CLINICOPATHOLOGIC REPORTS, CASE REPORTS, AND SMALL CASE SERIES SECTION EDITOR: W. RICHARD GREEN, MD leukemia that manifested initially as cyte marker), cytokeratin (epithe- Acute Myeloid Leukemia an epibulbar mass. lial marker), and melanoma mark- Manifesting Initially ers HMB-45 and S100 protein. A stain as a Conjunctival Mass Report of a Case. A 47-year-old Af- for myeloperoxidase was weakly posi- rican American man with acquired tive, and the Leder chloroacetate es- in a Patient With immunodeficiency syndrome (AIDS) terase stain disclosed cytoplasmic Acquired Immunodeficiency who was being treated with highly ac- granules in tumor cells evincing Syndrome tive antiretroviral therapy devel- granulocytic differentiation (Figure oped bilateral pink conjunctival le- 2B). The findings were consistent The ophthalmic manifestations of sions during a 2-week period. There with granulocytic or myeloid sar- human immunodeficiency virus were 3 lesions noted on the bulbar coma. Myeloid sarcoma indicates tis- (HIV) infection range from mollus- conjunctiva of each eye (Figure 1). sue involvement by acute myeloid cum contagiosum of the eyelid to The subconjunctival tumors mea- leukemia cells. cotton-wool spots of the retina.1-4 sured up to 7 mm in basal diameter The patient reported previous Additionally, HIV infection is asso- and 2 mm in thickness and had no small subcutaneous lesions on his ciated with several opportunistic apparent intrinsic vasculature. Di- arms, back, abdomen, and legs, most cancers of the eye and orbit,2 includ- lated, tortuous blood vessels fed each of which had appeared and re- ing conjunctival squamous cell car- tumor. Visual acuity was 20/20 OU, gressed during the prior 10 weeks. cinoma,3 Kaposi sarcoma,4 and non- and both fundi were normal. An excisional biopsy specimen of Hodgkin lymphoma.4 In this report, Excisional biopsy of one of the one of the skin tumors originally was we illustrate the rare association of lesions was performed to role out Ka- diagnosed elsewhere as a high- HIV infection with acute myeloid posi sarcoma, non-Hodgkin lym- grade lymphoma, but the tumor was phoma, leukemia, or an opportunis- reclassified by the National Cancer tic infection. Routine histopathologic Institute as an immature hemato- examination showed a diffuse infil- poietic malignancy consistent with trate of poorly differentiated malig- myeloid sarcoma with monocytic nant cells that had large, irregular ve- differentiation. Additional immu- sicular nuclei, prominent nucleoli, nohistochemical studies per- and relatively abundant cytoplasm formed at the National Cancer In- (Figure 2A). The cells showed posi- stitute confirmed that the tumor cells tive immunoreactivity for CD43 (my- were reactive for lysozyme, KP-1, eloid marker), lysozyme (leukocyte and CD43 but were negative for marker), and KP-1 (CD68, mono- CD20 (B lymphocyte marker), cyte cytoplasmic antigen marker). CD79A (B lymphocyte marker), my- They stained negative for lympho- eloperoxidase (leukocyte marker), Figure 1. The left eye of a patient with acquired immunodeficiency syndrome shows a pink bulbar cyte markers CD20 (B lymphocyte CD30 (B lymphocyte, T lympho- subconjunctival mass with dilated feeder vessels. marker) and UCHL-1 (T-lympho- cyte, and monocyte marker), CD34 A B 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 ϫ250). B, A photomicrograph of a section of conjunctival granulocytic sarcoma stained with Leder chloroacetate esterase demonstrates the presence of cytoplasmic lysozyme granules (original magnification ϫ250). (REPRINTED) ARCH OPHTHALMOL / VOL 120, DEC 2002 WWW.ARCHOPHTHALMOL.COM 1741 ©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 (pluripotent progenitor cell marker), Acute myeloid leukemia oc- delphia (Dr C. L. Shields), the Macula and human herpesvirus 8. In situ hy- curs most commonly in white men, Foundation, New York, NY (Dr C. L. bridization studies for Epstein-Barr and its incidence increases with age. Shields), the Paul Kayser Interna- virus were negative. A bone mar- The average age at diagnosis is older tional Award of Merit in Retina Re- row biopsy and aspiration showed than 65 years.5 The initial symp- search, Houston, Tex (Dr J. A. hypocellular marrow with left- toms of acute myeloid leukemia in- Shields), and the Noel T. and Sarah shifted myelopoiesis. The periph- clude fatigue, weight loss, bleed- L. Simmonds Endowment for Oph- eral blood smear contained rare ing, easy bruising, and susceptibility thalmic Pathology, Wills Eye Hospi- immature hematopoietic cells con- to infection. The incidence of acute tal, Philadelphia (Dr Rice). sistent with myeloid origin. The pa- myeloid leukemia in patients with Corresponding author: Carol L. tient was treated with chemo- AIDS is approximately 2-fold com- Shields, MD, Oncology Service, Wills therapy with initial favorable results, pared with the general popula- Eye Hospital, 900 Walnut St, Phila- but systemic involvement was found tion.6 Acute myeloid leukemia is delphia, PA 19107. 