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Concurrent diagnosis of giant cell arteritis and chronic lymphocytic leukemia in a temporal artery biopsy

Published:January 14, 2017DOI:https://doi.org/10.1016/j.jcjo.2016.11.011
      Multiple reports have suggested an association between vasculitis and hematologic malignancies, but most have involved small or medium-sized vasculitides.
      • Bourantas K.
      • Malamou-Mitsi V.D.
      • Christou L.
      • Filippidou S.
      • Drosos A.A.
      Cutaneous vasculitis as the initial manifestation in acute myelomonocytic leukemia.
      • Fortin P.R.
      Vasculitides associated with malignancy.
      Concurrent malignancies have been shown to exist in 7.4% of patients with biopsy-proven giant cell arteritis (GCA), with 45% being hematologic in nature.
      • Liozon E.
      • Loustaud V.
      • Fauchais A.L.
      • et al.
      Concurrent temporal (giant cell) arteritis and malignancy: report of 20 patients with review of the literature.
      Although GCA and chronic lymphocytic leukemia (CLL) have been shown to exist concurrently, rarely has the temporal artery biopsy (TAB) led to a simultaneous diagnosis of CLL.
      • Gonzalez-Gay M.A.
      • Blanco R.
      • Gonzalez-Lopez M.A.
      Simultaneous presentation of giant cell arteritis and chronic lymphocytic leukemia.
      • Disdier P.
      • Harle J.R.
      • Costello R.
      • Figarella-Branger D.
      • Horschowski N.
      • Weiller P.J.
      Horton’s disease and chronic lymphoid leukemia.
      • Webster E.
      • Corman L.C.
      • Braylan R.C.
      Syndrome of temporal arteritis with perivascular infiltration by malignant cells in a patient with follicular small cleaved cell lymphoma.
      • Martinez-Taboada V.
      • Brack A.
      • Hunder G.G.
      • Goronzy J.J.
      • Weyand C.M.
      The inflammatory infiltrate in giant cell arteritis selects against B lymphocytes.
      In this case study, we describe the unique finding of 2 simultaneous disease processes, GCA and CLL, on TAB.

      Case Report

      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.
      Fig. 1
      Fig. 1Pathologic analysis of temporal artery biopsy. (A) Low power (250×) of noninflamed temporal artery and suspicious lymphocytic infiltrate in the included periadvential soft tissue (H&E). (B) High power (630×) of the monotonous small lymphocytic cell population (hematoxylin and eosin). (C) High power (400×) of CD 20 immunostain for B lymphocytes shows positivity in the majority of cells. (D) Medium power (100×) of temporal artery showing healed arterial injury (circle) with intimo-medial fibrosis and loss of internal elastic lamina (hematoxylin and eosin).
      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.

      Discussion

      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.
      • Martinez-Taboada V.
      • Brack A.
      • Hunder G.G.
      • Goronzy J.J.
      • Weyand C.M.
      The inflammatory infiltrate in giant cell arteritis selects against B lymphocytes.
      The inflammatory infiltrate in GCA in the context of underlying CLL has been shown to be tightly regulated.
      • Martinez-Taboada V.
      • Brack A.
      • Hunder G.G.
      • Goronzy J.J.
      • Weyand C.M.
      The inflammatory infiltrate in giant cell arteritis selects against B lymphocytes.
      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.
      • Martinez-Taboada V.
      • Brack A.
      • Hunder G.G.
      • Goronzy J.J.
      • Weyand C.M.
      The inflammatory infiltrate in giant cell arteritis selects against B lymphocytes.
      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.
      • Martinez-Taboada V.
      • Brack A.
      • Hunder G.G.
      • Goronzy J.J.
      • Weyand C.M.
      The inflammatory infiltrate in giant cell arteritis selects against B lymphocytes.
      This may be due to an overall reduction in the amount of fully matured plasma cells, as this process is interrupted in B cell leukemias.
      • Martinez-Taboada V.
      • Brack A.
      • Hunder G.G.
      • Goronzy J.J.
      • Weyand C.M.
      The inflammatory infiltrate in giant cell arteritis selects against B lymphocytes.

      Conclusion

      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.

      Disclosure

      The authors have no proprietary or commercial interest in any materials discussed in this article.

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