A clinico-haematologic profile of paroxysmal nocturnal haemoglobinuria.
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.
Saxena
R
R
Department of Medicine, All India Institute of Medical Sciences, New Delhi.
Malhotra
O P
OP
Saraya
A K
AK
D000293:Adolescent; D000328:Adult; D000753:Anemia, Refractory / Q000097:blood / Q000188:drug therapy; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D006397:Hematinics / Q000627:therapeutic use; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood* / Q000188:drug therapy; D006801:Humans; D007970:Leukopenia / Q000097:blood; D008297:Male; D008875:Middle Aged; D010110:Oxymetholone / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D012008:Recurrence; D013921:Thrombocytopenia / Q000097:blood
D006397:Hematinics; D011239:Prednisolone; D010110:Oxymetholone