Thrombotic thrombocytopenic purpura - analysis of clinical features, laboratory characteristics and therapeutic outcomeof 24 patients treated at a Tertiary Care Center in Saudi Arabia

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.

Section: Discussioncontrasting

confidence: 99%

Section: Discussionmentioning

confidence: 62%

Section: Discussionmentioning

confidence: 67%

Section: Discussionmentioning

confidence: 99%

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

Swart

Schapkaitz

Mahlangu

2018

“…As an initial therapy, all patients in our series received TPE with a median of seven sessions; this is less than the medians of 8 to 21 sessions reported elsewhere, with the exception of a Chinese report in which the median was 3 . The response rate achieved in our patients was slightly lower (80%) compared to studies where more TPE sessions were performed (82%‐90%), but higher than that reported in the Chinese population where fewer TPE sessions were performed (70%) .…”

Section: Discussioncontrasting

confidence: 62%

Clinical presentation in thrombotic thrombocytopenic purpura: Real‐world data from two Mexican institutions

Bugarin-Estrada

León

Lopez-Garcia

et al. 2018

Background Thrombotic thrombocytopenic purpura (TTP) is a hematologic disease that can be fatal if not treated early. We aimed to describe the clinical characteristics of Mexican patients with idiopathic TTP. Study design and methods We conducted a retrospective study, including all adult patients diagnosed with idiopathic TTP from 2011 to 2017 in two Mexican centers. We further compared our results with the published literature. Results Twenty patients were included; 70% were female, with a median age of 38.5 years at diagnosis (range 16‐63). The median time from onset of symptoms to hospital admission was 1.5 days (range 0‐16). Most patients (85%) presented with at least one systemic manifestation at admission (including fever) and 90% had neurological symptoms, most of them major (70%) including loss of consciousness, transient focal abnormalities, headache, and confusion. Only one patient (5%) had the classical pentad at the time of admission. Kidney failure was present in 25% of patients and hemorrhagic symptoms in 60%. Digestive and cardiorespiratory symptoms were less common (45% and 15%, respectively). Median platelet count and lactate dehydrogenase were 10 500/μL and 1319 IU/L, respectively. Eighty percent of patients achieved remission following treatment. Patients admitted within the first 48 hours (after the onset of symptoms) tended to have better overall survival. Conclusion Clinical presentation in Mexican TTP patients is similar to that in other countries. Early admission and a high suspicion for the disease will avoid delays in the initial work‐up and initiation of therapy, further improving prognosis.

A Saudi multicenter experience on therapeutic plasma exchange for patients with thrombotic thrombocytopenic purpura: A call for national registry

Radhwi,

Badawi,

Almarzouki

et al. 2023

BackgroundThe improvement in the clinical care for patients with thrombotic thrombocytopenic purpura (TTP) is evolving, and many efforts are being put to standardize it. Here, we aimed to assess the provided care at a national level and identify deficiencies.MethodsA national Saudi retrospective descriptive study was carried out at six tertiary referral centers and included all patients who underwent therapeutic plasma exchange (TPE) for the diagnosis of TTP between May 2005, and July 2022. Collected information included demographic data, clinical features on presentation, and the results of laboratory investigations at admission and discharge. In addition, the number of TPE sessions, days till the first session of TPE, usage of immunological agents, and clinical outcomes were all collected.ResultsOne hundred patients were enrolled, predominantly female (56%). The mean age was 36.8 years. At diagnosis, 53% of patients showed neurological involvement. The mean platelet count at presentation was 21 × 109/L. All patients had anemia (mean hematocrit 24.2%). Schistocytes were present in the peripheral blood film of all patients. The mean number of TPE rounds was 13 ± 9.3, and the mean days to start TPE since admission for the first episode was 2.5 days. ADAMTS13 level was measured in 48% of patients and was significantly low in 77% of them. Assessing for clinical TTP scores, 83%, 1000%, 64% of eligible patients had an intermediate/high PLASMIC, FRENCH, and Bentley scores, respectively. Caplacizumab was used on only one patient, and rituximab was administered to 37% of patients. A complete response for the first episode was achieved in 78% of patients. The overall mortality rate was 25%. Neither time to TPE, the use of rituximab or steroid affected survival.ConclusionsOur study shows an excellent response to TPE with a survival rate approximate to the reported international literature. We observed a deficiency in using validated scoring systems in addition to confirming the disease by ADAMTS13 testing. This emphasizes the need for a national registry to facilitate proper diagnosis and management of this rare disorder.