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In this case study, we describe the unique finding of 2 simultaneous disease processes, GCA and CLL, on TAB.
A 76-year-old male presented with a 2-month history of scalp tenderness and bitemporal pain that occurred episodically over several years. He denied any visual symptoms, jaw claudication, amaurosis fugax, diplopia, arthralgia, myalgia, or loss of appetite or weight, but confirmed occasional night sweats, which began several weeks before his visit. Relevant medical history included hypertension, cerebrovascular accident, and stable thoraco-abdominal aneurysm.
At presentation, best corrected visual acuity was 20/40 bilaterally. The afferent and efferent neuro-ophthalmic examinations were normal. Blood work revealed an elevated erythrocyte sedimentation rate of 93.0 mm/hour and a normal platelet count of 357 × 109/L. There was a high suspicion for GCA, so the patient was started on oral prednisone and underwent bilateral TAB.
Histopathologic analysis revealed no giant cells or lymphocytic infiltration within the arterial wall (Fig. 1). The absence of inflammatory activity within the artery indicated no active arteritis. However, there was intimo-medial fibrosis and loss of the internal elastic lamina, consistent with healing arteritis. Notably, some lymphocytes were noted in the tissue surrounding the artery suggesting the presence of a lymphoproliferative disorder, such as B-cell CLL or small lymphocytic lymphoma.
Hematologic testing revealed an abundance of lymphocytes, at 8.74 × 109/L, and smudge cells. Peripheral blood flow immune-phenotyping identified a population (34%) of lymphocytes that expressed CD19, CD20, HLA-DR, CD23, and CD5. The cells were negative for CD10, CD38, and cyclin D. The population was lambda light-chain restricted. Results were compatible with CLL. The patient was followed conservatively with regard to CLL, and oral prednisone was slowly tapered over several months. Continued surveillance revealed subjective and objective stability without recurrence of GCA symptoms or signs.
In the present case, the tissue surrounding the artery contained lymphocytes with surface markers consistent with CLL. There were no B cells noted within the artery despite the abundance of B cells in CLL and their tendency to diffusely seed tissue.
Thus, it would be rare to find an infiltrate composed of cells other than those usually found in GCA, which include giant cells, macrophages, and T cells. The reason for the lack of recruitment of B cells to the inflammatory infiltrate in GCA is unknown but believed to be related to cytokines, adhesion molecules, or chemoattractants.
The absence of arterial inflammatory infiltration in the present case suggested healing arteritis. However, the usual complement of plasma cells in the GCA infiltrate has interestingly been shown to be markedly reduced in the adventitia of patients with concurrent CLL.
TAB yields a diagnostic value that exceeds the diagnosis of vasculitis. The inflammatory infiltrate in GCA appears to be tightly regulated and limited to the usual cells found in most biopsy-proven GCA cases. The presence of inflammatory cells not typical of GCA should raise suspicion for an alternate diagnosis including lymphoproliferative malignancies. The present case is unique in that clinical and histopathological assessments supported the simultaneous presence of both diagnoses.
The authors have no proprietary or commercial interest in any materials discussed in this article.
Cutaneous vasculitis as the initial manifestation in acute myelomonocytic leukemia.