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1.
BMC Nephrol ; 22(1): 411, 2021 12 11.
Article in English | MEDLINE | ID: covidwho-1571745

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.


Subject(s)
/adverse effects , COVID-19/prevention & control , Purpura, Thrombotic Thrombocytopenic/chemically induced , Adult , Humans , Male , Platelet Count , Purpura, Thrombotic Thrombocytopenic/therapy , SARS-CoV-2
2.
Blood Purif ; 50(3): 290-297, 2021.
Article in English | MEDLINE | ID: covidwho-1533118

ABSTRACT

The principles and use of plasmapheresis are often little understood by intensivists. We propose to review the principles, the main indications, and the methods of using this technique.


Subject(s)
Critical Care/methods , Plasma Exchange/methods , Animals , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/therapy , COVID-19/therapy , Equipment Design , Guillain-Barre Syndrome/therapy , Humans , Liver Failure, Acute/therapy , Membranes, Artificial , Plasma Exchange/instrumentation , Purpura, Thrombotic Thrombocytopenic/therapy
4.
BMJ Case Rep ; 14(8)2021 Aug 11.
Article in English | MEDLINE | ID: covidwho-1354557

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterised by thrombocytopenia, microangiopathic haemolytic anaemia and microvascular thrombosis. Congenital TTP accounting for less than 5% of all TTP cases can have a late presentation in adulthood mostly triggered by predisposing factors such as infection, pregnancy and inflammation. We present a case of a 23-year-old woman who presented to us in the postpartum period with mesenteric artery thrombosis with infarcts and later was diagnosed as a case of TTP based on congenital a disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS-13) deficiency detected on ADAMTS-13 levels and gene sequencing. She was successfully managed initially with therapeutic plasma exchanges and is now on prophylactic fortnightly fresh frozen plasma infusions at 15 mL/kg body weight and continues to be in remission.


Subject(s)
ADAMTS13 Protein/deficiency , Anemia, Hemolytic , Purpura, Thrombotic Thrombocytopenic , Thrombosis , ADAMTS13 Protein/genetics , Female , Humans , Plasma Exchange , Pregnancy , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy , Young Adult
5.
Transfus Apher Sci ; 60(4): 103174, 2021 Aug.
Article in English | MEDLINE | ID: covidwho-1251570

ABSTRACT

Vaccine-induced immune thrombotic thrombocytopenia (VITT) is associated with high titers of immunoglobulin G class antibodies directed against the cationic platelet chemokine platelet factor 4 (PF4). These antibodies activate platelets via FcγIIa receptors. VITT closely resembles heparin-induced thrombocytopenia. Inflammation and tissue trauma substantially increase the risk for forming pathogenic PF4 antibodies. We therefore propose the use of therapeutic plasma exchange as rescue therapy in VITT to deplete antibodies plus factors promoting inflammation such as excess cytokines in the circulation as well as extracellular vesicles derived from activated platelets.


Subject(s)
COVID-19 Vaccines/adverse effects , Plasma Exchange , Platelet Factor 4/immunology , Purpura, Thrombotic Thrombocytopenic/therapy , Salvage Therapy , Albumins , Antibody Specificity , Anticoagulants , Autoantibodies/immunology , COVID-19 Vaccines/pharmacology , Citrates , Contraindications, Procedure , Cytokines/blood , Extracellular Vesicles , Humans , Immunoglobulin G/immunology , Inflammation , Plasma Exchange/adverse effects , Plasma Exchange/methods , Platelet Activation , Platelet Transfusion/adverse effects , Purpura, Thrombotic Thrombocytopenic/etiology , Purpura, Thrombotic Thrombocytopenic/immunology , Registries , Thrombocytopenia/etiology , Thrombosis/etiology
6.
Int J Hematol ; 114(5): 626-629, 2021 Nov.
Article in English | MEDLINE | ID: covidwho-1310613

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) is a known menace in hematology and is quite rare in practice with known triggers. Lately, in the COVID-19 pandemic, hematology has seen a new pathology amongst which TTP associated with COVID-19 messenger RNA (mRNA) vaccine is unique. We report a case of a 69-year-old male with multiple comorbidities who presented to the hospital with severe fatigue and shortness of breath. Labs were significant for thrombocytopenia, anemia, and hemolysis with schistocytes consistent with TTP with a second dose of BNT162b2 mRNA vaccine as a likely culprit been documented.


