scholarly journals Heparin-induced thrombocytopenia as a cause of prolonged low platelet count in patient with thrombotic thrombocytopenic purpura treated with plasmapheresis

2017 ◽  
Vol 64 (2) ◽  
Author(s):  
Agata Winiarska ◽  
Norbert Kwella ◽  
Tomasz Stompór
Keyword(s):  
Platelet Count ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Low Molecular Weight Heparin ◽  
Careful Analysis ◽  
Low Molecular Weight ◽  
Thrombotic Microangiopathies ◽  
Thrombocytopenic Purpura ◽  
Heparin Induced Thrombocytopenia ◽  
Low Platelet Count ◽  
Functional Deficiency

Thrombotic thrombocytopenic purpura (TTP) is a rare disorder belonging to thrombotic microangiopathies (TMA) and is caused by functional deficiency of metalloproteinase ADAMTS-13. Plasma exchange (PE) remains the treatment of choice in this disease. Here were describe the case of patient who apparently recovered from TTP following multiple sessions of PE, but remained thrombocytopenic. Careful analysis revealed the development of heparin-induced thrombocytopenia (HIT) that precluded platelet count (PLT) normalization. Full normalization of PLT followed discontinuation of PE and low-molecular weight heparin.

scholarly journals Pregnancy onset congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) mimicking HELLP syndrome: a case report

2021 ◽  
Vol 29 (3) ◽  
pp. 270-273
Author(s):  
Başak Ergin ◽  
Berna Buse Kobal ◽  
Zeynep Yazıcı ◽  
Ali Hakan Kaya ◽  
Sezin Canbek ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Hellp Syndrome ◽  
Epigastric Pain ◽  
Novel Mutation ◽  
Adamts13 Activity ◽  
Thrombotic Microangiopathies ◽  
Thrombocytopenic Purpura ◽  
Uremic Syndrome ◽  
Congenital Thrombotic Thrombocytopenic Purpura ◽  
Low Platelet Count

Objective Thrombotic thrombocytopenic purpura is a thrombotic microangiopathic condition characterized by hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. Thrombotic microangiopathies such as preeclampsia and HELLP syndrome are pregnancy-specific, whereas others such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome are not. In this report, we present a case at which we identified a novel mutation which led to a significant reduction of ADAMTS13 activity. Case(s) A nulliparous pregnant woman of 32-year-old presenting with epigastric pain, hypertension and low platelet count was first suspected of HELLP syndrome, but was diagnosed with congenital TTP after delivery. Conclusion HELLP syndrome co-existed with undiagnosed TTP in this case. We strive to have sufficient awareness in order to distinguish these two pathologies from each other on an antenatal basis, because the causes of the managements are entirely different.

Platelet transfusion and catheter insertion for plasma exchange in patients with thrombotic thrombocytopenic purpura and a low platelet count

Transfusion ◽  
2015 ◽  
Vol 55 (7) ◽  
pp. 1798-1802 ◽  
Author(s):  
Etienne Riviere ◽  
Mélanie Saint-Léger ◽  
Chloé James ◽  
Yahsou Delmas ◽  
Benjamin Clouzeau ◽  
...  
Keyword(s):  
Platelet Count ◽  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Platelet Transfusion ◽  
Catheter Insertion ◽  
Thrombocytopenic Purpura ◽  
Low Platelet Count

P0202FIRST REAL-LIFE DATA OF PATIENTS TREATED FOR THROMBOTIC THROMBOCYTOPENIC PURPURA FROM THE FRENCH THROMBOTIC MICROANGIOPATHIES NETWORK

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Mehdi Maanaoui ◽  
Eric Maury ◽  
AUGUSTO Jean François ◽  
Jean Michel Halimi ◽  
Christelle Barbet ◽  
...  
Keyword(s):  
Platelet Count ◽  
Serum Creatinine ◽  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Clinical Practices ◽  
Thrombotic Microangiopathies ◽  
Thrombocytopenic Purpura ◽  
Multicentric Study ◽  
Count Response ◽  
Reported Data

