Platelet transfusion in thrombotic microangiopathy

BACKGROUND: High titer of donor-specific antibodies (DSA) increases the risk of graft rejection after mismatched related hematopoietic cell transplantation (HCT). There is no data regarding the incidence of anti-HLA recipient-specific antibodies (RSA) and their role after transplantation. OBJECTIVE: To identify the incidence of RSA in a mismatched related hematopoietic cell donor population and their possible impact on immune-mediated complications such as acute graft-versus-host (aGvHD) disease and complications resulting from endothelial injury such as transplant-associated thrombotic microangiopathy (TA-TMA) and veno-occlusive disease (VOD)...

Thrombotic thrombocytopenic purpura (TTP) is a microangiopathy characterized by mechanical hemolytic anemia, resulting in end-organ damage. We describe a case of TTP which presented as an ischemic stroke. The patient presented with stroke as the primary manifestation of TTP despite a normal platelet count and mildly elevated lactate dehydrogenase level (LDH). The patient underwent two transfusions of fresh frozen plasma (FFP), and ADAMTS13 levels confirmed the diagnosis of TTP after discharge. This case demonstrates the importance of maintaining a high index of suspicion for TTP in the setting of normal laboratory values and reveals the many atypical manifestations of TTP...

This study aims to assess the efficacy of second allogeneic hemopoietic stem cell transplantation (allo-HSCT) for treating hemophagocytic syndrome with first engraftment failure. Among a total of 35 patients who underwent allo-HSCT between June 2015 and July 2021 for HLH, 10 patients who underwent a second HSCT following graft rejection were retrospectively analyzed. Various factors, such as the treatment course and outcome, the remission status, donor selection, and the conditioning regimen of patients before second allo-HSCT, were scrutinized for transplant-related complications and transplant-related mortality, as well as transplant outcomes...

There is limited data on the use of NAC in the literature. We would like to present the satisfactory results we obtained in our resistant and relapsed patients as a case series.Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy caused by ADAMTS13 (a disintegrin with thrombospondin type 1 motif and metalloprotease activity, member13) deficiency. Von Willebrand factor (vWF) initiates platelet aggregation and thus thrombus formation. The multimers of vWF are cleaved by ADAMTS13...

OBJECTIVES: To investigate the clinical features of thrombotic microangiopathy associated with allogeneic hematopoietic stem cell transplantation in children. METHODS: A retrospective analysis of continuous clinical data from HSCT received in the Department of Hematology and Oncology of Wuhan Children's Hospital from August 1, 2016 to December 31, 2021. RESULTS: During this period, 209 patients received allo-HSCT in our department, 20 (9.6%) of whom developed TA-TMA...

BACKGROUND: Thrombotic microangiopathy (TMA) is a group of disorders that converge on excessive platelet aggregation in the microvasculature, leading to consumptive thrombocytopenia, microangiopathic hemolysis and ischemic end-organ dysfunction. In predisposed patients, TMA can be triggered by many environmental factors. Glucocorticoids (GCs) can compromise the vascular endothelium. However, GC-associated TMA has rarely been reported, which may be due to the lack of awareness of clinicians...

BACKGROUND: Gemcitabine is a broadly used chemotherapeutic agent that can cause a rare but life-threatening complication called thrombotic microangiopathy (TMA). Early recognition is crucial as therapy options are limited. CASE DESCRIPTION: We report the case of a 46-year-old patient with pancreatic adenocarcinoma who presented with severe anemia and thrombocytopenia as well as acute kidney injury. A diagnosis of gemcitabine-induced TMA was made. He became rapidly transfusion and dialysis dependent...

Transplantation-associated thrombotic microangiopathy (TA-TMA) is an increasingly recognized complication of hematopoietic cell transplantation (HCT) associated with significant morbidity and mortality. However, TA-TMA is a clinical diagnosis, and multiple criteria have been proposed without universal application. Although some patients have a self-resolving disease, others progress to multiorgan failure and/or death. Poor prognostic features also are not uniformly accepted. The lack of harmonization of diagnostic and prognostic markers has precluded multi-institutional studies to better understand incidence and outcomes...

INTRODUCTION: -Thrombotic microangiopathy (TMA), which is the triad of acute kidney injury (AKI), microangiopathic hemolysis hemolytic anemia (MAHA), and thrombocytopenia, is a rare complication of snakebites, and in Sri Lanka, it is commonly seen with hump-nosed pit viper (HNPV) bites. METHODS: -We conducted a prospective observational study of patients with AKI caused by HNPV bites in Teaching Hospital, Ratnapura, Sri Lanka for 6 y, commencing in June 2015. Some patients with TMA underwent therapeutic plasma exchange (TPE) and some did not...

BACKGROUND: Recently, real-world data confirmed the effectiveness of caplacizumab in the treatment of acquired thrombotic thrombocytopenic purpura (aTTP); however, limitations as different treatment protocols from multicenter experiences and the front-line use of rituximab could overshadow the real impact of the addition of caplacizumab. STUDY DESIGN AND METHODS: We report the clinical characteristics and response to treatment of 30 consecutive cases of aTTP treated under a homogeneous therapeutic protocol with the only exception of the addition of caplacizumab in the last 10 cases (caplacizumab group), whose primary outcome we compare with the previous 20 cases (control group)...

