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Induction of thrombotic thrombocytopenic purpura by dengue virus infection in a critical patient: Role of therapeutic plasma exchange

1 Department of Transfusion Medicine, Apollo Gleneagles Hospitals, Kolkata, West Bengal, India
2 Department of Clinical Hematology, Apollo Gleneagles Hospitals, Kolkata, West Bengal, India

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Date of Submission12-May-2022
Date of Decision22-May-2022
Date of Acceptance29-May-2022
Date of Web Publication26-Sep-2022


Thrombotic thrombocytopenic purpura (TTP) secondary to dengue fever is a very unusual occurrence. Both conditions are fatal and can result in significant mortality and morbidity if left untreated. In this case, we present a young lady who suffered dengue fever followed by microangiopathic anemia, thrombocytopenic purpura, and altered sensorium. Investigations revealed microangiopathic hemolysis; there was no evidence of disseminated intravascular coagulation. As soon as, we diagnosed the patient as having TTP, we treated her with serial plasma exchange therapy, steroids, and monoclonal antibodies such as rituximab. The patient responded very well to the treatment and completely recovered from neurological symptoms and laboratory parameters also normalized. Hence, timely diagnosis and starting appropriate treatment immediately are key factors for successful outcomes.

Keywords: Dengue, therapeutic plasma exchanges, thrombotic thrombocytopenic purpura

How to cite this URL:
Biswas RN, Das SS, Chakrapani A, Sen S. Induction of thrombotic thrombocytopenic purpura by dengue virus infection in a critical patient: Role of therapeutic plasma exchange. Asian J Transfus Sci [Epub ahead of print] [cited 2022 Dec 4]. Available from:

   Introduction Top

Thrombotic thrombocytopenic purpura (TTP) is a rare disorder. It has significant morbidity and mortality if left untreated. Serial therapeutic plasma exchanges (TPEs) have been found to reduce morbidity and mortality significantly.[1] Usually, it is idiopathic or autoimmune, but several predisposing factors have been described to cause secondary TTP. In this case report, we discussed a very rare presentation of TTP secondary to dengue virus infection and how timely diagnosis along with appropriate management helped us to cure the patient.

   Case Report Top

A 21-year-old female with no other comorbidities was admitted to the hospital with complaints of increasing menstrual bleeding, fever and generalized weakness, myalgia of the lower limb, and retro-orbital pain for the past 6 days. Investigations revealed hemoglobin (Hb) of 5.7 g/L, platelet count of 30 × 109/L, and positive dengue IgM antibodies. She was symptomatically treated at a local hospital and was transfused six units of random donor platelets and one unit of packed red blood cells. In spite of this, her platelet count remained low and deteriorated from 2nd day of hospitalization. She was then referred to our hospital. On admission, she was having fever, drowsiness, tachycardia, and tachypnea and was unresponsive. A repeat laboratory investigations revealed anemia, thrombocytopenia, leukocytosis, and increased lactate dehydrogenase (LDH) level [Table 1]. Creatinine and prothrombin time were within normal limits. Peripheral blood smear revealed numerous schistocytes more than 1%. Antinuclear antibody, direct Coomb's test, and red cell antibody screening were negative. There were features of thrombocytopenia, microangiopathic anemia, fever, and altered sensorium without any evidence of disseminated intravascular coagulation strongly suggested a diagnosis of TTP and we planned urgent TPE. On the 2nd day of the admission, she had an episode of convulsion which was treated with anticonvulsive medication. Magnetic resonance imaging of the brain revealed no significant abnormality. We started empirically intravenous (IV) antibiotic and low-dose IV methylprednisolone considering its autoimmune etiology. TPE was started promptly after diagnosis on a daily basis with approximately 1–1.5 volume plasma removal using an automated cell separator. Her general condition including hemolysis, fever, and sensorium started improving after the 4th TPE procedure. A total of consecutive eight TPE procedures were performed using cryopoor plasma and human albumin as replacement fluids. Her Hb recovered to 10.7 g/L with increased platelet count of 121 × 109/L [Table 1]. The patient responded well to the plasma exchange therapy with complete recovery of symptoms. She was discharged on day 11 of hospitalization and was on complete remission after 12 months of follow-up.
Table 1: Therapeutic plasma exchange and laboratory data

