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1.
Int J Hematol ; 116(1): 55-59, 2022 Jul.
Article in English | MEDLINE | ID: covidwho-1877965

ABSTRACT

Autoimmune and complement-related hematological side effects have been observed with messenger ribonucleic acid (mRNA) vaccines. Here, we report the incidence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine-induced hemolysis in patients with paroxysmal nocturnal hemoglobinuria (PNH). We reviewed the medical records of seventeen patients with PNH visiting the University of Tsukuba Hospital who had received two doses of the SARS-CoV-2 mRNA vaccine between May 2021 and November 2021. Twelve patients were being treated with complement inhibitors. The median age of all patients was 62 years (range 29-89 years).; six were males and eleven were females. Fourteen patients received the BNT162b2 vaccine (Pfizer/BioNTech) and three received the mRNA-1273 vaccine (Moderna). The median percentages of PNH clones in erythrocytes and granulocytes were 37.61% (range 8.11-85.71%) and 59.73% (range 3.76-97.82%), respectively. Of the twelve patients receiving complement inhibitors, only one had a hemolytic reaction after vaccination, but it did not meet the definition of breakthrough hemolysis. By contrast, hemolytic attacks were observed in two of the five untreated patients with PNH, and one of them required a blood transfusion. Appropriate administration of complement inhibitors to patients with PNH may prevent hemolysis induced by SARS-CoV-2 mRNA vaccination.


Subject(s)
COVID-19 Vaccines , COVID-19 , Hemoglobinuria, Paroxysmal , Hemolysis , 59829/adverse effects , Adult , Aged , Aged, 80 and over , 59828/adverse effects , COVID-19/complications , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Complement Inactivating Agents/therapeutic use , Female , Hemoglobinuria, Paroxysmal/complications , Hemoglobinuria, Paroxysmal/drug therapy , Humans , Male , Middle Aged , SARS-CoV-2 , Vaccination/adverse effects
2.
Rinsho Ketsueki ; 63(3): 224-228, 2022.
Article in Japanese | MEDLINE | ID: covidwho-1780264

ABSTRACT

Atypical hemolytic uremic syndrome (aHUS) is a lethal disease resulting in systemic thrombotic microangiopathies due to complement dysregulation. Immune activation by viral infections, such as SARS-CoV-2, may trigger hemolytic attack. A 38-year-old man, who had been previously diagnosed with aHUS due to complement component 3 mutation, was proven to be positive for SARS-CoV-2 without respiratory symptoms. No specific intervention was given to the patient, and he developed hematuria and oliguria three days after diagnosis. The patient was subsequently referred to our hospital and treated with eculizumab (900 mg). Afterward, the hemolytic symptoms improved rapidly. To the best of our knowledge, there have been reports of at least ten cases of hemolysis triggered by COVID-19 in patients with aHUS, and a potential clinical benefit of eculizumab for hemolytic attack, as well as for COVID-19, has been suggested. Here, we report the findings of a case, which indicate the efficacy of eculizumab introduction at an early stage.


Subject(s)
Atypical Hemolytic Uremic Syndrome , COVID-19 , Thrombotic Microangiopathies , Adult , Atypical Hemolytic Uremic Syndrome/diagnosis , COVID-19/complications , Hemolysis , Humans , Male , SARS-CoV-2 , Thrombotic Microangiopathies/diagnosis
3.
J Clin Apher ; 36(4): 628-633, 2021 Aug.
Article in English | MEDLINE | ID: covidwho-1748739

ABSTRACT

BACKGROUND: Criteria for selection of FFP blood type has not been clearly established and use of group AB plasma is preferred by numerous transplantation protocols. AIMS: This study assesses the safety and efficacy of alternative group A or B plasma in ABO incompatible solid organ transplantation. MATERIALS & METHODS: Alternative use of group A or B plasma (incompatible plasma) was inevitable during the shortage of group AB plasma. Experience from select number of patients during the period of extreme group AB plasma shortage is described. RESULTS: The result of alternative use of group A or B plasma was within expectation, showing effective reduction of isoagglutinin titers for pre-operative desensitization and efficacy for treatment of post-operative patients. No immediate hemolytic transfusion reaction was reported. DISCUSSION: While validation in a larger cohort of patients is necessary, our limited experience have shown satisfactory clinical outcomes without adverse events. CONCLUSIONS: Use of incompatible group A or B plasma is a viable option when group AB plasma is limited.


