Clinical and laboratory profile of paroxysmal nocturnal haemoglobinuria in Kelantan - A ten year experience

Abstract

The clinical records of 11 patients diagnosed to have paroxysmal nocturnal haemoglobinuria (PNH) in Hospital USM and Hospital Kota Bharu between March 1983 and February 1994 were reviewed. Positivity in Ham's test was the major diagnostic criteria. Presenting features were similar to those in most series ie. lethargy (73%) and red urine (36%). Compared to European studies, the patients presented at a younger age (mean age 39 years, range 15-71). The complications seen included two cases of haemorrhage and one case of thrombosis (stroke) which is infrequent compared to European studies but similar to other Asian studies. The laboratory findings at presentation revealed that all the patients were anaemic (mean Hb 6.0 g/dl, range 3.3-8.9) and four patients (36%), had pancytopenia. Reticulocyte counts ranged between 0.1-30%. Bone marrow was hypocellular in four cases.