Paroxysmal cold hemoglobinuria in a 7-year-old male child – Was it really cold induced? A diagnostic and management dilemma

摘要

Paroxysmal cold haemoglobinuria (PCH) is a rare acquired cause of intravascular haemolysis in children. We are reporting an unusual case of PCH, a 7-year-old boy who presented with acute febrile illness, severe anaemia and passage of red colored urine. His initial haemolytic workup had shown positive direct agglutination test along with evidence of ongoing haemolysis with severe leukocytosis. Further evaluation for autoimmune haemolytic anaemia revealed positivity for Donath Landsteiner Antibody, hence establishing a diagnosis of PCH. During the hospital stay, the child required multiple red blood cells, crossmatched although “P” antigen untyped transfusions. Child gradually improved and became transfusion independent within one week. The child has been under regular follow-up since last six months and all his laboratory parameters are within normal limits.