Subject(s)
ABO Blood-Group System , Blood Group Incompatibility/therapy , Plasma Exchange/methods , Transplantation/methods , Agglutinins/chemistry , Blood Banks/supply & distribution , Graft Survival , Hemolysis , Humans , Kidney Transplantation/adverse effects , Patient Safety , Plasma/immunology , Plasmapheresis , Transfusion Reaction , Treatment Outcome
4.
Hematol Oncol Clin North Am ; 36(2): 353-363, 2022 04.
Article in English | MEDLINE | ID: covidwho-1734426

ABSTRACT

Autoimmune hemolytic anemia (AHIA) is the group of acquired autoimmune conditions resulting from the development of autologous antibodies directed against autologous red blood cell antigens resulting in red cell lysis. Beyond the presence, severity, and duration of hemolysis which can lead to symptomatic anemia, additional complications at presentation and during treatment require a high degree of clinical vigilance. These include among others cutaneous, thrombotic, renal disorders, and infectious disorders. Complications can be due to the presence of the pathologic antibody itself, the process of hemolysis, or attributed to treatment. Comprehensive management of AIHA requires awareness and assessment of complications at diagnosis, during, and following treatment.


Subject(s)
Anemia, Hemolytic, Autoimmune , Hematopoietic Stem Cell Transplantation , Thrombosis , Anemia, Hemolytic, Autoimmune/diagnosis , Anemia, Hemolytic, Autoimmune/etiology , Anemia, Hemolytic, Autoimmune/therapy , Erythrocytes , Hematopoietic Stem Cell Transplantation/adverse effects , Hemolysis , Humans
5.
Int J Mol Sci ; 23(4)2022 Feb 16.
Article in English | MEDLINE | ID: covidwho-1701642

ABSTRACT

Several diseases (such as diabetes, cancer, and neurodegenerative disorders) affect the morpho-functional aspects of red blood cells, sometimes altering their normal metabolism. In this review, the hematological changes are evaluated, with particular focus on the morphology and metabolic aspects of erythrocytes. Changes in the functionality of such cells may, in fact, help provide important information about disease severity and progression. The viral infection causes significant damage to the blood cells that are altered in size, rigidity, and distribution width. Lower levels of hemoglobin and anemia have been reported in several studies, and an alteration in the concentration of antioxidant enzymes has been shown to promote a dangerous state of oxidative stress in red blood cells. Patients with severe COVID-19 showed an increase in hematological changes, indicating a progressive worsening as COVID-19 severity progressed. Therefore, monitored hematological alterations in patients with COVID-19 may play an important role in the management of the disease and prevent the risk of a severe course of the disease. Finally, monitored changes in erythrocytes and blood, in general, may be one of the causes of the condition known as Long COVID.


Subject(s)
COVID-19/blood , COVID-19/diet therapy , Erythrocytes/virology , Anemia/virology , Antiviral Agents/pharmacology , COVID-19/complications , COVID-19/etiology , COVID-19/metabolism , Erythrocytes/metabolism , Erythrocytes/pathology , Hemoglobins/metabolism , Hemolysis , Humans , Oxidative Stress
6.
Scand J Clin Lab Invest ; 82(2): 138-142, 2022 04.
Article in English | MEDLINE | ID: covidwho-1684251