Thrombocytopenia is a platelet count of less than 150 × 103 per μL and can occur from decreased platelet production, increased destruction, splenic sequestration, or dilution or clumping. Patients with a platelet count greater than 50 × 103 per μL are generally asymptomatic. Patients with platelet counts between 20 and 50 × 103 per μL may have mild skin manifestations such as petechiae, purpura, or ecchymosis. Patients with platelet counts of less than 10 × 103 per μL have a high risk of serious bleeding...

Microangiopathic hemolytic anemia (MAHA) defines a group of disorders characterized by the formation of microthrombi in capillaries and arterioles and the fragmentation of erythrocytes that pass through. Cancer-related MAHA is a rare but serious condition that is encountered in patients diagnosed with a malignancy. This clinical picture is thought to be linked to certain tumor characteristics; particularly, adenocarcinoma histology, vascular invasion, and bone marrow infiltration. MAHA is most commonly associated with tumors of gastric, prostate, and breast origin...

BACKGROUND: Patients with Human Immunodeficiency Virus (HIV) infection are at risk of thrombotic microangiopathies (TMAs) notably thrombotic thrombocytopenic purpura (TTP) and disseminated intravascular coagulation (DIC). Overlap between laboratory results exists resulting in diagnostic ambiguity. METHODS: Routine laboratory results of 71 patients with HIV-associated TTP (HIV-TTP) and 81 with DIC with concomitant HIV infection (HIV-DIC) admitted between 2015 and 2021 to academic hospitals in Johannesburg, South Africa were retrospectively reviewed...

BACKGROUND: The PLASMIC score is a rapid and inexpensive clinical assessment tool for predicting severe ADAMTS13 deficiency (<10% activity) in patients with suspected thrombotic thrombocytopenic purpura (TTP). The score includes 7 parameters: absence of active cancer, patient not having received stem cell transplant or organ transplant, platelet count <30×109 /L, haemolysis, mean corpuscular volume <90 fl, International Normalized Ratio <1.5, and serum creatinine <2 mg/dL...

Background: Transplant-associated thrombotic microangiopathy (TA-TMA) is a serious complication of bone marrow transplantation (BMT). Recently, abnormalities in the complement system have been identified in the pathogenesis of TA-TMA, and there are series of reports stating that anti-C5 monoclonal antibody (eculizumab) is effective in patients with high levels of the membrane attack complex (C5b-9). Case Presentation: A 12-year-old boy underwent autologous BMT after receiving high-dose chemotherapy for malignant lymphoma...

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy (TMA) requiring urgent treatment. Standardization of its diagnosis and optimal management is challenging. This study aimed to evaluate the role of centralized, rapid testing of ADAMTS13 in patients experiencing acute TMAs requiring plasma-exchange (PEX) and to estimate the incidence of TTP in a large Italian Region. METHODS: We perfomed a cohort study in the frame of the project "Set-up of a Lombardy network for the study and treatment of patients undergoing apheresis", including 11 transfusion centers in the Region...

Chemotherapy-induced thrombocytopenia (CIT) is a common complication of the treatment of non-hematologic malignancies. Many patient-related variables (e.g., age, tumor type, number of prior chemotherapy cycles, amount of bone marrow tumor involvement) determine the extent of CIT. CIT is related to the type and dose of chemotherapy, with regimens containing gemcitabine, platinum, or temozolomide producing it most commonly. Bleeding and the need for platelet transfusions in CIT are rather uncommon except in patients with platelet counts below 25x109/L in whom bleeding rates increase significantly and platelet transfusions are the only treatment...

PURPOSE: Hematopoietic stem-cell transplantation-associated thrombotic microangiopathy (HSCT-TMA) is a serious complication with significant mortality and no approved therapy. HSCT-TMA results from endothelial injury, which activates the lectin pathway of complement. Narsoplimab (OMS721), an inhibitor of mannan-binding lectin-associated serine protease-2 (MASP-2), was evaluated for safety and efficacy in adults with HSCT-TMA. METHODS: In this single-arm open-label pivotal trial (NCT02222545), patients received intravenous narsoplimab once weekly for 4-8 weeks...

BACKGROUND: Acute thrombotic thrombocytopenic purpura (TTP) is an aggressive thrombotic microangiopathy that if not treated, can have a 90% mortality rate. Timely, extensive plasma exchange (PEX) has been indicated to reduce the mortality rate to < 10%, but its side effects are not well-known. We present here a case of a patient presented with Comb (+) TTP and developed catheter-associated deep vein thrombosis (DVT). CASE PRESENTATION: A 27-year-young man presented with persistent thrombocytopenia and Coombs positive anemia was firstly diagnosed with Evans syndrome...

Hematopoietic stem cell transplantation associated-thrombotic microangiopathy (TA-HCT) is one of the early complications of endothelial origin in the course of HCT. Endothelial damage to the microvascular structure and the platelet-rich microthrombi, which are formed as a result of accompanying complement activation, constitute the main pathological conditions resulting TA-TMA. Early diagnosis and management are of utmost importance to prevent multi-organ failure and death. This review summarizes the current understanding of TA-TMA regarding pathogenesis, definition, differential diagnosis, risk factors, surveillance for early diagnosis and management...