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G490(P) Coombs positive autoimmune haemolytic anaemia complicating post streptococcal glomerulonephritis. (a very rare occurrence)
  1. R Sesham1,
  2. E Lewin1,
  3. B Enchill2,
  4. J Evans2
  1. 1Paediatrics, James Paget University Hospital, Great Yarmouth, UK
  2. 2Paediatric Nephrology, Nottingham University Hospitals, Nottingham, UK

Abstract

Introduction Post streptococcal glomerulonephritis (PSGN) is the most common type of glomerulonephritis in children. Anaemia is one of its complications and is commonly secondary to fluid retention.

We describe a very rare occurrence of Autoimmune Haemolytic Anaemia (AIHA) in a 10 year old girl who presented with post streptococcal glomerulonephritis.

This case serves to alert the clinician about this rarely reported association.

Case report A 10 year girl was admitted with a 12 day history of diarrhoea and vomiting. She had been treated for pustular tonsillitis 6 weeks prior. Cola coloured urine was noted at presentation. She has no significant past medical history.

She was oliguric, pale, oedematous, hypertensive with haematuria. Initial investigations showed a mixed nephritic/nephrotic picture with direct coomb’s test (DAT) positive haemolytic anaemia with spherocytes on the blood film.

Her DAT was negative for anti-gG, but positive anti-C3b-C3d, indicating that the auto–antibodies were IgM in nature4.

She was transfused in view of symptomatic anaemia (Hb: 60 grams/L) and commenced on antihypertensive medication prior to her transfer to a tertiary renal unit.

Her renal biopsy showed a crescentic glomerulonephritis with the characteristic features of acute phase, PSGN along with acute tubular injury. She had an increased ASO titre and antiDNAase level. Her initial C3 was low, with normal C4 confirming PSGN. Her renal function improved on dialysis.

Though the acute kidney injury has improved, she continues to have proteniuria, and is on antihypertensive medication. However, her haemolytic anaemia has resolved completely.

Discussion The combination of AIHA and PSGN is very rare, to our knowledge this might be the first reported case in UK A cross reaction between antibodies induced by streptococcal infection against the red blood cells has been described as a possible mechanism. Both streptolysin O antigen and NAD–glycohydrolase (NADase) may play a role in causing haemolysis.

Intravascular haemolysis resulting in haemoglobinuria could aggravate the acute kidney injury, hence the importance of recognising this association.

Our patient’s haemolytic anaemia improved along with her renal recovery.

Conclusion In children presenting with severe anaemia in the context of PSGN, the possibility of AIHA must be considered.

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