ABSTRACT

Modern blood gas analyzers are not able to identify hemolysis, lipemia and icterus; therefore, the aim of this study was to assess the influence of hemolysis on blood gas samples. Blood gas analysis represents an essential part in the diagnosis and treatment of critically ill patients, including those affected by the pandemic coronavirus disease 2019 (COVID-19). Hemolysis, lipemia, and icterus, are causes of clinical misinterpretation of laboratory tests. A total of 1244 blood gas specimens were collected over a one-week period from different clinical wards, including the Emergency Department, and were assessed for serum indices on Cobas C6000 CE (Roche Diagnostics, Mannheim, Germany). The prevalence of hemolysis, lipemia, and icterus were 5%, 12%, and 14%, respectively. Sample storage at room temperature, delivery to central laboratory using pneumatic tube system, as well as small sample size, strongly affected blood gas parameters (p < .01). Hemolysis led to an increase in analytical bias for pH, pO2, and potassium, and a significant decrease for pCO2, HCO3-, sodium, and Ca2+ (p <.01). Currently, hemolysis detection systems are not yet widespread, and a rapid centrifugation of samples after blood gas analysis along with the assessment of serum indices represent the only prompt approach to identify unsuitable results, avoiding pitfalls in clinical decision-making, although it cannot be applied to the Emergency Department routine. Blood gas analyzers manufacturers and suppliers should implement automated built-in serum indices detection systems.


Subject(s)
COVID-19 , Hyperlipidemias , Jaundice , Blood Gas Analysis/methods , Hematologic Tests , Hemolysis , Humans
7.
Biosci Rep ; 41(12)2021 12 22.
Article in English | MEDLINE | ID: covidwho-1592575

ABSTRACT

Parasporin-2Aa1 (PS2Aa1) is a toxic protein of 37 KDa (30 kDa, activated form produced by proteolysis) that was shown to be cytotoxic against specific human cancer cells, although its mechanism of action has not been elucidated yet. In order to study the role of some native peptide fragments of proteins on anticancer activity, here we investigated the cytotoxic effect of peptide fragments from domain-1 of PS2Aa1 and one of the loops present in the binding region of the virus spike protein from Alphacoronavirus (HCoV-229E), the latter according to scientific reports, who showed interaction with the human APN (h-APN) receptor, evidence corroborated through computational simulations, and thus being possible active against colon cancer cells. Peptides namely P264-G274, Loop1-PS2Aa, and Loop2-PS2Aa were synthesized using the Fmoc solid-phase synthesis and characterized by mass spectrometry (MS). Additionally, one region from loop 1 of HCoV-229E, Loop1-HCoV-229E, was also synthesized and characterized. The A4W-GGN5 anticancer peptide and 5-fluorouracil (5-FU) were taken as a control in all experiments. Circular dichroism revealed an α-helix structure for the peptides derived from PS2Aa1 (P264-G274, Loop1-PS2Aa, and Loop2-PS2Aa) and ß-laminar structure for the peptide derived from Alphacoronavirus spike protein Loop1-HCoV-229E. Peptides showed a hemolysis percentage of less than 20% at 100 µM concentration. Besides, peptides exhibited stronger anticancer activity against SW480 and SW620 cells after exposure for 48 h. Likewise, these compounds showed significantly lower toxicity against normal cells CHO-K1. The results suggest that native peptide fragments from Ps2Aa1 may be optimized as a novel potential cancer-therapeutic agents.


Subject(s)
Antineoplastic Agents/pharmacology , Colorectal Neoplasms/drug therapy , Endotoxins/pharmacology , Peptide Fragments/pharmacology , Spike Glycoprotein, Coronavirus/pharmacology , Alphacoronavirus , Animals , Antineoplastic Agents/chemical synthesis , Antineoplastic Agents/toxicity , CD13 Antigens/metabolism , CHO Cells , Cell Line, Tumor , Cell Proliferation/drug effects , Colorectal Neoplasms/metabolism , Colorectal Neoplasms/pathology , Cricetulus , Endotoxins/toxicity , Hemolysis/drug effects , Humans , Molecular Docking Simulation , Peptide Fragments/chemical synthesis , Peptide Fragments/toxicity , Protein Conformation, alpha-Helical , Sheep, Domestic , Spike Glycoprotein, Coronavirus/toxicity , Structure-Activity Relationship
10.
Front Immunol ; 12: 767347, 2021.
Article in English | MEDLINE | ID: covidwho-1528823