Abstract Background and Aims Caplacizumab, a bivalent Nanobody, targets the A1 domain of von Willebrand Factor, inhibiting the interaction between ultra-large vWF and platelets in the treament of Acquired Thrombotic Thrombocytopenic Purpura (aTTP). Results of the Phase 3 HERCULES study confirm that treatment with caplacizumab reduces the time to platelet count response, resulting in faster resolution of aTTP. Treatment with caplacizumab also resulted in a highly clinically meaningful reduction in aTTP-related death, recurrence of aTTP, or a major thromboembolic event during study drug treatment. Data from clinical practice confirming the clinical trial data is still scarce. We report here the first real world evidence collected in the French thrombotic microangiopathies network. Method Patients with an acute episode of aTTP were included in an observational prospective multicentric study. The diagnosis of aTTP was done according to the French score (platelet count < 30 g/l and serum creatinine< 200µmol/l) or ADAMTS13 activity if available. Adult patients were proposed to be treated with daily plasma exchange (PE) in addition to corticosteroid and frontline rituximab plus early initiation of caplacizumab. PE was stopped after 2 days of platelet count normalization. Results Fifty patients (36 females, median age of 46 years old) were treated for aTTP. 30 patients (60%) had cerebral involvement and 13 (26%) had cardiac involvement. Cardiac troponin I was above upper normal value for 61% of patients. Before treatment, median platelet count was 16 g/l and median serum creatinine was 86 µmol/l. Three initial clinical suspicions of aTTP were finally diagnosed as HUS (2 patients) or vitamin deficiency (1 patient) resulting in the stop of treatment. A median number of 6 plasma exchanges was administered (min:3 - max:16). 375mg/m rituximab injections (3 to 4 for 35/43 patients with reported data) were mostly initiated within 3 days after the first plasma exchange. Caplacizumab treatment was initiated early (37/42 patients with reported data within 2 days after 1st plasma exchange) with a total median duration of treatment of 33 days (min:18 - max:35). The median platelet count normalization was 5 days (min:4 - max:6). 6/41 (14,6%) experienced exacerbations. No deaths and no patients refractory to therapy were reported. Eight adverse events related to caplacizumab therapy were reported, all with good outcomes. Conclusion Concomitant start of additional treatments allow to target severe ADAMTS13 deficiency and platelet aggregation which causes early mortality. The clinical French score seems useful in clinical practices.

scholarly journals Severe Autoimmune LMWH-Induced Thrombocytopenia Presenting with Aortic Thromboses, Adrenal Hemorrhage and Pulmonary Embolism: Response to High-Dose Intravenous Immunoglobulin

2018 ◽  
Vol 13 (3) ◽  
pp. 29-34
Author(s):  
Joshua Nero ◽  
Patricia Araneta ◽  
Theodore E Warkentin ◽  
Otto Moodley
Keyword(s):  
Emergency Department ◽  
Molecular Weight ◽  
Pulmonary Embolism ◽  
Platelet Count ◽  
Low Molecular Weight Heparin ◽  
Adrenal Hemorrhage ◽  
Low Molecular Weight ◽  
High Dose ◽  
Heparin Induced Thrombocytopenia ◽  
Delayed Onset

