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Challenging Problems in Malignancy

CASE 3. Chronic Lymphocytic Leukemia With Ocular Complications and Superior Vena Cava Obstruction

Department of Ophthalmology and Visual Sciences, Department of Anatomical and Cellular Pathology, The Chinese University of Hong Kong, Shatin, Hong Kong, People's Republic of China

A 69-year-old Chinese woman presented initially in March 2000 with bilateral cervical lymphadenopathy. Excisional lymph nodes biopsy showed diffuse infiltration by small lymphoid cells with scanty cytoplasm, round nuclei, and small single nucleoli, admixed with a few medium-sized prolymphocytes effacing the normal follicular architecture (Fig 1A). Immunophenotypic studies of the lymphoid cells revealed B-cell population expressing CD5 and CD23 (Figs 1B and 1C), together with the positive L26 (CD20) immunostain, confirming chronic lymphocytic leukemia (CLL) of B-cell lineage. She refused chemotherapy and did not return to the clinic after that. Two years later, she came back with diplopia, headache, and proptosis in both eyes. General inspection revealed that she was cachexic with congested superficial facial veins extending to the neck and supraclavicular region. Marked bitemporal soft tissue fullness and exophthalmos were apparent. Multiple cervical lymph nodes were palpable. Visual acuity of both eyes was 20/30 but gross restriction of extraocular motility was present, leading to binocular diplopia in all directions of the patient's gaze. Ophthalmoscopy revealed bilateral optic disc swelling, peripapillary retinal hemorrhage, and preretinal hemorrhage (Figs 2A and 2B). Computed tomography of brain and orbit demonstrated extensive tumor infiltrations over face with mass extending into both orbits (Fig 2C; bilateral enlarged lacrimal glands, white arrow; optic discs infiltration, black arrow; intraconal infiltrative mass of the left orbit, dotted arrow). In view of the possibility of superior vena cava obstruction, computed tomography of the thorax was performed and marked mediastinal lymphadenopathy (Fig 3; dotted arrow) resulting in superior vena cava and internal jugular vein compression (Fig 3; solid arrow) were noted. Hematologic investigation showed white cell counts 104,000/µL with 85% lymphocytosis (89,400/µL), hemoglobin 10.4 g/dL, RBCs 3,410/µL, and platelet count of 163,000/µL. Flow cytometry of peripheral blood confirmed CD19+, CD20+, CD22 weakly +, and surface immunoglubulin M+ with co-expression of CD5 and CD23. She had CLL of Binet stage B disease and an indolent disease with ocular complications and superior vena cava obstruction.^1,2 The patient refused bone marrow aspiration or trephine biopsy and therapy of any form.

View larger version (176K): [in this window] [in a new window]   Fig 1.

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Authors' Disclosures of Potential Conflicts of Interest

The authors indicated no potential conflicts of interest.

REFERENCES

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2. Caligaris-Cappio F, Hamblin TJ: B-cell chronic lymphocytic leukemia: A bird of a different feather. J Clin Oncol 17:399-408, 1999[Abstract/Free Full Text]

3. Schachat AP, Markowitz JA, Guyer DR, et al: Ophthalmic manifestations of leukemia. Arch Ophthalmol 107:697-700, 1989[Abstract/Free Full Text]

4. Hatton MP, Rubin PAD, Mass B: Chronic lymphocytic leukemia of the orbit. Arch Ophthalmol 120:990-991, 2002[Free Full Text]

5. Schmid-Wendtner MH, Sander C, Volkenandt M, et al: Unusual manifestations of B-cell disorders. Case 2: chronic lymphocytic leukemia presenting with cutaneous lesions. J Clin Oncol 17:1084-1085, 1999

6. Buchan J, McKibbin M, Burton T: The prevalence of ocular disease in chronic lymphocytic leukemia. Eye 17:27-30, 2003[Medline]

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This Article Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Liu, D. T.L. Articles by Lam, D. S.C. Search for Related Content PubMed PubMed Citation Articles by Liu, D. T.L. Articles by Lam, D. S.C. Social Bookmarking                            What's this?