ABSTRACT

Infection with SARS-CoV-2 triggers the simultaneous activation of innate inflammatory pathways including the complement system and the kallikrein-kinin system (KKS) generating in the process potent vasoactive peptides that contribute to severe acute respiratory syndrome (SARS) and multi-organ failure. The genome of SARS-CoV-2 encodes four major structural proteins - the spike (S) protein, nucleocapsid (N) protein, membrane (M) protein, and the envelope (E) protein. However, the role of these proteins in either binding to or activation of the complement system and/or the KKS is still incompletely understood. In these studies, we used: solid phase ELISA, hemolytic assay and surface plasmon resonance (SPR) techniques to examine if recombinant proteins corresponding to S1, N, M and E: (a) bind to C1q, gC1qR, FXII and high molecular weight kininogen (HK), and (b) activate complement and/or the KKS. Our data show that the viral proteins: (a) bind C1q and activate the classical pathway of complement, (b) bind FXII and HK, and activate the KKS in normal human plasma to generate bradykinin and (c) bind to gC1qR, the receptor for the globular heads of C1q (gC1q) which in turn could serve as a platform for the activation of both the complement system and KKS. Collectively, our data indicate that the SARS-CoV-2 viral particle can independently activate major innate inflammatory pathways for maximal damage and efficiency. Therefore, if efficient therapeutic modalities for the treatment of COVID-19 are to be designed, a strategy that includes blockade of the four major structural proteins may provide the best option.


Subject(s)
Antigens, Viral/immunology , COVID-19/immunology , Complement System Proteins/immunology , Kallikrein-Kinin System , SARS-CoV-2/immunology , Viral Structural Proteins/immunology , Carrier Proteins/genetics , Carrier Proteins/immunology , Hemolysis , Humans , Mitochondrial Proteins/genetics , Mitochondrial Proteins/immunology , Recombinant Proteins/immunology , Viral Structural Proteins/genetics
11.
Biosci Rep ; 41(12)2021 12 22.
Article in English | MEDLINE | ID: covidwho-1526113

ABSTRACT

Parasporin-2Aa1 (PS2Aa1) is a toxic protein of 37 KDa (30 kDa, activated form produced by proteolysis) that was shown to be cytotoxic against specific human cancer cells, although its mechanism of action has not been elucidated yet. In order to study the role of some native peptide fragments of proteins on anticancer activity, here we investigated the cytotoxic effect of peptide fragments from domain-1 of PS2Aa1 and one of the loops present in the binding region of the virus spike protein from Alphacoronavirus (HCoV-229E), the latter according to scientific reports, who showed interaction with the human APN (h-APN) receptor, evidence corroborated through computational simulations, and thus being possible active against colon cancer cells. Peptides namely P264-G274, Loop1-PS2Aa, and Loop2-PS2Aa were synthesized using the Fmoc solid-phase synthesis and characterized by mass spectrometry (MS). Additionally, one region from loop 1 of HCoV-229E, Loop1-HCoV-229E, was also synthesized and characterized. The A4W-GGN5 anticancer peptide and 5-fluorouracil (5-FU) were taken as a control in all experiments. Circular dichroism revealed an α-helix structure for the peptides derived from PS2Aa1 (P264-G274, Loop1-PS2Aa, and Loop2-PS2Aa) and ß-laminar structure for the peptide derived from Alphacoronavirus spike protein Loop1-HCoV-229E. Peptides showed a hemolysis percentage of less than 20% at 100 µM concentration. Besides, peptides exhibited stronger anticancer activity against SW480 and SW620 cells after exposure for 48 h. Likewise, these compounds showed significantly lower toxicity against normal cells CHO-K1. The results suggest that native peptide fragments from Ps2Aa1 may be optimized as a novel potential cancer-therapeutic agents.


Subject(s)
Antineoplastic Agents/pharmacology , Colorectal Neoplasms/drug therapy , Endotoxins/pharmacology , Peptide Fragments/pharmacology , Spike Glycoprotein, Coronavirus/pharmacology , Alphacoronavirus , Animals , Antineoplastic Agents/chemical synthesis , Antineoplastic Agents/toxicity , CD13 Antigens/metabolism , CHO Cells , Cell Line, Tumor , Cell Proliferation/drug effects , Colorectal Neoplasms/metabolism , Colorectal Neoplasms/pathology , Cricetulus , Endotoxins/toxicity , Hemolysis/drug effects , Humans , Molecular Docking Simulation , Peptide Fragments/chemical synthesis , Peptide Fragments/toxicity , Protein Conformation, alpha-Helical , Sheep, Domestic , Spike Glycoprotein, Coronavirus/toxicity , Structure-Activity Relationship
14.
Biomolecules ; 11(5)2021 04 27.
Article in English | MEDLINE | ID: covidwho-1215327