Subject(s)
COVID-19 Vaccines/adverse effects , COVID-19 , Immunization, Secondary/adverse effects , Pandemics , Purpura, Thrombotic Thrombocytopenic/etiology , SARS-CoV-2 , ADAMTS13 Protein/immunology , Aged , COVID-19/prevention & control , COVID-19 Vaccines/immunology , Combined Modality Therapy , Dyspnea/etiology , Fatigue/etiology , HIV Infections/complications , Hepatitis B, Chronic/complications , Humans , Hypertension/complications , Immunosuppressive Agents/therapeutic use , Kidney Failure, Chronic/complications , Male , Plasma Exchange , Purpura, Thrombotic Thrombocytopenic/blood , Purpura, Thrombotic Thrombocytopenic/therapy , Venous Thrombosis/complications
7.
J Stroke Cerebrovasc Dis ; 30(9): 105942, 2021 Sep.
Article in English | MEDLINE | ID: covidwho-1281474

ABSTRACT

Vaccine-induced immune thrombotic thrombocytopenia is a rare syndrome following the ChAdOx1 nCov-19 or Ad26.COV2.S vaccine. Reported patients developed mainly venous thrombosis. We describe a case of a young healthy women suffering from acute ischemic stroke due to large vessel occlusion without cerebral venous thrombosis 8 days after vaccination and its consequences on recanalization strategy. Considering the thrombocytopenia, intravenous thrombolysis was contraindicated. She underwent mechanical thrombectomy with complete recanalization and dramatically improved clinically. Positive detection of anti-PF4-heparin-antibodies confirmed vaccine-induced immune thrombotic thrombocytopenia diagnosis. In case of acute ischemic stroke after recent ChAdOx1 nCov-19 or Ad26.COV2.S vaccine, platelet count should be systematically checked before giving thrombolysis, and direct mechanical thrombectomy should be proposed in patients with large vessel occlusion.


Subject(s)
COVID-19 Vaccines/adverse effects , Ischemic Stroke/therapy , Purpura, Thrombotic Thrombocytopenic/therapy , Thrombectomy , Vaccination/adverse effects , Adult , Antibodies/blood , Blood Platelets/immunology , COVID-19 Vaccines/administration & dosage , Female , Heparin/immunology , Humans , Ischemic Stroke/blood , Ischemic Stroke/chemically induced , Ischemic Stroke/diagnosis , Platelet Factor 4/immunology , Purpura, Thrombotic Thrombocytopenic/blood , Purpura, Thrombotic Thrombocytopenic/chemically induced , Purpura, Thrombotic Thrombocytopenic/diagnosis , Treatment Outcome
8.
BMJ Case Rep ; 14(5)2021 May 19.
Article in English | MEDLINE | ID: covidwho-1236429

ABSTRACT

Haemophagocytic lymphohistiocytosis has been reported as an uncommon complication of severe COVID-19 disease while thrombotic thrombocytopenic purpura has been rarely reported. Here, we are reporting a 21-year-old man who developed a combination of these complications during the hospital stay in the post-COVID-19 recovery period. He presented with fever and bilateral COVID-19-related pneumonia requiring invasive ventilation. His hospital course was complicated by the development of pneumothorax, ventilator-associated pneumonia, thrombotic thrombocytopenic purpura and haemophagocytic lymphohistiocytosis. He received remdesivir, IVIG, steroid, fresh frozen plasma and supportive care but had a fatal outcome.


Subject(s)
COVID-19 , Lymphohistiocytosis, Hemophagocytic , Purpura, Thrombotic Thrombocytopenic , Adult , Humans , Lymphohistiocytosis, Hemophagocytic/complications , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/drug therapy , Male , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy , SARS-CoV-2 , Young Adult
9.
Am J Emerg Med ; 49: 441.e3-441.e4, 2021 11.
Article in English | MEDLINE | ID: covidwho-1213004

