scholarly journals COVID-19 vaccine (mRNA BNT162b2) and COVID-19 Infection-Induced Thrombotic Thrombocytopenic Purpura in Adolescents

2021 ◽  
Author(s):  
Luna Vorster ◽  
Susan Kirk ◽  
Eyal Muscal ◽  
Jenny Despotovic ◽  
Clay Cohen ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
De Novo ◽  
Thrombocytopenic Purpura ◽  
Adolescent Patients ◽  
Relapsed Disease

The mRNA COVID-19 vaccine and COVID-19 infection caused by the SARS-CoV-2 virus may be immunologic triggers for the development of thrombotic thrombocytopenic purpura (TTP). There is not yet literature that discusses TTP induced by COVID-19 vaccination or infection in pediatric or adolescent patients. We describe 4 adolescents presenting with TTP (both de novo and relapsed disease) following administration of the Pfizer COVID-19 vaccine or after COVID-19 infection. Our observations demonstrate that the Pfizer-BioNTech mRNA vaccine and COVID-19 infection can act as triggers for the development/relapse of both congenital and acquired TTP.

scholarly journals De Novo Mutation of the ADAMTS13 Gene with Mesenteric Ischemia in an Infant with Congenital Thrombotic Thrombocytopenic Purpura

2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Ibrahim Alharbi ◽  
Sarah Alqarni ◽  
Wed Khayyat ◽  
Amirah Almatrafi
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Autosomal Recessive ◽  
De Novo ◽  
Autosomal Recessive Disease ◽  
Rare Complication ◽  
Homozygous Deletion ◽  
De Novo Mutation ◽  
Histopathological Analysis ◽  
Thrombocytopenic Purpura ◽  
Congenital Thrombotic Thrombocytopenic Purpura

Introduction. Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare autosomal recessive disease characterized by ADAMTS13 deficiency or a severe decrease in its activity that is caused by homozygous or combined heterozygous mutations in its encoding gene. Here, we describe a de novo genetic mutation of the ADAMTS13 gene and a rare complication of cTTP in a neonate. Case Presentation. A full-term baby boy developed tachypnea, bradycardia, and oxygen desaturation at 2 h of life and was shifted to the newborn intensive care unit. He was oliguric in the first 24 h of life and had one episode of epistaxis. Blood-stained urine was observed in the urine catheter, and a coffee-ground-colored fluid was drained from the orogastric tube. Histopathological analysis revealed gastric perforation with features of ischemic insult. On day 8, genetic analysis confirmed the diagnosis of autosomal recessive familial thrombotic thrombocytopenic purpura and revealed a unique homozygous deletion mutation on exon 23 of ADAMTS13: c.2883del p.(Cys962Alafs ∗ 3). Conclusion. cTTP is a rare life-threatening autosomal recessive disease with a high mortality rate. Early detection and initiation of aggressive treatment with plasma infusion could be a life-saving strategy in such cases.

Newly-Diagnosed Versus Non-Initial Episodes of Thrombotic Thrombocytopenic Purpura: A Comparison of Presenting Clinical Characteristics and Response to Treatment.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2287-2287
Author(s):  
Alberto Alvarez-Larrán ◽  
Julio del Río-Garma ◽  
Josep Muncunill ◽  
Javier de la Rubia ◽  
Manuel Hernández-Jodrá ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Clinical Characteristics ◽  
De Novo ◽  
Remission Rate ◽  
Response To Treatment ◽  
Newly Diagnosed ◽  
Biological Profile ◽  
Lower Serum ◽  
Thrombocytopenic Purpura ◽  
Biological Features