5 months later, and the patient de- caused by numerous nonrandom 1. Cunningham ET, Margolis TP. Ocular manifes- clined further treatment. chromosomal abnormalities, the ma- tations of human immunodeficiency virus in- jority of which are translations, and fection. N Engl J Med. 1998;339:236-244. environmental carcinogens.5 2. Purtilo DT. Opportunistic cancers in patients Comment. Patients with AIDS have with immunodeficiency syndromes. Arch Pathol impaired cellular immunity that can Granulocytic sarcoma is a vari- Lab Med. 1987;111:1123-1129. lead to opportunistic infections and ant of acute myeloid leukemia that 3. Winward KE, Curtin VT. Conjunctival squa- 1,2 mous cell carcinoma in a patient with human im- malignancies. Previously pub- occurs as an invasive solid mass, most munodeficiency virus infection. Am J Ophthal- 7 lished reports have shown a relation- frequently in bone. Zimmerman and mol. 1989;107:554-555. ship between AIDS and conjuncti- Font7 described 33 patients with 4. Tunc M, Simmons M, Char D, Herndier B. Non- Hodgkin’s lymphoma and Kaposi’s sarcoma in val malignancies such as Kaposi granulocytic sarcoma of the ocular an eyelid of a patient with acquired immunode- sarcoma, non-Hodgkin lymphoma,4 tissues, involving the orbit (26 pa- ficiency syndrome. Arch Ophthalmol. 1997;115: 3 tients), eyelid (4 patients), lacrimal 1464-1466. and squamous cell carcinoma. These 5. Giles FJ, Koeffler PH. Myeloid neoplasms. In: ocular malignancies found in pa- gland (3 patients), uveal tract (2 pa- Haskell CM, ed. Cancer Treatment. 4th ed. Phila- tients with AIDS are suspected to be tients), and conjunctiva (1 patient). delphia, Pa: WB Saunders; 1995:905-921. 1-4 6. Sutton L, Guenel P, Tanguy ML, et al. Acute my- of viral origin. There is evidence In 52% of these patients, hemato- eloid leukaemia in human immunodeficiency vi- linking squamous cell carcinoma to logic evidence of leukemia was pres- rus–infected adults: epidemiology, treatment fea- human papillomavirus.3 Kaposi sar- ent at diagnosis. In 88% of patients, sibility, and outcome. Br J Haematol. 2001;112: 900-908. coma–associated human herpesvi- the ophthalmic lesion was the ini- 7. Zimmerman LE, Font RL. Ophthalmic manifes- rus 84 and Epstein-Barr virus2 have tial sign of leukemia. In a series of 121 tations of granulocytic sarcoma (myeloid sar- children from Turkey with acute my- coma or chloroma). Am J Ophthalmol. 1975;80: been detected in Kaposi sarcoma and 975-990. lymphoma tissue, respectively. It has elomonocytic leukemia (M4), 27% 8. Cavdar AO, Babacan E, Go¨szdas¸og˘lu S, et al. High been postulated that HIV may play a presented with granulocytic sar- risk subgroup of acute myelomonocytic leuke- 8 mia with orbito-ocular granulocytic sarcoma in permissive role by allowing these vi- coma in either the orbit or eyes. Turkish children. Acta Haematol. 1989;81:80-85. ruses to evade the host immune sys- The pink conjunctival tumors tem and proliferate.2 Epstein-Barr vi- present in our patient were initially rus and human herpesvirus 8 were suspicious for Kaposi sarcoma, lym- not detected in our patient’s tumor, phoma, or leukemia. All 3 malig- In Vivo Confocal however. nancies can be associated with HIV Microscopy of the Cornea According to the French- infection. The immunodeficient pa- in Nephropathic Cystinosis American-British classification of tient should be watched carefully for acute leukemia, acute myeloid leu- opportunistic infections and malig- We report a case of nephropathic kemia is classified into 9 different nancies. Appropriate diagnosis is cystinosis, with corneal crystals, im- subtypes depending on the morpho- crucial for providing proper treat- aged and analyzed by in vivo con- logical and cytochemical character- ment. Thus, in addition to Kaposi focal microscopy. The subject had istics of cells.5 These subtypes range sarcoma, non-Hodgkin lymphoma, a visual acuity of 6/6 OU. Slitlamp from M0 (acute myeloid leukemia and squamous cell carcinoma, acute biomicroscopy revealed dense, hy- without cytologic maturation) to M7 myeloid leukemia should also be rec- perreflective cysteine crystals cor- (acute megakaryocytic leukemia). ognized as a conjunctival malig- responding to a “Gahl score” of 2.75 The most commonly diagnosed sub- nancy associated with AIDS. to 3.00 in both eyes. In vivo confo- type