A clinico-haematologic profile of paroxysmal nocturnal haemoglobinuria

J Assoc Physicians India. 1991 Oct;39(10):741-3.

Comment in:
    J Assoc Physicians India. 1991 Oct;39(10):735-6.

A clinico-haematologic profile of paroxysmal nocturnal haemoglobinuria.

Saxena R, Malhotra OP, Saraya AK.

Department of Medicine, All India Institute of Medical Sciences, New Delhi.

Clinico-haematological parameters in sixteen patients of paroxysmal nocturnal
haemoglobinuria (PNH) are presented. Their modes of presentation included
recurrent episodes of cola-coloured urine (6/16), refractory anaemia (9/16) and
predominant thrombotic manifestations (1/16). Laboratory investigations revealed
the presence of anaemia (16/16), reticulocytosis (14/16), thrombocytopenia
(11/16), leucopenia (5/16) and cellular bone marrow (14/16). Two patients had
hypoplastic bone marrow initially but subsequently developed PNH. The patients
were treated with haematinics, prednisolone (16/16) and oxymethalone (2).
Prednisone was effective in suppressing haemolytic episodes. Oxymethalone given
to the 2 patients with hypoplastic bone marrow resulted in amelioration of
anaemia in one but no effect in the other patient.