The occurrence of heparin-induced thrombocytopenia (HIT) in the setting of low-molecular-weight heparin (LMWH) exposure is uncommon, with incidence reported at around 0.2%. Delayed-onset (autoimmune) HIT in the setting of LMWH use is rarer, with only two other case reports in the literature. An 83-year old man was admitted to hospital for an acute exacerbation of chronic obstructive pulmonary disease, receiving low-molecular-weight heparin (LMWH, tinzaparin) while in hospital for prophylaxis against deep venous thrombosis (DVT). One day after discharge, he presented to the emergency department with acute chest pain and dyspnea. Computed tomography revealed bilateral pulmonary embolism, multiple abdominal aortic thromboses, and unilateral adrenal hemorrhage, and he was given a bolus of intravenous unfractionated heparin (UFH) in the emergency department. His platelet count (prior to UFH bolus) was found to be markedly reduced (39 × 109/L) from normal values two days prior. We suspected heparin-induced thrombocytopenia (HIT) to have caused the thrombocytopenia and thromboses (arterial and venous), and thus anticoagulation therapy was changed from heparin to argatroban. His HIT assay was strongly positive, including features of autoimmune reactivity (serum-induced platelet activation in the absence of heparin). HIT developing after exposure to tinzaparin is relatively rare, and use of a scoring system helped to facilitate an early diagnosis. Additionally, this case demonstrates heparin-independent platelet activation, a marker for autoimmune HIT (aHIT).  The patient's serum tested strongly positive for IgG-specific anti-PF4/heparin EIA and serotonin release assay. The presence of these antibodies would also explain the further decline in his platelet count to 10 x 109/L after he received a bolus dose of heparin at the beginning of his second hospitalization. This case highlights the third reported case of delayed-onset HIT in the setting of LMWH, and the rapid response to high-dose intravenous immunoglobulin.

Adherence to Guidelines for Monitoring for Heparin-Induced Thrombocytopenia in Patients Treated with Low Molecular Weight Heparin

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1282-1282
Author(s):  
Maarten ten Berg ◽  
Patricia Van den Bemt ◽  
Albert huisman ◽  
Fred Schobben ◽  
Toine Egberts ◽  
...  
Keyword(s):  
Molecular Weight ◽  
Platelet Count ◽  
Clinical Guidelines ◽  
Low Molecular Weight Heparin ◽  
Low Molecular Weight ◽  
Platelet Factor ◽  
Heparin Induced Thrombocytopenia ◽  
Alternative Anticoagulation ◽  
Life Threatening ◽  
Platelet Count Monitoring

Abstract Laboratory monitoring for early detection of adverse drug reactions is recommended for many drugs. For patients treated with low molecular weight heparin (LMWH), the Summary of Product Characteristics (SPC) and clinical guidelines recommend to monitor the platelet count for heparin-induced thrombocytopenia (HIT), a potentially life-threatening adverse event, characterised by a typical drop in platelet count. When the platelet count drops without obvious explanation in these patients, testing for heparin-platelet factor 4 antibodies (HPF4-Ab) and initiating alternative anticoagulation are advised. In the current study adherence to recommended platelet count monitoring in clinical patients without thrombocytopenia-associated diseases treated with LMWH for at least five days at our institution, and adherence to recommended testing for HPF4-Ab and initiation of alternative anticoagulation in patients with potential HIT (defined as a drop of at least 50% in platelet count between days 5 and 14 following the start of LMWH treatment, or stopdate, whichever occurred first, compared to the highest platelet count within days 1–4) were investigated. Data from the Utrecht Patient Oriented Database (UPOD) were used for this retrospective cohort study. Inpatients exposed to the LMWHs dalteparin or nadroparin for at least five days during the period 2004–2005 were included. Patients with thrombocytopenia-related diseases were excluded. Firstly, adherence to recommended platelet count monitoring, based on recommendations from SPCs and clinical guidelines, was investigated. Secondly, the association between patient- and treatment characteristics and obtaining at least 2 platelets counts during treatment was investigated. Thirdly, adherence to recommended testing for HPF4-Ab and initiating treatment with danaparoid was investigated in patients with potential HIT. 6,804 patients with 7,770 episodes of LMWH treatment of at least five days were included. Adherence to the recommendations for platelet count monitoring from the SPC of nadroparin and dalteparin was 36.5% and 26.3% respectively. Adherence to the different platelet count monitoring recommendations from the 2002 clinical guideline on HIT was 23.0% and 41.5%. Obtaining at least 2 platelet counts during treatment was found to be strongly associated with ICU admission, previous UFH exposure, and a treatment duration of at least 10 days. There were 98 patients with potential HIT. Adherence to testing for HPF4-Ab in patients with potential HIT was 6.1%. Adherence to starting alternative anticoagulation in patients with potential HIT treatment was 0%. The results of this study suggest that adherence to recommendations for monitoring for HIT with LMWH is low at our institution. The results of this study justify to say that there is a need to think of appropriate actions for improving the awareness of HIT as an adverse reaction to LMWH, and to secure the safe use of LMWH.

