Postpartum TTP-HUS Syndrome: A Rare Autopsy Case Report in a Tertiary Care Hospital.
Keywords:
Postpartum, TTP-HUS, autopsy, platelet-fibrin thrombi
Abstract
Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are the two main disorders included under Thrombotic Microangiopathy. 1 in 25,000 pregnancies present with these rare disorders, mostly seen after uncomplicated gestation and delivery. A diagnostic pentad for TTP was given by Amorosi & Ultmann in 1966: thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms and signs, renal functional abnormalities and fever without other explanation. HUS has features of TTP along with acute renal failure.12%-31% of TTP patients are women during pregnancy or postpartum period.Pathological diagnosis requires hyaline thrombi in terminal arterioles andcapillaries. We present a case of a 28 years old primigravida female, with 38 weeks of gestation, who was apparently alright earlier and presented with prolonged second stage of labour. However, day 2 postpartum she developed fever, breathlessness, loose motions and vomiting. She also developed pallor, icterus and petechial haemorrhages.Laboratory investigations revealed low platelet count, elevated bilirubin (direct more than indirect), elevated serum creatinine, and mildly raised hepatic transaminases. Day 5, she developed anuria and grade 3 dyspnoea and succumbed to death. Complete autopsy was performed .Histopathology on sections from kidneys revealed glomerular capillaries and arterioles showing platelet-fibrin thrombi and diffuse thickening of glomerular capillary wall with double contour of glomerular basement membrane. Lungs showed lobar pneumonia. Cause of death given was acute renal failure with lobar pneumonia with HUS-TTP in a postpartum female. TTP-HUS is a rare disorder and although some clinical features may suggest diagnosis, histopathological examination of renal specimen and applying special stains like PAS stain, silver stain and MSB (Mauritus,Scarlet,Blue) stain for fibrin, confirms the diagnosis. DOI: 10.21276/APALM.1240References
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2 ) Fakhouri F, Roumenina L, Provot F, Sallee M, Caillard S, Couzi L et al. Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations. J Am Soc Nephrol. 2010; 21(5): 859–867.
3) George JN. The association of pregnancy with thrombotic thrombocytopenic purpura- hemolytic uremic syndrome. Curr Opin Hematol 2003, 10: 339–344.
4) Zheng XL. Structure-Function and regulation of ADAMTS13 protease. J Thromb Haemost. 2013; 11(01): 11-23.
5) Machado S, Figueiredo N, Borges A, Pais MS. Acute kidney injury in pregnancy: a clinical challenge. J Nephrol 2012; 25(01):19-30.
6) Furlan M, Lammle B. Heamolytic-uraemic syndrome and thrombotic thrombocytopenic purpura-new insights into underlying biochemical mechanisms. Nephrol Dial Transplant 2000; 15: 1112-1114.
Published
2017-05-29
Issue
Section
Case Report
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