thrombocytopenic purpura

When de-novo immune-mediated thrombotic thrombocytopenic purpura (TTP) is diagnosed following an invasive procedure, clinical presentation patterns and outcomes are poorly defined. Therefore, in a systematic literature review of patients diagnosed with TTP following an invasive surgical or non-surgical procedure, we identified 19 studies reporting data on 25 patients. These data suggest that 1) TTP pathogenesis likely begins prior to the invasive procedure, 2) patients experience significant diagnostic delays, and 3) there is a high incidence of renal replacement therapy...

Medical professionals, particularly in regions with a high burden of human immunodeficiency virus (HIV), should be alert to the hematological complications of HIV, which may include cytopenias, malignancy, and coagulation disturbances. Patients may present with these conditions as the first manifestation of HIV infection. Hematological abnormalities are often multifactorial with opportunistic infections, drugs, malignancy, and HIV infection itself contributing to the clinical presentation, and the diagnosis should consider all these factors...

Thrombotic thrombocytopenic purpura (TTP), a rare but fatal disease if untreated, is due to alteration in Von Willebrand factor cleavage resulting in capillary microthrombi formation and ischemic organ damage. Interleukin-1 (IL-1), has been shown to drive sterile inflammation following ischemia and could play an essential contribution to post-ischemic organ damage in TTP. Our objectives were to evaluate IL-1 involvement during TTP and to test the efficacy of the recombinant IL-1 receptor antagonist, anakinra, in a murine TTP model...

Chronic myelomonocytic leukemia (CMML) presents as a complex hematologic malignancy with myelodysplastic and myeloproliferative features. Our case report explores the rare coexistence of CMML with immune thrombocytopenic purpura (ITP) in a 63-year-old female patient. CMML diagnosis followed World Health Organization criteria, and the patient was classified as having high-risk myelodysplastic syndrome (MDS)-CMML stage 2. Initial treatment with subcutaneous azacytidine for CMML proved partially effective, highlighting persistent severe thrombocytopenia...

We present the first published case of simultaneous pneumonitis and immune thrombocytopenic purpura secondary to primary cytomegalovirus (CMV) infection in an immunocompetent patient. Treatment with oral valganciclovir for 2 weeks successfully led to complete clinical recovery. CMV is traditionally associated with infection in immunocompromised patients and neonates; however, evidence of severe CMV infections in immunocompetent hosts is emerging. It is important to highlight the broad range of clinical presentations of CMV infections to prevent diagnostic delay and associated morbidity and expense...

Background: The therapeutic management of immune-mediated thrombotic thrombocytopenic purpura (iTTP) has recently benefited from the introduction of caplacizumab, an agent directed at the inhibition of platelet aggregation. This real-world analysis investigated the epidemiology and the demographic and clinical characteristics of iTTP patients in Italy before and after caplacizumab introduction in 2020. Methods: Hospitalized adults with iTTP were included using the administrative databases of healthcare entities covering 17 million residents...

Cobalamin-deficient thrombotic microangiopathy or vitamin B12 deficiency presenting as pseudo-thrombotic microangiopathy is a rare disorder that can be misdiagnosed as thrombotic thrombocytopenic purpura. Patients with this condition are at risk of receiving unnecessary plasmapheresis with a potential delay in appropriate therapy with vitamin B12 supplementation. There are no established diagnostic criteria for this condition in clinical practice. We performed a systematic review of case reports published between January 2018 and January 2023 to analyze the clinical characteristics, risk factors, and patterns of laboratory markers to improve the diagnostic criteria for this condition...

A 27-year-old woman was diagnosed with idiopathic thrombocytopenic purpura in the neonatal period, and was admitted to our hospital after presenting with impaired consciousness, purpura, nausea and vomiting, with a platelet count of 10×109 /l. Congenital thrombotic thrombocytopenic purpura (cTTP) was suspected on the basis of recurrent thrombocytopenia and impaired consciousness, so tests for ADAMTS13 activity and inhibitor were performed. ADAMTS13 activity was severely decreased, ADAMTS13 inhibitor was negative, and platelet count increased after transfusion of fresh frozen plasma...

BACKGROUND: Laboratory diagnosis of immune-mediated thrombotic thrombocytopenic purpura (iTTP) remains challenging when ADAMTS13 activity ranges between 10-20%. To prevent misdiagnosis, open ADAMTS13 conformation gained clinical attention as a novel biomarker especially to diagnose acute iTTP in patient with diagnostic undecisive ADAMTS13 activity. Plasma ADAMTS13 conformation analysis corrects for ADAMTS13 antigen with both parameters being characterized in enzyme-linked immunosorbent assay (ELISA)-based reference assays requiring expert technicians...

This case study reports a patient with Myosin Heavy Chain 9 (MYH9)-related disorder (MYH9-RD) which is characterized by congenital macrothrombocytopenia, Döhle-like bodies, sensorineural hearing loss, cataracts, and glomerulopathy. Often misdiagnosed as idiopathic thrombocytopenic purpura (ITP), MYH9-RD requires accurate identification to avoid inappropriate treatments like steroids, rituximab, or splenectomy. Platelet transfusions were traditionally the only therapeutic option, but thrombopoietin receptor agonists (TPO-RA), specifically eltrombopag, have shown success in MYH9-RD treatment...

