Ab1804

Hypergammaglobulinemic purpura of Waldenström: Report of 3 cases with a short review

A.N. Malaviya, P. Kaushik, S. Budhiraja, M. Al-Mutairi, M.R.N. Nampoory, A. Hussein, A.O. Akanji

Department of Medicine, Faculty of Medicine, Kuwait University, and Department of Medicine, Mubarak Al-Kabeer Hospital, Ministry of Health, Kuwait

ABSTRACT
Benign hypergammaglobulinemic purpura of Waldenström (HGPW) is an uncommon cause of non-thrombocytopaenic purpura that may create diagnostic difficulties. The presence of constitutional symptoms associated with prominent immunological abnormalities may raise alarm, leading to extensive and often unnecessary investigations. This report describes 3 young women with HGPW. Clinical features were characterised by recurrent episodes of bilateral asymmetrical palpable purpuric lesions on the lower extremities that were precipitated by a prolonged increase in hydrostatic pressure (e.g. prolonged standing, tight stockings etc.) associated with constitutional features. In one patient the condition was secondary to Sjögren's syndrome with type IV renal tubular acidosis. Laboratory abnormalities included a persistently elevated erythrocyte sedimentation rate, marked polyclonal hypergammaglobulinemia, and high titers of rheumatoid factor and anti-nuclear antibody of the anti-SSA(anti-Ro)/anti-SSB(anti-La) subsets.
This topic is reviewed briefly with the emphasis that in its 'primary' form this condition could be considered a `benign' systemic immunoinflammatory disease that requires neither extensive investigations nor any aggressive form of therapy. Greater awareness of HGPW may increase the frequency of its diagnosis, especially in the patient group with non-thrombocytopenic purpura or the so-called cutaneous vasculitic syndromes with `palpable purpura'.

Key words
Purpura, hypergamma-globulinemic purpura of Waldenström, vasculitis.

Please address correspondence and reprint requests to: Prof. A.N. Malaviya, Department of Medicine, Faculty of Medicine, P.O. Box 24923, Safat, Kuwait Code 13110.
E-mail: anand@hsc.kuniv.edu.kw