Uncommon association of hypertriglyceridemia and autoimmune haemolytic anaemia in an infant with transfusion dependent thalassemia

Hypertriglyceridemia thalassemia syndrome (HTS) is not a well understood entity, and is mainly identified in the Indian subcontinent. We present a unique case of this syndrome along with severe autoimmune haemolysis that presented with life-threatening anaemia. An infant with transfusion dependent t...

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Bibliographic Details
Published in:Pediatric hematology oncology journal 2021-06, Vol.6 (2), p.110-112
Main Authors: Thatikonda, Kasi Bharathi, Kalra, Manas, Sachdeva, Pallavi, Ranjan, Vivek, Sachdeva, Anupam
Format: Article
Language:English
Subjects:
Autoimmune hemolytic anemia
Hypertriglyceridemia
Transfusion dependent thalassemia
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Summary:Hypertriglyceridemia thalassemia syndrome (HTS) is not a well understood entity, and is mainly identified in the Indian subcontinent. We present a unique case of this syndrome along with severe autoimmune haemolysis that presented with life-threatening anaemia. An infant with transfusion dependent thalassemia (TDT) presented with suspected mismatched blood transfusion leading to severe haemolysis. Severe haemolysis, lipemic serum were initial findings. Detailed evaluation revealed IgG, C3d positive mixed autoimmune haemolytic anaemia (AIHA), severe hypertriglyceridemia that improved with steroids and least mismatched packed red cell transfusion. Children with thalassemia presenting with excessive hemolysis may have mismatched transfusion reaction, alloimmunization or an autoimmune hemolysis. Baseline extended blood grouping, detailed immune hemolytic work-up aids in prompt diagnosis. HTS on a background of autoimmune hemolysis is rare and needs urgent medical support.
ISSN:2468-1245
2468-1245
DOI:10.1016/j.phoj.2021.01.003