Thrombotic thrombocytopenic purpura in patients with retroviral infection is highly responsive to plasma infusion therapy

Abstract

Summary We prospectively studied presentation biological differences and the response to therapy in patients with thrombotic thrombocytopenic purpura (TTP) associated with, or unrelated to human immunodeficiency virus (HIV) infection. TTP patients underwent standard evaluations and were treated with prednisone 1 mg/kg in addition to infusions of fresh frozen plasma (FFP; 30 ml/kg/d) until normalization of the platelet count. Unresponsive patients were referred for plasma exchange. Compared with HIV− TTP patients (n = 23), in HIV+ subjects (n = 21) microangiopathy was dominant among Black females, who had lower presentation Hb (median 5·8 g/dl; P = 0·03), platelet count (13 × 109/l; P = 0·05) and a CD4 count of 0·096 × 109/l. HIV+ individuals responded to FFP faster than HIV− patients and none of them required apheresis. Ten HIV− TTP patients required apheresis (P = 0·03) and four died. Responses in the HIV+ and HIV− groups occurred after treatment with a median of 33 and 55 units (one unit = 320 ml) of FFP (P = 0·004) respectively. Response to this protocol was seen in 84% (95% response in HIV+ patients). Regression analysis showed that survival was associated with younger age (P = 0·001), rapid platelet (P = 0·001) and Hb (P = 0·0009) recovery, and fewer FFP units to normal lactate dehydrogenase levels (P = 0·006). We conclude that in HIV+ individuals, microangiopathy is highly responsive to plasma infusions. This observation is important particularly when apheresis is not available.

ADAMTS13 activity levels in patients with human immunodeficiency virus‐associated thrombotic microangiopathy and profound CD4 deficiency

“…The peripheral blood smear showed rare schistocytes (<1/HPF), and ADAMTS13 level was 71% at the time of presentation. Because of a report in the literature of response of HIV-infected patients with a TTP-like syndrome to plasma infusion, clinicians started the patient on a continuous plasma infusion [12]. The patient's respiratory status continued to deteriorate leading to intubation.…”

Thrombotic thrombocytopenic purpura: recognition and management

“…Fortunately, the era of highly advanced anti-retroviral therapy (HAART) has brought about a decline in incidence of HIV-TMA to well-under 1% of HIV-infected patients [18]. A high response rate is reported to plasma infusion and anti-retroviral therapy, reducing the need for plasma exchange in treating this complication [19,20]. ADAMTS13 levels are generally not decreased, supporting an alternative mechanism for TMA in these patients [16].…”

Pregnancy-associated atypical haemolytic uraemic syndrome in the postpartum period: a case report and review of the literature

“…4 Plasma exchange is not a technique that is available at all hospitals, and there is evidence to show that high-dose plasma infusion (25 -30 mL/kg/day) can, if tolerated, serve as emergency initial treatment. 13,14 Temporary dialysis is often employed to treat the accompanying renal failure. Complement activation modulators (Eculizumab and human Factor H) have been used for severe forms of aHUS, but their efficacy is yet to be established in cases of P-aHUS.…”