Hemorrhagic Stroke in an Adolescent Female with HIV-Associated Thrombotic Thrombocytopenic Purpura

Abstract

HIV-1 infection can trigger acute episodes of Idiopathic Thrombocytoponic Purpura (ITP), and Thrombotic Thrombocytopenic Purpura (TTP), particularly in populations with advanced disease and poor adherence to antiretroviral therapy (ART). These diseases should be distinguished because they respond to different treatments. Previous studies done in adults with HIV-TTP have recommended the prompt initiation or re-initiation of ART in parallel with plasma exchange therapy to improve the clinical outcome of these patients. Here, we describe a case of HIV-TTP resulting in an acute hemorrhagic stroke in a 16 year old female with perinatally acquired HIV infection and non-adherence to ART, who presented with severe thrombocytopenia, microangiopathic hemolytic anemia, and a past medical history of HIV-ITP. Both differential diagnosis and treatments for HIV-ITP and HIV-TTP were considered simultaneously. A decrease in plasma ADAMTS13 activity (<5%) without detectable inhibitory antibodies confirmed the diagnosis of HIV-TTP. Re-initiation of ART and plasma exchange resulted in a marked decrease in the HIV-RNA viral load, recovery of the platelet count, and complete recovery was achieved with sustained virologic suppression.

Cerebrovascular disease in children with HIV‐1 infection

“…Coagulopathies which occur in some children with HIV infection include deficiencies of protein S and protein C, immune thrombocytopenia, and thrombotic thrombocytopenia . Systemic HIV infection is thought to trigger consumptive coagulopathies and immune mediated mechanisms …”

“…Stroke due to vasculopathy may be the initial presentation of HIV infection in children . The age of vasculopathy presentation in children with HIV infection may vary from early infancy to late adolescence . Cerebrovascular complications in children with HIV infection are more common in those who acquired their HIV infection during the perinatal period, thus prophylactic ART administered to pregnant females infected with HIV may provide a line of defence against HIV infection and cerebrovascular complications in their offspring …”

“…For haemorrhagic stroke, clotting profile is needed . For suspected thromboembolic events, coagulation screen should include protein S, protein C, antithrombin III, factor V Leiden, antiphospholipid antibodies, and anticardiolipid antibodies …”

“…Twenty-seven case reports and series (including four autopsy studies) and four larger patient cohorts 2,[36][37][38] (inclusive of children with cerebrovascular disease) were identified. In total, there were 74 cases of cerebrovascular disease in children infected with HIV reported to date from North and Central America (USA, Mexico), [9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25]37,38 South America (Columbia, Brazil), [26][27][28][29] Europe (Netherlands, France, Germany), [30][31][32] Africa (South Africa, Ethiopia), 2,36 and Asia (India, Thailand). [33][34][35] Most reports were from the USA and reported before 2002.…”

“…Opportunistic vasculitis 38,41,48 Varicella zoster virus Cytomegalovirus Mycobacterium Candida albicans Crypotococcus Treponema Malignancies 11,21,41,48 Primary CNS lymphoma Secondary tumours Coagulopathies 9,21,33,38 Protein S deficiency Protein C deficiency Thrombocytopenia Cardioembolism 11,39,43 Bacterial endocarditis Marantic endocarditis HIV-associated cardiac dysfunction Antiretroviral drugs 43,51 Protease inhibitors a In the direct mechanism, the vasculopathy is due to vessel wall invasion by the HIV. In the indirect mechanism, the vasculopathy is due to autoimmune/inflammation within the vessel wall secondary to a systemic HIV infection.…”