scholarly journals Heparin-Induced Thrombocytopenia Complicating Thrombotic Thrombocytopenic Purpura

2017 ◽  
Vol 24 (1) ◽  
pp. 43-48
Author(s):  
Galila F. Zaher ◽  
Maha A. Badawi
Keyword(s):  
Pulmonary Embolism ◽  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Therapeutic Plasma Exchange ◽  
Low Molecular Weight ◽  
Early Interventions ◽  
Thromboembolism Prophylaxis ◽  
Thrombocytopenic Purpura ◽  
Heparin Induced Thrombocytopenia ◽  
History Of

Since the introduction of therapeutic plasma exchange for the management of thrombotic thrombocytopenic purpura, the prognosis of this disease improved signifi cantly. Some patients suffer from refractory disease and adjunctive therapy needs to be considered. In addition, alternative explanations for thrombocytopenia may bepresent. In this report we discuss a patient who presented with typical fi ndings of thrombotic thrombocytopenic purpura and responded initially to therapeutic plasma exchange and steroids. Shortlyafterwards, his platelet count deteriorated and he was found to have acute pulmonary embolism. Prior to the pulmonary embolism, the patient had received venous thromboembolism prophylaxis in the form of low molecular weight heparin and had a history of previous exposure to unfractionated heparin. Testing for heparin-induced thrombocytopenia antibodies was positive and the patient was started on an alternative anticoagulant. Despite these early interventions the patient did not survive. It is essential that physicians be aware of such possible associations to request appropriate investigations and start appropriate management, accordingly.

scholarly journals First diagnosis of thrombotic thrombocytopenic purpura after SARS-CoV-2 vaccine – case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Bilgin Osmanodja ◽  
Adrian Schreiber ◽  
Eva Schrezenmeier ◽  
Evelyn Seelow
Keyword(s):  
Acute Kidney Injury ◽  
Differential Diagnosis ◽  
Platelet Count ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Reference Range ◽  
Kidney Injury ◽  
Thrombocytopenic Purpura ◽  
Important Differential Diagnosis ◽  
First Case ◽  
Low Platelet Count

Abstract Background We report a case of a 25-year-old male patient, who developed acquired thrombotic thrombocytopenic purpura (aTTP) after receiving a first dose of mRNA-based SARS-CoV-2 vaccine Spikevax (mRNA-1273, Moderna Biotech, USA). While this is the first case in literature describing a case of aTTP after receiving the Spikevax vaccine, there are two other cases after mRNA-based Covid-19 vaccine and two after adenoviral SARS-CoV-2 vaccine. Case presentation The patient presented with persisting malaise, fever, headache, word-finding difficulties, nausea, vomiting, petechial bleeding, and hematuria 13 days after receiving a first dose of vaccination. Laboratory testing showed low platelet count, Coombs-negative hemolytic anemia, and mild acute kidney injury. We excluded vaccine induced immune thrombotic thrombocytopenia (VITT) as another important differential diagnosis and the final diagnosis was established after ADAMTS-13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13) activity was found to be < 1% (reference range > 40%) and ADAMTS-13 antibodies being 72.2 IU/L (reference range < 12 IU/L). We initiated empiric therapy of plasmapheresis and corticosteroids on admission and started caplacizumab the day after. The patient’s thrombocyte count normalized 3 days after admission, hemolysis and acute kidney injury resolved after 2 weeks. The patient received 2 doses of rituximab (1 g each) after the diagnosis of immune TTP was established. One month after the initial presentation, the patient is in good overall condition, but still receives daily caplacizumab due to ADAMTS-13 activity of < 1%. Conclusions Low platelet count after vaccination against SARS-CoV-2 has gained attraction after vaccine-induced immune thrombotic thrombocytopenia (VITT) has been described as a rare but severe complication of adenoviral-based vaccines. Thrombotic thrombocytopenic purpura (TTP) is an important differential diagnosis, but there are only few reports of TTP following SARS-CoV-2 vaccination. Despite pathophysiological and clinical differences of both entities, diagnostic uncertainty can result in the acute setting, since they share main symptoms such as headache and neurological alterations in addition to thrombocytopenia. In difference to other cases reported, this patient developed first symptoms of TTP as early as 4 days after vaccination, which suggests that vaccination merely acted as trigger for occult TTP, instead of truly inducing an autoimmunological process.