OBJECTIVES: Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening hematological disorder. Early differentiation between TTP and primary immune thrombocytopenia (ITP) accompanied by anemia is crucial to initiate an appropriate therapeutic strategy. The objective of this study was to evaluate the predictive value of red blood cell lifespan (RBCLS), determined using the carbon monoxide breath test, in the differential diagnosis of these two diseases. METHODS: We conducted a retrospective analysis of 23 patients with TTP and 32 patients with ITP accompanied by anemia...

Thrombotic thrombocytopenic purpura (TTP) is an uncommon, but potentially disabling or even deadly, thrombotic microangiopathy with a well-studied mechanism of ADAMTS13 deficiency or dysfunction. While established treatments are largely effective, the standard ADAMTS13 testing required to definitively diagnose TTP may cause delays in diagnosis and treatment, highlighting the need for rapid and effective diagnostic methods. Additionally, the heterogeneous presentation and varied inciting events of TTP suggest more variation in its mechanism than previously thought, implying three potential pathways rather than the accepted two...

Altered mental status (AMS) is a common condition encountered in daily practice. Finding the cause is essential for treatment, but sometimes this may be challenging. Spontaneous coronary artery dissection (SCAD) is frequently underdiagnosed and is a potentially fatal cause of acute coronary syndrome. Clinical presentation depends on the extent of SCAD, ranging from unstable angina to sudden death. AMS has not been reported with this condition, but it may be possible in hypoperfusion states. Thrombotic thrombocytopenic purpura (TTP) is part of the microangiopathic hemolytic anemia (MAHA) spectrum, presenting with AMS as the cardinal symptom...

European real-world data indicate that front-line treatment with caplacizumab is associated with improved clinical outcomes compared with delayed caplacizumab treatment. The objective of the study was to describe the characteristics, treatment patterns, and outcomes in hospitalized patients with an immune-mediated thrombotic thrombocytopenic purpura (iTTP) episode treated with front-line versus delayed caplacizumab in the US. This retrospective cohort analysis of a US hospital database included adult patients (≥18 years) with an acute iTTP episode (a diagnosis of thrombotic microangiopathy and ≥1 therapeutic plasma exchange [TPE] procedure) from January 21, 2019, to February 28, 2021...

BACKGROUND: Cancer-related thrombotic microangiopathy (CR-TMA) is a rare type of Coombs-negative hemolytic anemia, which is caused by malignancy and has a poor prognosis. CASE: A 76-year-old female was referred to our hospital due to Coombs-negative hemolytic anemia, which was causing fatigue and dyspnea on exertion, accompanied by schistocytosis. A bone marrow examination demonstrated bone marrow carcinomatosis, and the tumor cells were morphologically suspected to be signet-ring cell carcinoma cells...

Neutrophil NETosis is a unique form of cell death, characterized by release of decondensed chromatin and anti-microbial contents to the extracellular space, involved in infection, inflammation, and thrombosis. However, the role of NETosis in pathogenesis of immune-mediated thrombotic thrombocytopenic purpura (iTTP) and how a targeted therapy affects the accumulation of neutrophil extracellular traps (NETs) under flow remain to be determined. Flow cytometry demonstrated that the percentage of neutrophils undergoing NETosis in whole blood from iTTP patients on admission is significantly increased with a concurrent decrease in the capacity of inducible NETosis by shigatoxin...

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening blood disorder with a mortality rate of over 90% if left untreated, multiple long-term complications for survivors, and a lifelong risk of relapse. There is a valuable role for the clinical nurse specialist in both the acute and long-term care of patients with TTP. Historically part of the team caring for patients with TTP, specialist nurses have played a vital role in co-ordinating and facilitating treatment for patients, promoting patient advocacy, supporting continuous service improvement, and delivering education to the wider clinical team to disseminate best practice...

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Previous studies have demonstrated that more than 38% of immune-mediated thrombotic thrombocytopenic purpura (TTP) patients in remission with activity >50% had an open ADAMTS13 conformation. We assessed ADAMTS13 conformation in remission (ADAMTS13 activity >60%), focussing on peak ADAMTS13 activity levels and longitudinal assessment in 420 samples across 157 patients. Fewer cases had open conformation at peak ADAMTS13 activity compared to unselected remission samples with ADAMTS13 activity>60% (23% vs 43%)...

While awaiting confirmatory results, empiric therapy for patients suspected to have immune thrombotic thrombocytopenic purpura (iTTP) provides benefits and also accrues risks and costs. Rapid assays for ADAMTS13 may be able to avoid the cost and risk exposure associated with empiric treatment. We conducted the first cost-effectiveness evaluation of testing strategies with rapid versus traditional ADAMTS13 assays in patients with intermediate to high-risk PLASMIC scores, with and without caplacizumab use. We built a Markov cohort simulation with four clinical base-case analyses: 1) Intermediate-risk PLASMIC score with caplacizumab, 2) Intermediate-risk PLASMIC score without caplacizumab, 3) High-risk PLASMIC score with caplacizumab, 4) High-risk PLASMIC score without caplacizumab...