scholarly journals Diagnostic dilemma: Severe thrombotic microangiopathy in pregnancy

2017 ◽  
Vol 18 (4) ◽  
pp. 348-351 ◽  
Author(s):  
Sarah Birkhoelzer ◽  
Alexandra Belcher ◽  
Helen Peet
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Haemolytic Anaemia ◽  
Thrombotic Microangiopathy ◽  
Interdisciplinary Approach ◽  
Severe Thrombocytopenia ◽  
Diagnostic Dilemma ◽  
Thrombocytopenic Purpura ◽  
Microangiopathic Haemolytic Anaemia ◽  
Elevated Liver Enzymes ◽  
In Pregnancy

A diagnostic dilemma occurred when thrombotic microangiopathy developed during pregnancy. The diagnostic criteria of thrombotic microangiopathy include thrombocytopenia (platelets <100) and microangiopathic haemolytic anaemia (including thrombotic thrombocytopenic purpura and haemolytic-uraemic syndrome). An urgent interdisciplinary approach is required to treat thrombotic microangiopathy in pregnancy to differentiate between thrombotic microangiopathy and HELLP syndrome (haemolysis, elevated liver enzymes, low platelets).1 This case presented with the pentad of thrombotic thrombocytopenic purpura: severe thrombocytopenia (platelets 9 × 109/L), microangiopathic haemolytic anaemia (reticular count 245 × 109/L (20–110)), LDH >5000 U/L (<425)), neurological abnormalities (Glasgow Coma Scale 10/15), renal failure (creatinine 140 µmol/L (<97)), fever (37.7℃). A Disintegrin And Metalloproteinase with a Thrombospondin type 1 motif, member 13 (ADAMTS13) activity of less than 5% and anti-ADAMTS13 antibodies retrospectively confirmed the diagnosis of acquired idiopathic thrombotic thrombocytopenic purpura in pregnancy. The immediate management in the Emergency Department with an interdisciplinary team of Consultant Nephrologists, Intensivists, Haematologists and Obstetricians facilitated prompt diagnosis resulting in immediate plasma exchange (PEX) and coordination of semi-elective delivery of the foetus.

Pregnancy-associated thrombotic thrombocytopenic purpura

2009 ◽  
Vol 101 (02) ◽  
pp. 248-251 ◽  
Author(s):  
Jens Gerth ◽  
Ekkehard Schleussner ◽  
Karim Kentouche ◽  
Martin Busch ◽  
Mandy Seifert ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Normal Values ◽  
Successful Outcome ◽  
Severe Thrombocytopenia ◽  
Management Problem ◽  
Thrombocytopenic Purpura ◽  
Close Cooperation ◽  
Male Neonate ◽  
Plasma Infusions ◽  
In Pregnancy

SummaryThrombocytopenia during pregnancy is a common diagnostic and management problem. Several differential diagnosis must be considered including manifestations of thrombotic thrombocytopenic purpura (TTP). We report here on a case of a 21-year-old pregnant woman who presented initially severe thrombocytopenia (8 Gpt/l) in the 20th+1 week of gestation. The patient had an antibody against ADAMTS13, and enzyme activity was <5%. Immediate plasmapheresis treatment was initiated, followed by plasma infusions, and again plasmapheresis. A male neonate was delivered by caesarean section in the 32nd week of gestation. The child had an uncomplicated postnatal development. After delivery, the mother’s platelet count and ADAMTS13 activity increased to normal values. This case shows interesting aspects of TTP in pregnancy and a close cooperation between obstetricians, nephrologists and pediatricians is necessary for a successful outcome of the pregnancy.

Thrombotic, thrombocytopenic purpura (ADAMTS-13 deficiency): a rare neonatal presentation

2015 ◽  
Vol 4 (1) ◽  
Author(s):  
Rachael Sutton ◽  
Andrew Will ◽  
Minju Kuruvilla ◽  
Suresh Victor
Keyword(s):  
Cerebral Infarction ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Haemolytic Anaemia ◽  
Exchange Transfusion ◽  
Neonatal Period ◽  
Kidney Damage ◽  
Coombs Test ◽  
Thrombocytopenic Purpura ◽  
Microangiopathic Haemolytic Anaemia ◽  
Neonatal Presentation

AbstractCongenital ADAMTS-13 deficiency is rare, with only between 150 and 200 living cases described internationally. It can present in the neonatal period with thrombocytopenia that may be associated with thrombosis rather than haemorrhage, microangiopathic haemolytic anaemia (MAHA) and jaundice requiring exchange transfusion. We report a case of a large cerebral infarction resulting from severe ADAMTS-13 deficiency in the immediate neonatal period. The diagnosis of ADAMTS-13 deficiency should be considered in babies with haemolytic anaemia, jaundice, thrombocytopenia and a negative direct antiglobulin (Coombs) test (DAT). It is important to diagnose and treat early in order to prevent further brain and kidney damage.

scholarly journals A new mouse model mimicking thrombotic thrombocytopenic purpura: correction of symptoms by recombinant human ADAMTS13

Blood ◽  
2012 ◽  
Vol 119 (25) ◽  
pp. 6128-6135 ◽  
Author(s):  
Alexandra Schiviz ◽  
Kuno Wuersch ◽  
Christina Piskernik ◽  
Barbara Dietrich ◽  
Werner Hoellriegl ◽  
...  
Keyword(s):  
Platelet Aggregation ◽  
Mouse Model ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombotic Microangiopathy ◽  
Knockout Mice ◽  
Serum Lactate Dehydrogenase ◽  
Severe Thrombocytopenia ◽  
Dependent Manner ◽  
Thrombocytopenic Purpura ◽  
Induce Platelet Aggregation

Abstract Deficiency of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), a VWF-cleaving protease, is the key factor in the pathogenesis of thrombotic thrombocytopenic purpura (TTP), a life-threatening thrombotic microangiopathy. It is well established that ADAMTS13 deficiency results in elevated plasma levels of ultra-large VWF multimers (ULVWF), which are prone to induce platelet aggregation; however, the actual trigger of TTP development remains uncertain. Here we describe a new animal model in which some TTP-like symptoms can be triggered in ADAMTS13 knockout mice by challenge with 2000 units/kg body weight of recombinant human VWF containing ULVWF multimers. Animals rapidly showed clinical symptoms and developed severe thrombocytopenia. Schistocytosis, a decrease in hematocrit, and elevated serum lactate dehydrogenase levels were observed. The heart was identified as the most sensitive target organ with rapid onset of extensive platelet aggregation in the ventricles and myocardial necrosis. Prophylactic administration of 200 units/kg recombinant human ADAMTS13 protected ADAMTS13 knockout mice from developing TTP. Therapeutic administration of 320 units/kg rhADAMTS13 reduced the incidence and severity of TTP findings in a treatment interval-dependent manner. We therefore consider this newly established mouse model of thrombotic microangiopathy highly predictive for investigating the efficacy of new treatments for TTP.

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