Dengue Virus Infection Triggering Thrombotic Thrombocytopenic Purpura in Pregnancy

Abstract

Abstract. We report a case of thrombotic thrombocytopenic purpura (TTP) that immediately followed symptomatic dengue virus infection in a pregnant lady. The patient developed dengue fever at 16 weeks of gestation, resulting in spontaneous abortion. Subsequently, fever reappeared with persistent thrombocytopenia and jaundice. Investigations revealed microangiopathic hemolysis; there was no evidence of disseminated intravascular coagulation. The TTP episode resolved after six cycles of therapeutic plasma exchange with fresh-frozen plasma. An ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif 13 repeats) activity assay, done during convalescence, showed normal activity. The patient had an uneventful second pregnancy and has remained free of TTP recurrence for more than 2 years now. We review the pathophysiological basis of TTP in dengue infection, and suggest that jaundice with disproportionate elevation of serum aspartate aminotransferase level in a patient with dengue should arouse the suspicion of TTP.

Dengue fever a rare cause of thrombotic thrombocytopenic purpura: a case report

“…TTP is a rare and life-threatening pathophysiology which occurs as a result of congenital or acquired deficiency ADAMTS13, a metalloprotease that is important to cleave large Von Willibrand factor multimers. This leads to formation of disseminated microvascular thrombi and organ ischaemia.It is characterized by a pentad of thrombocytopenia, microangiopathic haemolytic anaemia (MAHA) , fluctuating neurological signs, renal impairment and fever [5]. Our patient manifested the classic pentad, which is observed in less than 10% of patients with TTP [3,5].…”

“…This leads to formation of disseminated microvascular thrombi and organ ischaemia.It is characterized by a pentad of thrombocytopenia, microangiopathic haemolytic anaemia (MAHA) , fluctuating neurological signs, renal impairment and fever [5]. Our patient manifested the classic pentad, which is observed in less than 10% of patients with TTP [3,5]. The ADAMTS13 assay helps to confirm the diagnosis but it is not essential.…”

“…It is important to make the diagnosis because the mortality is very high if untreated and can be reduced with the early initiation of plasma exchange [3]. The presence of TTP in dengue viral infection is rare but has been reported in the literature [4,5].…”

Viral hemorrhagic fevers due to endotheliopathy-associated disseminated intravascular microthrombosis and hepatic coagulopathy: pathogenesis based on “two activation theory of the endothelium”

“…In viral hemorrhagic fevers, endotheliopathy-associated DIT/ VMTD could trigger TTP-like syndrome [6,[28][29][30][31], which is characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia (MAHA)/atypical MAHA (aMAHA) (if schistocytes are fewer) and hypoxic organ dysfunction syndromes. Unlike true DIC in which hemostatic (coagulation) disorder occurs following tissue factor (TF) pathway activation, endotheliopathy-associated DIT/VMTD is the microthrombotic disorder occurring as a result of microthrombogenesis.…”

Pathogenesis of Ebola Viral Haemorrhagic Fever: TTP-like Syndrome Associated with Hepatic Coagulopathy based on "Two Activation Theory of the Endothelium"

“…DIT is the underlying pathological condition leading to vascular microthrombootic disease (VMTD In Ebola viral haemorrhagic fever, endotheliopathy-associated DIT/VMTD could trigger TTP-like syndrome [5,[26][27][28][29], which is characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia (MAHA)/atypical MAHA (aMAHA) (if schistocytes are fewer in number) and hypoxic organ dysfunction syndromes. Unlike DIC, in which an abnormal hemostatic (coagulation) disorder occurs following tissue factor (TF) pathway activation, endotheliopathy-associated DIT/VMTD is a pathological microthrombotic disorder occurring as a result of microthrombogenesis.…”