Abstract The remission rate with plasma exchange (PE) in thrombotic thrombocytopenic purpura (TTP) exceeds the 80%, but the disease relapses in up to 20–30% of the cases. Clinical characteristics and response to treatment of relapsed TTP is not well defined. The objective of the present study was to analyze the presenting clinical and biological features of relapsed TTP as compared with de novo TTP in a series of 102 TTP episodes (70 de novo and 32 relapses) followed prospectively and treated by daily PE and corticosteroids according to a homogeneous protocol. In comparison with de novo TTP, relapsed TTP episodes showed a higher Hb level (122 g/L versus 91 g/L, p<0.001) and lower serum LDH (2.2 versus 4.5 fold above the upper limit of normality, p<0.001). Neurological symptoms and fever were less frequent in patients with relapsed TTP than in patients with de novo TTP. There were no statistically significant differences in the percentage of patients with a severe deficit of ADAMTS13 activity (72% in de novo TTP versus 81% in relapsed TTP, p not significant) or the presence of inhibitory autoantibodies (87% versus 81%, p not significant). Patients with relapsed TTP needed fewer PE sessions (5 versus 10, p= 0.02) and a smaller volume of plasma (221 ml/kg versus 468 ml/kg, p=0.004) to achieve remission than those with de novo TTP. There were no significant differences in the rate of recrudescence under treatment, the need for complementary treatments (e.g. rituximab or splenectomy) or the frequency of refractoriness to PE therapy. In conclusion, relapsed TTP presents with a milder clinical and biological profile and responds easier to PE than de novo TTP.

Normal Prostacyclin-Like Activity in Thrombotic Thrombocytopenic Purpura

1981 ◽  
Vol 46 (02) ◽  
pp. 571-571 ◽  
Author(s):  
M Pini ◽  
C Manotti ◽  
R Quintavalla ◽  
A G Dettori
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura

The Pathogenesis of Thrombotic Thrombocytopenic Purpura

1979 ◽  
Author(s):  
H. C. Kwaan
Keyword(s):  
Plasminogen Activator ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Degradation Products ◽  
Vascular Lesions ◽  
Local Defect ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Fibrin Degradation Products ◽  
Severity Of The Disease ◽  
Circulating Levels

The vascular lesions with microthrombi were studied in 12 patients with thrombotic thrombocytopenic purpura (TTP), diagnosed by the characteristic clinical and laboratory findings and confirmed histologically in each case. While defibrination was not observed, and with only minimal changes in the circulating levels of fibrinogen, fibrin degradation products and plasminogen activator, the microthrombotic lesion was invariably present. Immunofluorescent and histochemical studies indicated that both platelet and fibrin were present in the microthrombi with the platelet components dominant in many cases. Using the fibrin slide method, plasminogen activator was demonstrated in the uninvolved blood vessels but totally absent in the vessels occluded by microthrombi. in contrast, fibrinolysis is always present in the vessels afflicted with other types of thrombosis, such as the microthrombi in disseminated intravascular coagulation. Since circulating fibrinolytic activity was normal in TTP, the absence of vascular fibrinolysis is a local defect due to either inhibition by the platelet deposits or by local vascular damage. The inability of thrombolysis may explain the absence of systemic defibrination and the severity of the disease.

Thrombotic Thrombocytopenic Purpura: Mechanism for Effectiveness of Plasmapheresis

1979 ◽  
Author(s):  
J. G. Kelton ◽  
P. B. Neame ◽  
I. Walker ◽  
A. G. Turpie ◽  
J. McBride ◽  
...  
Keyword(s):  
Platelet Count ◽  
Thrombotic Thrombocytopenic Purpura ◽  
The Other ◽  
Unknown Etiology ◽  
Normal Range ◽  
Antiplatelet Antibody ◽  
Thrombocytopenic Purpura ◽  
Serious Illness ◽  
Normal Platelet ◽  
Very High

Thrombotic thrombocytopenic purpura (TTP) is a rare but serious illness of unknown etiology. Treatment by plasmapheresis has been reported to be effective but the mechanism for benefit is unknown. We have investigated the effect of plasmapheresis in 2 patients with TTP by quantitating platelet associated IgG (PAIgG) levels prior to and following plasmapheresis. Both patients had very high levels of PAIgG at presentation (90 and A8 fg IgG/platelet respectively, normal 0-5). in both, the PAIgG levels progressively fell to within the normal range and the platelet count rose following plasmapheresis. One patient remained in remission with normal platelet counts and PAIgG levels. The other relapsed after plasmapheresis and the PAIgG level rose prior to the fall in platelet count. Plasmapheresis was repeated and resulted in normalization of both the platelet count and PAIgG level. It is suggested that plasmapheresis removes antiplatelet antibody or immune complexes which may be of etiological importance in this illness.

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