ABSTRACT

The U.S. Food and Drug Administration (FDA) recently issued an Emergency Use Authorization (EUA) for two highly effective Sars-CoV-2 (COVID-19) vaccines from Pfizer-BioNTech and Moderna. More recently, EUA was granted for the Johnson and Johnson COVID-19 vaccine which uses traditional virus-based technology. In this vaccine, researchers added the gene for the coronavirus spike protein to modified Adenovirus 26 and named it Ad26.COV2-S. Nearly 7 million doses of the Ad26.COV2-S have been administered as of mid-April 2021. Recently the Federal Drug Administration and Center for Disease Control and Prevention reviewed data involving six reported cases in the United States of cerebral venous sinus thrombosis in combination with thrombocytopenia in people who received the vaccination. All cases were in women between 18 and 48, with symptoms developing six to 13 days after vaccination. A recent study in the United Kingdom reported similar events in 23 patients age 21 to 77, 61% of which were female, with cases of presumed vaccine induced thrombosis and thrombocytopenia occurring six to 24 days after vaccination. We report a 62-year-old female who presented to the emergency department (ED) with acute onset of altered mental status. She had received the Ad26.COV2-S vaccine 37 days prior to ED presentation. She developed thrombotic thrombocytopenic purpura (TTP) and no other cause was found. To our knowledge this is the first case in the United States of thrombotic thrombocytopenic purpura after receiving the Ad26.COV2-S COVID-19 vaccine.


Subject(s)
Acute Kidney Injury/etiology , COVID-19 Vaccines/adverse effects , Chemical and Drug Induced Liver Injury/etiology , Purpura, Thrombotic Thrombocytopenic/etiology , Acute Kidney Injury/therapy , Chemical and Drug Induced Liver Injury/therapy , Female , Humans , Mental Disorders/etiology , Methylprednisolone/therapeutic use , Middle Aged , Plasma Exchange , Purpura, Thrombotic Thrombocytopenic/therapy , Renal Dialysis , Treatment Outcome
10.
J Thromb Thrombolysis ; 52(2): 504-507, 2021 Aug.
Article in English | MEDLINE | ID: covidwho-1202813

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) which can cause significant mortality is a thrombotic microangiopathy due to deficiency of VWF cleaving protease ADAMTS13 and as per medical literature there are examples that TTP can be caused by COVID 19 infection. A 35 years old female after admission with right sided weakness and slurring of speech was found to be COVID positive and diagnosed as a case of TTP. Patient had absent ADAMTS13 level on day 1. Treatment was started with therapeutic plasma exchange (TPE) later injection Vincristine and Rituximab was given after 4th TPE as it was suspected as refractory case. Finally patient received 16 TPE procedures with cryo poor plasma as exchange fluid and gradually her platelet count started to maintain normal and she was discharged. Specific management and such association of this type of cases need to be studied more judiciously.


Subject(s)
ADAMTS13 Protein , COVID-19 , Purpura, Thrombotic Thrombocytopenic , Rituximab/administration & dosage , Vincristine/administration & dosage , ADAMTS13 Protein/blood , ADAMTS13 Protein/deficiency , Adult , Antineoplastic Agents/administration & dosage , COVID-19/blood , COVID-19/complications , COVID-19/diagnosis , Female , Humans , Immunologic Factors/administration & dosage , Plasma Exchange/methods , Platelet Count/methods , Purpura, Thrombotic Thrombocytopenic/blood , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/etiology , Purpura, Thrombotic Thrombocytopenic/therapy , SARS-CoV-2/isolation & purification , Treatment Outcome
11.
Crit Care Med ; 49(5): e512-e520, 2021 05 01.
Article in English | MEDLINE | ID: covidwho-1185992