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   Discussion Top

Dengue is one of the most rapidly spreading mosquito-borne viral diseases in the tropical world. Dengue infection is often associated with rare severe complications such as dengue hemorrhagic fever and dengue shock syndrome. Secondary TTP to human immunodeficiency virus infection has been reported by previous authors.[2] Viral serology was nonreactive which may cause secondary TTP. Rossi et al. observed autoantibodies against (a disintegrin-like and metalloprotease with a thrombospondin type 1 motif 13) ADAMTS13 in the acute phase of dengue infection.[1] Increased levels of von Willebrand factor (vWF) including the presence of abnormal vWF multimers and decreased levels of ADAMTS13 are described in severe dengue infection.[3],[4] Dengue-induced TTP is very rare and only few authors have reported the condition.[1],[3],[4],[5] Our patient was detected dengue IgM in enzyme-linked immunosorbent assay. During the acute phase of dengue infection, our patient developed thrombotic microangiopathic hemolysis, thrombocytopenic purpura, and altered sensorium. Clinical and laboratorial data suggested a case of TTP. Here, we observed derange of liver enzyme and total bilirubin which may not be directly associated with TTP but dengue infection may increase the enzyme. The patient developed sepsis which may be the cause of elevated serum bilirubin. TTP clinical features with high serum bilirubin may correlate with leptospirosis but we found a negative solid-phase assay of leptospira IgM antibody. Hemolytic uremic syndrome is usually present in children but in this case there was no history of diarrhea or gastrointestinal Shiga toxin-producing bacterial infection and she had normal renal function (Serum creatinine<1mg/dl). She responded well in TPE therapy and increased platelet, Hb, and decreased LDH. Plasma exchange and improving laboratory data are depicted in [Figure 1]. We used low-level vWF containing cryopoor plasma for replacement fluid during TPE. TPE is a type I indication in TTP and our patient dramatically recovered after plasma exchange. Responding to TPE therapy itself strongly favored TTP diagnosis along with clinical and laboratory investigation. Our drawback was not doing ADAMTS13 level during the acute phase due to resource-poor setup. During follow-up, we did ADAMTS13 gene analysis for academic interest. No pathogenic or likely pathogenic variants causative of the reported phenotype was detected in ADAMTS13 gene analysis.
Figure 1: Improvement of laboratory values with serial therapeutic plasma exchanges

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In conclusion, special attention must be given to dengue infection patients presenting signs and symptoms of thrombotic microangiopathic anemia in future studies, to provide appropriate early investigation, diagnosis, and TPE.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Rossi FC, Angerami RN, de Paula EV, Orsi FL, Shang D, del Guercio VM, et al. A novel association of acquired ADAMTS13 inhibitor and acute dengue virus infection. Transfusion 2010;50:208-12.  Back to cited text no. 1
Gunther K, Garizio D, Nesara P. ADAMTS13 activity and the presence of acquired inhibitors in human immunodeficiency virus-related thrombotic thrombocytopenic purpura. Transfusion 2007;47:1710-6.  Back to cited text no. 2
Djamiatun K, van der Ven AJ, de Groot PG, Faradz SM, Hapsari D, Dolmans WM, et al. Severe dengue is associated with consumption of von Willebrand factor and its cleaving enzyme ADAMTS-13. PLoS Negl Trop Dis 2012;6:e1628.  Back to cited text no. 3
Basuki PS. A glance at the von Willebrand factor in dengue virus infection. Southeast Asian J Trop Med Public Health 2003;34:559-63.  Back to cited text no. 4
Gavali AS, Shelgaonkar J, Bartakke S. Thrombotic thrombocytopenic purpura in a case of dengue fever: A rare presentation. Indian J Crit Care Med 2017;21:226-8.  Back to cited text no. 5
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Correspondence Address:
Rathindra Nath Biswas,
Department of Transfusion Medicine, Chittaranjan National Cancer Institute, Newtown, Kolkata - 700 156, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ajts.AJTS_56_22


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