. Dilated, tortuous blood vessels fed each tumor. Visual acuity was 20/20 OU, and both fundi were normal.

Figure 1.
The left eye of a patient with
acquired immunodeficiency syndrome shows a pink bulbar subconjunctival mass
with dilated feeder vessels.

The left eye of a patient with acquired immunodeficiency syndrome shows a pink bulbar subconjunctival mass with dilated feeder vessels.

Excisional biopsy of one of the lesions was performed to role out Kaposi sarcoma, non-Hodgkin lymphoma, leukemia, or an opportunistic infection. Routine histopathologic examination showed a diffuse infiltrate of poorly differentiated malignant cells that had large, irregular vesicular nuclei, prominent nucleoli, and relatively abundant cytoplasm (Figure 2A). The cells showed positive immunoreactivity for CD43 (myeloid marker), lysozyme (leukocyte marker), and KP-1 (CD68, monocyte cytoplasmic antigen marker). They stained negative for lymphocyte markers CD20 (B lymphocyte marker) and UCHL-1 (T-lymphocyte marker), cytokeratin (epithelial marker), and melanoma markers HMB-45 and S100 protein. A stain for myeloperoxidase was weakly positive, and the Leder chloroacetate esterase stain disclosed cytoplasmic granules in tumor cells evincing granulocytic differentiation (Figure 2B). The findings were consistent with granulocytic or myeloid sarcoma. Myeloid sarcoma indicates tissue involvement by acute myeloid leukemia cells.

Figure 2.
A, A photomicrograph shows a section
of conjunctival granulocytic sarcoma with large cells, prominent nuclei, eosinophilic
nucleoli, and relatively abundant cytoplasm (hematoxylin-eosin, original magnification
×250). B, A photomicrograph of a section of conjunctival granulocytic
sarcoma stained with Leder chloroacetate esterase demonstrates the presence
of cytoplasmic lysozyme granules (original magnification ×250).

A, A photomicrograph shows a section of conjunctival granulocytic sarcoma with large cells, prominent nuclei, eosinophilic nucleoli, and relatively abundant cytoplasm (hematoxylin-eosin, original magnification ×250). B, A photomicrograph of a section of conjunctival granulocytic sarcoma stained with Leder chloroacetate esterase demonstrates the presence of cytoplasmic lysozyme granules (original magnification ×250).

The patient reported previous small subcutaneous lesions on his arms, back, abdomen, and legs, most of which had appeared and regressed during the prior 10 weeks. An excisional biopsy specimen of one of the skin tumors originally was diagnosed elsewhere as a high-grade lymphoma, but the tumor was reclassified by the National Cancer Institute as an immature hematopoietic malignancy consistent with myeloid sarcoma with monocytic differentiation. Additional immunohistochemical studies performed at the National Cancer Institute confirmed that the tumor cells were reactive for lysozyme, KP-1, and CD43 but were negative for CD20 (B lymphocyte marker), CD79A (B lymphocyte marker), myeloperoxidase (leukocyte marker), CD30 (B lymphocyte, T lymphocyte, and monocyte marker), CD34 (pluripotent progenitor cell marker), and human herpesvirus 8. In situ hybridization studies for Epstein-Barr virus were negative. A bone marrow biopsy and aspiration showed hypocellular marrow with left-shifted myelopoiesis. The peripheral blood smear contained rare immature hematopoietic cells consistent with myeloid origin. The patient was treated with chemotherapy with initial favorable results, but systemic involvement was found 5 months later, and the patient declined further treatment.

Comment

Patients with AIDS have impaired cellular immunity that can lead to opportunistic infections and malignancies.1,2 Previously published reports have shown a relationship between AIDS and conjunctival malignancies such as Kaposi sarcoma, non-Hodgkin lymphoma,4 and squamous cell carcinoma.3 These ocular malignancies found in patients with AIDS are suspected to be of viral origin.14 There is evidence linking squamous cell carcinoma to human papillomavirus.3 Kaposi sarcoma–associated human herpesvirus 84 and Epstein-Barr virus2 have been detected in Kaposi sarcoma and lymphoma tissue, respectively. It has been postulated that HIV may play a permissive role by allowing these viruses to evade the host immune system and proliferate.2 Epstein-Barr virus and human herpesvirus 8 were not detected in our patient's tumor, however.

According to the French-American-British classification of acute leukemia, acute myeloid leukemia is classified into 9 different subtypes depending on the morphological and cytochemical characteristics of cells.5 These subtypes range from M0 (acute myeloid leukemia without cytologic maturation) to M7 (acute megakaryocytic leukemia). The most commonly diagnosed subtype is the M2 type (30% of patients) in which the cells show significant maturation. Morphological and immunohistochemical findings in our patient appeared most consistent with subtype M5, acute monocytic/monoblastic leukemia (10% of patients). Interestingly, M5 is the leukemic subtype most often associated with soft tissue involvement.

Acute myeloid leukemia occurs most commonly in white men, and its incidence increases with age. The average age at diagnosis is older than 65 years.5 The initial symptoms of acute myeloid leukemia include fatigue, weight loss, bleeding, easy bruising, and susceptibility to infection. The incidence of acute myeloid leukemia in patients with AIDS is approximately 2-fold compared with the general population.6 Acute myeloid leukemia is caused by numerous nonrandom chromosomal abnormalities, the majority of which are translations, and environmental carcinogens.5

Granulocytic sarcoma is a variant of acute myeloid leukemia that occurs as an invasive solid mass, most frequently in bone.7 Zimmerman and Font7 described 33 patients with granulocytic sarcoma of the ocular tissues, involving the orbit (26 patients), eyelid (4 patients), lacrimal gland (3 patients), uveal tract (2 patients), and conjunctiva (1 patient). In 52% of these patients, hematologic evidence of leukemia was present at diagnosis. In 88% of patients, the ophthalmic lesion was the initial sign of leukemia. In a series of 121 children from Turkey with acute myelomonocytic leukemia (M4), 27% presented with granulocytic sarcoma in either the orbit or eyes.8

The pink conjunctival tumors present in our patient were initially suspicious for Kaposi sarcoma, lymphoma, or leukemia. All 3 malignancies can be associated with HIV infection. The immunodeficient patient should be watched carefully for opportunistic infections and malignancies. Appropriate diagnosis is crucial for providing proper treatment. Thus, in addition to Kaposi sarcoma, non-Hodgkin lymphoma, and squamous cell carcinoma, acute myeloid leukemia should also be recognized as a conjunctival malignancy associated with AIDS.

This study was supported by the Eye Tumor Research Foundation, Philadelphia (Dr C. L. Shields), the Macula Foundation, New York, NY (Dr C. L. Shields), the Paul Kayser International Award of Merit in Retina Research, Houston, Tex (Dr J. A. Shields), and the Noel T. and Sarah L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital, Philadelphia (Dr Rice).

Corresponding author: Carol L. Shields, MD, Oncology Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107.

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