Related Experiment Videos
Autoimmune haemolysis in childhood and adolescence
Insights
Autoimmune haemolysis in children is often acute, frequently following infections, with most recovering fully within six months. Donath-Landsteiner haemolysis and warm autoantibody-mediated haemolysis were common, requiring prompt treatment for severe cases.
Area of Science:
- Pediatric Hematology
- Immunology
- Clinical Pathology
Background:
- Autoimmune haemolysis (AIH) is a rare but serious condition in children.
- Understanding the clinico-pathological spectrum of AIH is crucial for timely diagnosis and management.
- AIH presents diverse clinical and serological features in pediatric populations.
Purpose of the Study:
- To describe the clinico-pathological features of autoimmune haemolysis in a cohort of 42 children.
- To analyze the incidence, serological classification, triggers, clinical presentation, and outcomes of pediatric AIH.
- To evaluate the effectiveness of different treatment modalities and the overall prognosis.
Main Methods:
- Retrospective analysis of clinical and laboratory data from 42 pediatric patients diagnosed with autoimmune haemolysis.
- Serological classification of AIH cases, including identification of Donath-Landsteiner antibodies and warm autoantibodies.
- Review of clinical presentation, associated triggers (infections, collagenosis), treatment strategies, and patient outcomes.
Main Results:
- AIH predominantly affected children under 5 years, with a male predominance in younger age groups.
- Donath-Landsteiner haemolysis and warm autoantibody-mediated haemolysis accounted for 79% of cases.
- Acute infections were the most common trigger; severe illness occurred in 28 patients, but 83% achieved complete recovery, often within 6 months.
Conclusions:
- Autoimmune haemolysis in children is typically acute, often triggered by infections, and generally has an optimistic prognosis, especially when not associated with chronic disorders.
- Prompt treatment with prednisolone, transfusions, and antibiotics is effective, with splenectomy reserved for refractory cases.
- Donath-Landsteiner haemolysis and warm autoantibody AIH are significant subtypes requiring specific recognition in pediatric autoimmune haemolysis.
Abstract:
The clinico-pathological features of 42 children with autoimmune haemolysis are described. Over 65% of cases were seen before their 5th birthday. In this group males predominated by the ratio of 2.5:1, but in the older children both sexes were equally affected. The incidence decreased from 1 in 188 X 10(3) in young males to 1 in 1,780 X 10(3) in children over 10. Cases were classified serologically. Of particular note was the frequency of Donath-Landsteiner haemolysis which equalled that due to warm autoantibodies; together these groups made up 79% of the total cases. Most haemolytic episodes followed an acute infection. This was frequently mild and often involved the upper respiratory tract; in only 2 patients was haemolysis associated with underlying collagenosis. Typically there was a sudden onset of pallor and malaise; jaundice, splenomegaly and hepatomegaly were found in about half the subjects. Haemoglobinuria was characteristic of Donath-Landsteiner haemolysis. The illness was severe, with Hb levels falling below 6.0 g/dl in 28 patients. Prednisolone, blood transfusion and, where indicated, antibiotics were usually effective in treating the illness, with splenectomy reserved for cases where this treatment was unsatisfactory. In several individuals no treatment was required. Recovery was rapid, and complete recovery occurred in 83% of patients, usually within 6 months. Although 2 patients died, a generally optimistic prognosis can be given, particularly in the absence of an underlying chronic disorder.