ABSTRACT

OBJECTIVES: Prevention and therapy of immunothrombosis remain crucial challenges in the management of coronavirus disease 2019, since the underlying mechanisms are incompletely understood. We hypothesized that endothelial damage may lead to substantially increased concentrations of von Willebrand factor with subsequent relative deficiency of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). DESIGN: Prospective controlled cross-over trial. SETTING: Blood samples of patients with confirmed coronavirus disease 2019 and healthy controls were obtained in three German hospitals and analyzed in a German hemostaseologic laboratory. PATIENTS: Seventy-five patients with confirmed coronavirus disease 2019 of mild to critical severity and 30 healthy controls. MEASUREMENTS AND MAIN RESULTS: von Willebrand factor antigen, ADAMTS13, and von Willebrand factor multimer formation were analyzed. von Willebrand factor antigen was 4.1 times higher in COVID-19 patients compared with healthy controls (p < 0.0001), whereas ADAMTS13 activities were not significantly different (p = 0.18). The ADAMTS13/von Willebrand factor antigen ratio was significantly lower in COVID-19 than in the control group (24.4 ± 20.5 vs 82.0 ± 30.7; p < 0.0001). Fourteen patients (18.7%) undercut a critical ratio of 10 as described in thrombotic thrombocytopenic purpura. Gel analysis of multimers resembled a thrombotic thrombocytopenic purpura pattern with loss of the largest multimers in 75% and a smeary triplet pattern in 39% of the patients. The ADAMTS13/von Willebrand factor antigen ratio decreased continuously from mild to critical disease (analysis of variance p = 0.026). Furthermore, it differed significantly between surviving patients and those who died from COVID-19 (p = 0.001) yielding an area under the curve of 0.232 in receiver operating characteristic curve curve analysis. CONCLUSION: COVID-19 is associated with a substantial increase in von Willebrand factor levels, which can exceed the ADAMTS13 processing capacity resulting in the formation of large von Willebrand factor multimers indistinguishable from thrombotic thrombocytopenic purpura. The ADAMTS13/von Willebrand factor antigen ratio is an independent predictor of severity of disease and mortality. These findings provide a rationale to consider plasma exchange as a therapeutic option in COVID-19 and to include von Willebrand factor and ADAMTS13 in the diagnostic workup.


Subject(s)
ADAMTS13 Protein/deficiency , COVID-19/blood , COVID-19/immunology , Purpura, Thrombotic Thrombocytopenic/immunology , SARS-CoV-2/immunology , von Willebrand Factor/metabolism , Aged , Aged, 80 and over , Cross-Over Studies , Female , Germany/epidemiology , Humans , Male , Middle Aged , Plasma Exchange , Prospective Studies , Purpura, Thrombotic Thrombocytopenic/therapy
12.
Arch Ital Urol Androl ; 93(1): 71-76, 2021 Mar 19.
Article in English | MEDLINE | ID: covidwho-1146951

ABSTRACT

This collection of cases describes some unusual urological tumors and complications related to urological tumors and their treatment. Case 1: A case of uretero-arterial fistula in a patient with long-term ureteral stenting for ureteral oncological stricture and a second case associated to retroperitoneal fibrosis were described. Abdominal CT, pyelography, cystoscopy were useful to show the origin of the bleeding. Angiography is useful for confirming the diagnosis and for subsequent positioning of an endovascular prosthesis which represents a safe approach with reduced post-procedural complications. Case 2: A case of patient who suffered from interstitial pneumonitis during a cycle of intravesical BCG instillations for urothelial cancer. The patient was hospitalized for more than two weeks in a COVID ward for a suspected of COVID-19 pneumonia, but he did not show any evidence of SARS-CoV-2 infection during his hospital stay. Case 3: A case of a young man with a functional urinary bladder paraganglioma who was successfully managed with complete removal of the tumor, leaving the urinary bladder intact. Case 4: A case of a 61 year old male suffering from muscle invasive bladder cancer who was admitted for a radical cystectomy and on the eighth postoperative day developed microangiopathic hemolytic anemia and thrombocytopenia, which clinically defines thrombotic microangiopathy.


Subject(s)
Urologic Neoplasms/therapy , Administration, Intravesical , Adult , BCG Vaccine/therapeutic use , COVID-19/complications , COVID-19/therapy , Carcinoma, Transitional Cell/pathology , Computed Tomography Angiography , Cystectomy , Fistula/complications , Fistula/therapy , Humans , Male , Middle Aged , Paraganglioma/surgery , Paraganglioma/therapy , Pneumonia/complications , Pneumonia/therapy , Postoperative Complications/therapy , Purpura, Thrombotic Thrombocytopenic/etiology , Purpura, Thrombotic Thrombocytopenic/therapy , Ureteral Diseases/complications , Ureteral Diseases/diagnostic imaging , Ureteral Diseases/therapy , Urinary Bladder Neoplasms/complications , Urinary Bladder Neoplasms/therapy , Urologic Neoplasms/complications , Urologic Neoplasms/diagnostic imaging
13.
J Med Case Rep ; 15(1): 104, 2021 Mar 01.
Article in English | MEDLINE | ID: covidwho-1112451

