Abstract
Polyarteritis nodosa (PAN) is not commonly associated with hematologic abnormalities. We report the first case of pancytopenia as the presenting symptom of PAN. There was no associated malignancy or reactive hemophagocytic syndrome (RHS) to explain the findings. The patient, after successful treatment of PAN, subsequently developed RHS a few years later. He is the fourth reported case of RHS in a patient with PAN. In addition to describing the patient’s presentation and clinical course, a systematic review of the literature was performed examining the association between hematologic conditions, including RHS, and PAN.
Similar content being viewed by others
References
Watts RA, Lane SE, Bentham G, Scott DG (2000) Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum 43(2):414–419
Hunder G (1996) Vasculitis: diagnosis and therapy. Am J Med 100(2A):37S–45S
Segelmark M, Selga D (2007) The challenge of managing patients with polyarteritis nodosa. Curr Opin Rheumatol 19(1):33–38
Fujisaki K, Masutani K, Yoshimitsu T et al (2005) Thrombotic thrombocytopenic purpura associated with polyarteritis nodosa. Clin Nephrol 64(4):305–310
Camos M, Arellano-Rodrigo E, Abello D et al (2003) Idiopathic myelofibrosis associated with classic polyarteritis nodosa. Leuk Lymphoma 44(3):539–541
Bordin G, Ballare M, Paglino S et al (1996) Idiopathic CD4+ lymphocytopenia and systemic vasculitis. J Intern Med 240(1):37–41
Hamidou MA, Boumalassa A, Larroche C, El Kouri D, Bletry O, Grolleau JY (2001) Systemic medium-sized vessel vasculitis associated with chronic myelomonocytic leukemia. Semin Arthritis Rheum 31(2):119–126
Hasler P, Kistler H, Gerber H (1995) Vasculitides in hairy cell leukemia. Semin Arthritis Rheum 25(2):134–142
Leung AC, McLay A, Boulton-Jones JM (1986) Polyarteritis nodosa and monocytic leukaemia. Postgrad Med J 62(723):35–37
Dhote R, Simon J, Papo T et al (2003) Reactive hemophagocytic syndrome in adult systemic disease: report of twenty-six cases and literature review. Arthritis Rheum 49(5):633–639
Hayakawa I, Shirasaki F, Ikeda H et al (2006) Reactive hemophagocytic syndrome in a patient with polyarteritis nodosa associated with Epstein-Barr virus reactivation. Rheumatol Int 26(6):573–576
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Harrold, L.R., Liu, N.Y.N. Polyarteritis nodosa presenting as pancytopenia: case report and review of the literature. Rheumatol Int 28, 1049–1051 (2008). https://doi.org/10.1007/s00296-008-0572-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00296-008-0572-1