Compliance with Platelet Count Monitoring Recommendations and Management of Possible Heparin-Induced Thrombocytopenia in Hospitalized Patients Receiving Low-Molecular-Weight Heparin

2009 ◽  
Vol 43 (9) ◽  
pp. 1405-1412 ◽  
Author(s):  
Maarten J ten Berg ◽  
Patricia MLA van den Bemt ◽  
Albert Huisman ◽  
Alfred FAM Schobben ◽  
Toine CG Egberts ◽  
...  
Keyword(s):  
Molecular Weight ◽  
Platelet Count ◽  
Low Molecular Weight Heparin ◽  
Clinical Guideline ◽  
Hospitalized Patients ◽  
Surgical Patients ◽  
Low Molecular Weight ◽  
Heparin Induced Thrombocytopenia ◽  
Alternative Anticoagulation ◽  
Platelet Count Monitoring

Background: Summaries of product characteristics (SPCs) and clinical guideline recommendations are available for monitoring the platelet count for heparin-induced thrombocytopenia (HIT) in patients receiving low-molecular-weight heparin (LMWH). Testing for the presence of heparin-platelet factor 4 antibodies (HPF4-Ab) and starting alternative anticoagulation is recommended when HIT is suspected. Objective: To investigate the frequency of compliance with recommendations for platelet count monitoring and management of possible HIT in hospitalized patients receiving prophylaxis and treatment dosing of LMWH for at least 5 consecutive days. Methods: A retrospective cohort study within the Utrecht Patient Oriented Database (UPOD) was conducted. For all inpatients, all episodes of exposure to dalteparin or nadroparin for at least 5 consecutive days in 2004–2005 were selected. In 4 different nonexclusive groups of patients (all pts. receiving dalteparin, all pts. receiving nadroparin, surgical pts. with a prophylactic dose of either dalteparin or nadroparin, and pts. exposed to unfractionated heparin [UFH] within 100 days before receiving either dalteparin or nadroparin), compliance with recommendations for platelet count monitoring from SPCs and a clinical guideline was studied. The frequency of compliance with these recommendations was determined. In addition, it was determined whether patient and treatment characteristics were associated with regular platelet count monitoring. Finally, the frequency of testing for HPF4-Ab and the initiation of danaparoid treatment in patients with a drop of at least 50% in platelet count were investigated. Results: A total of 6804 patients, with 7770 episodes of LMWH treatment, were included in the analysis. The frequency of compliance with platelet count monitoring recommendations was 26.3% for all patients receiving dalteparin, 35.6% for all patients receiving nadroparin, 23.0% for surgical patients receiving prophylactic dosing of either dalteparin or nadroparin, and 41.5% for patients exposed to UFH within 100 days before the start of either dalteparin or nadroparin treatment. Regular platelet count monitoring was strongly positively associated with medical patients (relative risk [RR] 2.33), surgical patients (RR 2.03), critically ill patients (RR 2.60), and those with recent exposure to UFH (RR 2.19). The frequency of testing for HPF4-Ab was 5.4% and the initiation of alternative anticoagulation with danaparoid in patients with a 50% drop in platelet count was 0%. Conclusions: The results suggest that compliance with recommendations for platelet count monitoring and management of possible HIT is low at our institution. Policies and tools to improve compliance with recommended laboratory monitoring should be developed to secure the safe use of LMWH and other medications.

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