Thrombotic thrombocytopenic purpura in the presence of connective tissue disease and HIV infection: A diagnostic and therapeutic challenge in a resource- constrained setting

Abstract

Thrombotic thrombocytopenic purpura is a catastrophic disease and may occur in the presence of other confounding diseases. We present a case of TTP in a patient with connective tissue disease and HIV infection, in whom the diagnosis and management of TTP was challenging. It is important to understand the various underlying mechanisms that drive TTP in the presence of these comorbid diseases, so that an appropriate treatment strategy can be initiated. Our patient failed an initial trial of plasma infusion alone, but responded well to plasma exchange.

Thrombotic thrombocytopenic purpura: A 5‐year tertiary care centre experience

“…In contrast, to reports of TTP in other parts of the world, in this study, TTP was most commonly secondary to, or associated with, HIV infection ( n = 32/41,78.0%). This finding confirms the association of HIV in other previous South African studies . Similarly, in this study, HIV associated TTP was reported predominantly in African females with advanced HIV disease.…”

“…This finding confirms the association of HIV in other previous South African studies. 4,5,15 Similarly, in this study, HIV associated TTP was reported predominantly in African HIV induces cytokine production, namely tumour necrosis factor-α, interleukin −1, and −6, 16 as well as von Willebrand factor (VWF) and tissue factor expression, 17 which results in endothelial cell dysfunction. In HIV-associated TTP, chronic inflammation results in microthrombi production because of endothelial injury, activation of the coagulation cascade and VWF multimer release.…”

Systemic Lupus Erythematosus (SLE) and Thrombotic Thrombocytopenic Purpura (TTP) Presenting as Recurrent Strokes: A Case Report

“…TTP is a rare, potentially fatal illness that affects less than 0.5% of individuals diagnosed with SLE [ 7 , 8 ]. Although the pentad of severe thrombocytopenia, fever, neurological problems, renal insufficiency, and microangiopathic hemolytic anemia was initially described in 1924, it was not until 1966 that it was published [ 9 ].…”

“…It is postulated that individuals who have acquired TTP as a result of SLE typically have lower levels of ADAMTS13 in their blood [ 7 ]. This is because they develop autoantibodies against the enzyme, which cleaves large precursors of VWF into smaller units that have less adhesive surface area for platelets.…”

“…A high concentration of large multimeric forms of VWF accumulates in the blood due to ADAMTS13 deficiency, and these forms serve as strong adhesive surfaces for the development of platelet-rich thrombi that constrict the lumen of arterioles [ 8 , 9 ]. When red blood cells squeeze past these blood clots, they are under extreme stress, which damages their membranes and causes microangiopathic hemolysis [ 7 ].…”