ABSTRACT

BACKGROUND: Pregnancy seems to increase the risk of thrombotic thrombocytopenic purpura (TTP) relapses and make the TTP more severe in any of the pregnancy trimesters, or even during the postpartum period. CASE PRESENTATION: This study highlights details of treating a COVID-19 pregnant patient who survived. This 21-year addicted White woman was admitted at her 29th week and delivered a stillbirth. She was transferred to another hospital after showing signs of TTP, which was caused by a viral infection. CONCLUSION: This viral infection caused fever and dyspnea, and the patient was tested positive for COVID-19 infection. A chest computed tomography scan showed diffuse multiple bilateral consolidations and interlobar septal thickening. She stayed at the Intensive Care Unit for 20 days and treated with plasmapheresis. As far as we know, this is the first report of a TTP pregnant patient with COVID-19 infection.


Subject(s)
COVID-19/diagnosis , Plasmapheresis , Pregnancy Complications, Hematologic/diagnosis , Pregnancy Complications, Infectious/diagnosis , Purpura, Thrombotic Thrombocytopenic/diagnosis , Stillbirth , Acute Kidney Injury/therapy , Amphetamine-Related Disorders , Antiviral Agents/therapeutic use , COVID-19/therapy , Drug Combinations , Erythrocyte Transfusion , Female , Hemoglobins/metabolism , Humans , Hydroxychloroquine/therapeutic use , Intensive Care Units , L-Lactate Dehydrogenase/metabolism , Lopinavir/therapeutic use , Methamphetamine , Pregnancy , Pregnancy Complications, Hematologic/metabolism , Pregnancy Complications, Hematologic/therapy , Pregnancy Complications, Infectious/metabolism , Pregnancy Complications, Infectious/therapy , Purpura, Thrombotic Thrombocytopenic/metabolism , Purpura, Thrombotic Thrombocytopenic/therapy , Renal Dialysis , Ritonavir/therapeutic use , SARS-CoV-2 , Tomography, X-Ray Computed , Young Adult
14.
Transfus Apher Sci ; 60(3): 103107, 2021 Jun.
Article in English | MEDLINE | ID: covidwho-1104307

ABSTRACT

We report the case of a patient diagnosed with a clinical relapse of acquired immune-mediated thrombotic thrombocytopenic purpura (TTP) who was successfully treated with low-dose rituximab plus corticosteroids without the use of plasma exchange (PEx), which was unavailable at the time due to the COVID-19 pandemic. Rituximab 100 mg weekly for 4 weeks was administered, combined with 1 mg/kg of prednisone, obtaining a complete hematological response in 6 weeks. This case suggests that PEx may be unnecessary for a subset of patients with relapsed TTP who are clinically stable without significant end-organ damage. A brief literature review regarding TTP patients treated without plasma exchange is also included.


Subject(s)
COVID-19/epidemiology , Plasma Exchange/methods , Purpura, Thrombotic Thrombocytopenic/therapy , Adult , Female , Humans , Pandemics , SARS-CoV-2/isolation & purification , Young Adult
15.
Int Immunopharmacol ; 93: 107397, 2021 Apr.
Article in English | MEDLINE | ID: covidwho-1065226

ABSTRACT

INTRODUCTION: Thrombotic microangiopathies are a group of disorders that are mainly related to endothelial dysfunction. This category of endothelial dysfunction results of several imbalances between platelets, endothelium and immune system, also cytokine production. AIM OF THIS STUDY: To report cases with thrombotic thrombocytopenic purpura (TTP) and COVID-19 and review COVID-19 endothelial dysfunction literature. METHODS: Primary laboratory data, peripheral blood smear, ADAMTS13 antigen activity level, and antibody ordered for each of these four patients. Treatments for COVID-19 administered for all patients. Traditional treatments for TTP also were administered. RESULTS: There were numerous schistocytes (more than 5%) in peripheral blood smears for each patient. ADAMTS13 antigen activity level was below 10%, and ADAMTS13 antibody was elevated for each patient. COVID-19 PCR was positive for all patients, and CT-Scans were indicative of the involvement of COVID-19. CONCLUSION: In this case series, we reported four COVID-19 patients who presented with signs and symptoms of anemia and thrombocytopenia, resulting in thrombotic thrombocytopenic purpura.