ABSTRACT

The SARS-CoV-2 outbreak was declared a worldwide pandemic in 2020. Infection triggers the respiratory tract disease COVID-19, which is accompanied by serious changes in clinical biomarkers such as hemoglobin and interleukins. The same parameters are altered during hemolysis, which is characterized by an increase in labile heme. We present two computational-experimental approaches aimed at analyzing a potential link between heme-related and COVID-19 pathophysiologies. Herein, we performed a detailed analysis of the common pathways induced by heme and SARS-CoV-2 by superimposition of knowledge graphs covering heme biology and COVID-19 pathophysiology. Focus was laid on inflammatory pathways and distinct biomarkers as the linking elements. In a second approach, four COVID-19-related proteins, the host cell proteins ACE2 and TMPRSS2 as well as the viral proteins 7a and S protein were computationally analyzed as potential heme-binding proteins with an experimental validation. The results contribute to the understanding of the progression of COVID-19 infections in patients with different clinical backgrounds and may allow for a more individual diagnosis and therapy in the future.


Subject(s)
COVID-19/metabolism , Heme/metabolism , SARS-CoV-2/physiology , Angiotensin-Converting Enzyme 2/metabolism , COVID-19/pathology , Computational Biology , Hemolysis , Host-Pathogen Interactions , Humans , Inflammation/metabolism , Inflammation/pathology , Models, Biological , Models, Molecular , Protein Binding , Protein Interaction Maps , Serine Endopeptidases/metabolism , Viral Proteins/metabolism
16.
J Med Virol ; 93(2): 1164-1170, 2021 02.
Article in English | MEDLINE | ID: covidwho-1196441

ABSTRACT

The current pandemic due to coronavirus disease 2019 (COVID-19) has posed an unprecedented challenge for the medical communities, various countries worldwide, and their citizens. Severe acute respiratory syndrome coronavirus 2 has been studied for its various pathophysiological pathways and mechanisms through which it causes COVID-19. In this study, we discussed the immunological impact of COVID-19 on the hematological system, platelets, and red blood cells.


Subject(s)
COVID-19/complications , COVID-19/immunology , Hemolysis/immunology , Thrombocytopenia/virology , Adolescent , Adult , Aged , Aged, 80 and over , Blood Platelets/pathology , COVID-19/blood , Erythrocytes/pathology , Female , Humans , Male , Middle Aged , Young Adult
17.
BMJ Case Rep ; 14(3)2021 Mar 16.
Article in English | MEDLINE | ID: covidwho-1138315

ABSTRACT

We report a case of a 91-year-old Caucasian woman with a history of chronic lymphocytic leukaemia who developed acute hypoxic respiratory failure (AHRF) requiring intubation for less than 24 hours after receiving rasburicase. Laboratory workup was significant for methemoglobinemia and acute anaemia, and blood film demonstrated evidence of oxidative haemolysis with bite cells. The patient was given a presumptive diagnosis of glucose-6-phosphate dehydrogenase (G6PD) deficiency and was managed conservatively with successful resolution of AHRF and stabilisation of haemoglobin level. Seven days after admission, she passed away due to subsequent complications; hence, follow-up G6PD level could not be obtained. Haemolytic anaemia and methemoglobinemia in the setting of recent rasburicase administration should raise clinical suspicion for G6PD deficiency. In non-emergent cases, patients should be screened prior to receiving rasburicase regardless of risk factors. Because rasburicase is often needed emergently, patients at high risk of tumour lysis syndrome should be screened early for G6PD deficiency.


