Human immunodeficiency virus associated thrombotic thrombocytopenic purpura (TTP) - favourable outcome with plasma exchange and prompt initiation of Highly Active Antiretroviral Therapy (HAART).

Daniel Patrick Hart (1) , Ruth Sayer (2) , Rob F Miller (3) , Simon G Edwards (4) , Anne Kelly (5) , Trevor Baglin (6) , Beverley J Hunt (7) , Sylvia Benjamin (8) , Raj Patel (9) , Sam Machin (10) , Maria Scully (11)

1 Haematology
2 Mortimer Market Clinic
3 Centre for Sexual Health and HIV Research, Department of Population Sciences and Primary Care
4 Department of Genitourinary Medicine
5 Haematology
6 Haematology
7 Dept of Haematology
8 Haematology
9 Haematology
10 Haematology
11 Haematology

Background: Thrombotic thrombocytopenic Purpura is an acute prothrombotic disorder. HIV is an identified precipitant. Design and methods Thirty episodes of HIV associated TTP in 24 patients from the South-East England Apheresis units, over past 10 years, are reviewed. Results: All patients were heterosexual Black Africans. First presentation of TTP revealed a new diagnosis of HIV in 8 patients. TTP relapse occurred on 6 occasions (in 4 patients) as a result of non-adherence to HAART. Prompt initiation/re-initiation of HAART in parallel with PEX +/- steroid led to prompt remission. Adjunct immunomodulatory agents (e.g.Rituximab) was required in 10% of cases. Once-daily HAART regimens are recommended, being compatible with PEX requirement, maximising drug exposure between PEX. High viral loads (>500,000copies/ml) require more PEX to remission. ADAMTS 13 activity was reduced (<5%) as detected by CBA and Anti-ADAMTS 13 IgG antibodies raised in 80%. Continued HAART-adherence ensured a durable TTP remission with associated viral control resulted in no evidence of relapse. Conclusion: PEX and HAART are associated with replenishment of ADAMTS 13 and viral suppression. More PEX is required in cases with higher viral loads. Continued HAART maintains remission. In a small proportion of cases, further immunomodulatory therapy may be required.

Mots clés: Medicine

Daniel Patrick Hart, Ruth Sayer, Rob F Miller, Simon G Edwards, Anne Kelly, et al.. Human immunodeficiency virus associated thrombotic thrombocytopenic purpura (TTP) - favourable outcome with plasma exchange and prompt initiation of Highly Active Antiretroviral Therapy (HAART).. British Journal of Haematology, 2011, 153 (4), pp.515. ⟨10.1111/j.1365-2141.2011.08636.x⟩. ⟨hal-00621304⟩