Subject(s)
COVID-19/pathology , Purpura, Thrombotic Thrombocytopenic/virology , ADAMTS13 Protein/blood , Adult , COVID-19/blood , COVID-19/epidemiology , COVID-19/therapy , Female , Humans , Male , Middle Aged , Purpura, Thrombotic Thrombocytopenic/blood , Purpura, Thrombotic Thrombocytopenic/epidemiology , Purpura, Thrombotic Thrombocytopenic/therapy , SARS-CoV-2/isolation & purification
16.
J Clin Apher ; 36(3): 499-504, 2021 Jun.
Article in English | MEDLINE | ID: covidwho-1033045

ABSTRACT

Management of aTTP in patients who refuse or are intolerant to plasma remains challenging, but new drugs can be implemented with success. A 39-year-old woman presented to the Emergency department for bruises at the upper and lower limbs together with worsening anemia and thrombocytopenia; PLASMIC score was seven, indicative of high risk to have a thrombotic microangiopathy due to severe ADAMTS-13 deficiency: indeed, it was 1.4%. We immediately started Plasma Exchange, but after the third procedure she developed severe anaphylaxis to Octaplas plasma, so PEXs were discontinued. We proceeded to a salvage strategy with rituximab and caplacizumab that was rapidly effective to resolve symptoms and hemolysis. It has been already reported a case in which a patient developed severe reactions to fresh-frozen plasma that required discontinuation of PEX. Differently from this case, our patient was already using the less immunogenic pooled plasma units Octaplas, therefore a strategy with caplacizumab was the only available option. Moreover, rituximab is associated with a shorter time to obtain a durable remission in aTTP and a faster time (15 days) to final ADAMTS13 activity recovery >10%. To our knowledge, this is the first case of early discontinuation of caplacizumab in a patient allergic to PEX by actively monitoring ADAMTS13 activity, allowing optimization of healthcare resources during COVID-19 pandemic.


Subject(s)
Plasmapheresis/methods , Purpura, Thrombotic Thrombocytopenic/therapy , Rituximab/therapeutic use , Single-Domain Antibodies/therapeutic use , ADAMTS13 Protein/metabolism , Adult , Emergency Service, Hospital , Female , Hemolysis , Humans , Plasma Exchange , Remission Induction , Risk
17.
J Thromb Thrombolysis ; 52(2): 468-470, 2021 Aug.
Article in English | MEDLINE | ID: covidwho-1002138

ABSTRACT

Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disease that can be triggered by different events, including viral infections. It presents as thrombotic microangiopathy and can lead to severe complications that often require management in the intensive care unit (ICU). We report a patient who presented with acquired TTP following COVID-19 infection. A 44-year-old woman presented to the emergency department with severe anemia, acute kidney injury and respiratory failure due to COVID-19. Clinical and laboratory findings were suggestive for thrombotic microangiopathy. On day 8 laboratory tests confirmed the diagnosis of acquired TTP. The patient needed 14 plasma exchanges, treatment with steroids, rituximab and caplacizumab and 18 days of mechanical ventilation. She completely recovered and was discharged home on day 51. Acquired TTP can be triggered by different events leading to immune stimulation. COVID-19 has been associated with different inflammatory and auto-immune diseases. Considering the temporal sequence and the lack of other possible causes, we suggest that COVID-19 infection could have been the triggering factor in the development of TTP. Since other similar cases have already been described, possible association between COVID and TTP deserves further investigation.


Subject(s)
COVID-19 , Plasma Exchange/methods , Purpura, Thrombotic Thrombocytopenic , Respiration, Artificial/methods , Respiratory Insufficiency , Rituximab/administration & dosage , Single-Domain Antibodies/administration & dosage , Acute Kidney Injury/diagnosis , Acute Kidney Injury/etiology , Adult , COVID-19/complications , COVID-19/immunology , COVID-19/physiopathology , COVID-19/therapy , Female , Fibrinolytic Agents/administration & dosage , Humans , Immunologic Factors/administration & dosage , Male , Purpura, Thrombotic Thrombocytopenic/blood , Purpura, Thrombotic Thrombocytopenic/etiology , Purpura, Thrombotic Thrombocytopenic/physiopathology , Purpura, Thrombotic Thrombocytopenic/therapy , Respiratory Insufficiency/diagnosis , Respiratory Insufficiency/etiology , Respiratory Insufficiency/therapy , SARS-CoV-2/isolation & purification , SARS-CoV-2/pathogenicity , Thrombotic Microangiopathies/diagnosis , Thrombotic Microangiopathies/etiology , Treatment Outcome
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