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.1240
Highlights
Thrombotic Microangiopathy (TMA), where the characteristic pathogenesis is occlusive micro vascular thrombosis, clinically manifests as thrombocytopenia, microangiopathic hemolytic anemia, and variable features of organ ischemia. [1]Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are the two primary syndromes under TMA.There are several distinct pathophysiologic mechanisms responsible for these syndromes
[3] It is characterized by acute onset renal failure, thrombocytopenia, and thrombotic microangiopathic hemolytic anemia, mildly elevated liver function tests, normal coagulation profile parameters of PT, aPTT and increased serum Lactate Dehydrogenase levels
[4] This period carries profound risk for thrombotic events and other pregnancy associated syndromes like preeclampsia, eclampsia, and HELLP syndrome. These conditions may present with thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, and renal insufficiency, as a result of which it is difficult in making their distinction from HUS-TTP
Summary
Thrombotic Microangiopathy (TMA), where the characteristic pathogenesis is occlusive micro vascular thrombosis, clinically manifests as thrombocytopenia, microangiopathic hemolytic anemia, and variable features of organ ischemia. [1]Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are the two primary syndromes under TMA.There are several distinct pathophysiologic mechanisms responsible for these syndromes. Thrombotic Microangiopathy (TMA), where the characteristic pathogenesis is occlusive micro vascular thrombosis, clinically manifests as thrombocytopenia, microangiopathic hemolytic anemia, and variable features of organ ischemia. On gross examination, both kidneys were mildly enlarged and cut surface showed rim of peripheral cortical necrosis (Fig 1A). H & E stained sections from kidneys revealed glomerular capillaries and arterioles showing platelet – fibrin thrombi and fragmented RBCs without vasculitis (Fig 1B).PAS stain and MSB (Mauritus, Scarlet, Blue) stain for fibrin showed glomerular capillary thickening, fibrinoid necrosis of endothelium and fibrin thrombi in capillaries and afferent renal arterioles (Fig 2 A,B,C). Correlating clinical features, gross and microscopic findings, cause of death was given as acute renal failure with lobar pneumonia with HUS-TTP in a postpartum female
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