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

Abstract

Introduction Thrombotic thrombocytopenic purpura (TTP) is associated with high mortality if not managed timeously with therapeutic plasma exchange (TPE). TTP secondary to human immunodeficiency virus (HIV) infection is unique to sub‐Saharan Africa. The management and outcome of TTP in the era of improved access to therapy has not been described. Methods The present study describes the clinical presentation, treatment, therapeutic endpoints, and outcome of TTP patients at the Charlotte Maxeke Johannesburg Academic Hospital, South Africa. The inpatient and outpatient records of 41 consecutive adults with TTP were reviewed between 2012 and 2016. Patients were classified according to aetiology and treatment response. Results TTP was the initial presenting feature of HIV infection in 78.0%, and 12.5% were noncompliant with antiretroviral therapy (ART). Most study patients were of black ethnicity (95%) and female gender (78.1%). Treatment included initial TPE (87.8%), plasma infusion (78.1%), antiretroviral therapy (78.3%), corticosteroids (61.0%) intensive care admission (41.5%), renal dialysis (12.2%), and other immunosuppressive agents (4.9%). The median (range) number of TPEs was 10.0 (7.0‐15.0). A high rate of refractory disease (63.4%) was reported. Haemoglobin, platelet count, lactate dehydrogenase, red cell distribution width, and creatinine were reliable therapeutic end‐points (P < .05). The relapse rate was 9.8% and the mortality rate was 29.3%. Conclusion The high mortality rate emphasises the importance of early diagnosis, referral, and appropriate management of TTP. Anti‐retroviral therapy and adherence monitoring are essential to TTP management associated with HIV. Future studies to identify patients at risk for refractory disease are indicated.

Trombotic Trombocytopenic Purpura Presenting With Headache And Agitation: Case Report

“…Yapılan çalışmalarda insidansı milyon kişi başına 1 ile 13 olarak belirtilmektedir, kadınlarda daha sıkdır. Genellikle 30-40 yaşlarında daha sık görülür, [1,2] çocuklarda konjenital formlar ortaya çıkabilir. [2] TTP konjenital veya edinsel olabilir.…”

“…Genellikle 30-40 yaşlarında daha sık görülür, [1,2] çocuklarda konjenital formlar ortaya çıkabilir. [2] TTP konjenital veya edinsel olabilir. TTP, ADAMTS-13 enziminin azalması veya yokluğuna bağlı olarak gelişir.…”

“…Kadınlarda ve 30-40 yaş arasında daha sık görülür. [1,2] TTP'da hemolitik anemi, trombositopeni, hiperbilirübinemi, üremi ve artmış megakaryositik aktivite ile birlikte kemik iliğinin eritroid ve mi-yeloid hiperplazisi vardır. Laboratuvar tetkiklerinde kan sayımı, idrar tahlili, periferik kan yayması, direkt Coombs testi, laktat dehidrogenaz (LDH), protrombin zamanı (PT), parsiyel tromboplastin zamanı, fibrinojen, "a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13" (ADAMTS-13) aktivitesi, retikülosit sayımı, renal fonksiyon testleri, şistositler, direkt ve indirekt bilirubin seviyelerine bakılmalıdır.…”

“…TTP, tedavi yapılmazsa çok yüksek mortaliteye sahip tıbbi bir acil durum olduğundan erken tanı çok önemlidir. [1,2] TTP'nin standart tedavisi plazma değişimi (PD) ve kortikosteroidlerdir. Beyin bilgisayarlı tomografisi normal olarak değerlendirildi.…”

Evaluation of thrombotic thrombocytopenic purpura cases: A single-center experience

“…We believe LDH value is an important indicator of plasmapheresis response in the early term. Median plasmapheresis time was 8.5 days (range, 5-85); Swart et al [15] reported median plasmapheresis time of 10.0. Seven separate parameters including plasmic score, [8] platelet count, degree of hemolysis, presence of cancer, lack of transplantation, Mean corpuscular volume (MCV) value, international normalized ratio (INR), and creatinine allow evaluation of prognosis in patients with severe ADAMTS13 deficiency.…”