Subject(s)
Glucosephosphate Dehydrogenase Deficiency , Methemoglobinemia , Aged, 80 and over , Female , Glucosephosphate Dehydrogenase Deficiency/complications , Hemolysis , Humans , Methemoglobinemia/chemically induced , Methemoglobinemia/diagnosis , Methemoglobinemia/drug therapy , Urate Oxidase/adverse effects
18.
Nucleosides Nucleotides Nucleic Acids ; 40(5): 505-517, 2021.
Article in English | MEDLINE | ID: covidwho-1132307

ABSTRACT

COVID-19 has become a major public health problem since December, 2019 and no highly effective drug has been found until now. Numbers of infected people and deaths by COVID-19 are increasing every day worldwide, therefore self-isolation and protection are highly recommended to prevent the spread of the virus and especially to protect major risk groups such as the elderly population and people with comorbidities including diabetes, hypertension, cancer, cardiovascular diseases and metabolic syndrome. On the other hand, young people without any secondary disease have died by COVID-19 as well. In this study we compared two male patients infected by COVID-19 at the same age and one of them was diagnosed with G6PD deficiency. Both COVID-19 and G6PD deficiency enhance the risk of hemolysis and thrombosis. Serum biochemistry, hemogram and immunological parameters showed that risk of hemolysis and thrombosis may increase in the G6PD deficient patient infected by COVID-19.


Subject(s)
COVID-19/genetics , Glucosephosphate Dehydrogenase Deficiency/genetics , Glucosephosphate Dehydrogenase/genetics , Thrombosis/genetics , Adult , COVID-19/blood , COVID-19/complications , COVID-19/virology , Glucosephosphate Dehydrogenase Deficiency/blood , Glucosephosphate Dehydrogenase Deficiency/complications , Glucosephosphate Dehydrogenase Deficiency/pathology , Hemolysis/physiology , Humans , Male , Risk Factors , SARS-CoV-2/genetics , SARS-CoV-2/pathogenicity , Thrombosis/blood , Thrombosis/etiology , Thrombosis/virology
19.
Mol Biol Rep ; 48(3): 2973-2978, 2021 Mar.
Article in English | MEDLINE | ID: covidwho-1095719

ABSTRACT

The coronavirus disease 2019 (COVID-19) is until today a global health emergency. In an immense effort, effective drugs against COVID-19 are searched and intensive researches on possible repurposing of antiviral agents are performed. Since chloroquine (CQ) and hydroxychloroquine (HCQ) have shown in vitro anti- COVID-19 activities, the potential effect of CQ/HCQ to treat and/or prevent COVID-19 infection has caused global attention. However, concern regarding possible hemolysis in G6PD-deficient COVID-19 patients exists and for this reason, the association between HCQ and G6PD deficiency (G6PDD) is back in the limelight. This study aims to answer the question raised by Mastroianni et al. "Hydroxychloroquine: Culprit or Innocent Bystander in G6PD-Deficient Patients with COVID-19?", reporting all cases of HCQ in G6PD deficient COVID-19 patients published on PubMed (pubmed.ncbi.nlm.nih.gov), in addition to the Mastroianni's patient. In our opinion, after an accurate revision of these cases and responding the question raised by Mastroianni et al., we believe that it is difficult to reach a final verdict about the definitive role of HCQ in these patients. The COVID-19 pandemic has reopened attention on HCQ use and G6PDD. G6PD status is extremely important in modulating the level of reactive oxygen species and many cellular immune responses such as enhanced production of the pro-inflammatory cytokine and inflammasome activation. Since these processes are involved in COVID-19 infection, acute hemolytic anemia, a severe complication of the G6PDD, can occur in these patients. In this context, the role of HCQ, usually effective, safe, and well tolerated in G6PD deficient patients, must be redefined in these patients with COVID-19.As consequence, answering the question: "Hydroxychloroquine: Culprit or Innocent Bystander in G6PD-Deficient Patients with COVID-19?", we state that it is risky to believe that HCQ may be an "innocent bystander" in G6PD-deficient COVID-19 patients.


Subject(s)
Antiviral Agents , COVID-19 , Glucosephosphate Dehydrogenase Deficiency , Hydroxychloroquine , Adult , Aged , Antiviral Agents/adverse effects , Antiviral Agents/therapeutic use , COVID-19/complications , COVID-19/drug therapy , COVID-19/physiopathology , Glucosephosphate Dehydrogenase Deficiency/complications , Glucosephosphate Dehydrogenase Deficiency/physiopathology , Hemolysis , Humans , Hydroxychloroquine/adverse effects , Hydroxychloroquine/therapeutic use , Male , Middle Aged
20.
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
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