Stroke and myocardial infarction in hereditary thrombotic thrombocytopenic purpura: similarities to sickle cell anemia

Borogovac, Azra; George, James N.

doi:10.1182/bloodadvances.2019000959

Cited by 19 publications

(27 citation statements)

References 19 publications

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“…This is similar to observations in all patients with hereditary TTP, in whom myocardial infarction is much less frequent than TIA/stroke. 16,24,33 3.4 Diagnosis…”

Section: Acquired Ttpmentioning

confidence: 99%

Recognizing and managing hereditary and acquired thrombotic thrombocytopenic purpura in infants and children

Siddiqui

Journeycake

Borogovac

et al. 2021

Pediatric Blood & Cancer

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We describe how infants and children with hereditary and acquired autoimmune thrombotic thrombocytopenic purpura (TTP) initially present and how they can be promptly diagnosed and effectively managed. These are uncommon disorders that are commonly misdiagnosed and can be rapidly fatal. TTP is caused by a severe deficiency of the plasma protease, A disintegrin and Metalloprotease with a ThromboSpondin type 1 motif, member 13 (ADAMTS13). Measurement of ADAMTS13 activity is becoming easily accessible. A common presentation of hereditary TTP is neonatal severe hemolysis and hyperbilirubinemia. However, the median age of diagnosis is not until 5.5 years. Plasma is effective treatment for exacerbations and for prophylaxis. Plasma may be replaced by recombinant ADAMTS13 when it becomes available. Acquired TTP is more frequent in older children, in whom it is more common in girls and is commonly associated with systemic lupus erythematosus. For acquired TTP, plasma exchange and immunosuppression are the current treatment for acute episodes; caplacizumab is now commonly used in adults and may replace plasma exchange.

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“…This is similar to observations in all patients with hereditary TTP, in whom myocardial infarction is much less frequent than TIA/stroke. 16,24,33 3.4 Diagnosis…”

Section: Acquired Ttpmentioning

confidence: 99%

Recognizing and managing hereditary and acquired thrombotic thrombocytopenic purpura in infants and children

Siddiqui

Journeycake

Borogovac

et al. 2021

Pediatric Blood & Cancer

Self Cite

View full text Add to dashboard Cite

show abstract

“…The fresh frozen plasma was then effective in reducing back pain. It is difficult to interpret these findings correctly and we rather doubt having proof of smoldering TTP activity reacting exclusively to replenishment of ADAMTS13 via plasma infusion ( 5 , 6 , 31 ). There seems to be a significant placebo effect, especially in this patient with a huge language barrier and fear of deportation to his home country after possible failure of asylum procedures and expiration of residency permit.…”

Section: Discussionmentioning

confidence: 96%

“…Congenital TTP (or Upshaw-Schulman syndrome) is a very rare, autosomal recessive disease, which is caused by homozygous or compound heterozygous ADAMTS13 mutations (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). The diagnosis is confirmed by evidence of a persistent severe ADAMTS13 deficiency (<10%), absence of an ADAMTS13 inhibitor and presence of biallelic mutations of ADAMTS13.…”

Section: Discussionmentioning

confidence: 99%

Diagnosis of Hereditary TTP Caused by Homozygosity for a Rare Complex ADAMTS13 Allele After Salmonella Infection in a 43-Year-Old Asylum Seeker

et al. 2021

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A 43-year-old Armenian patient was diagnosed with salmonella infection and thrombotic microangiopathy (TMA). The clinical course was benign with resolution of all laboratory alterations after antibiotic treatment. Constantly deficient ADAMTS13 activity without ADAMTS13 inhibitors and evidence of homozygosity for a rare complex ADAMTS13 allele led to the diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP). Half-life of ADAMTS13 after plasma infusion was calculated (27,6h) and double blinded plasma infusion as well as ergometric exercise with and without prior plasma infusion undertaken to investigate suspected smoldering TTP activity.

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“…The case reports were identified by searching MEDLINE and PubMed from 2001 (when ADAMTS13 was first described) to March 20, 2020. This search was initially performed in 2019 3 and updated for this study. We identified 80 articles describing 180 patients.…”

Section: Frequency and Severity Of Pregnancy Complications In Women Wmentioning

confidence: 99%

Frequency and severity of pregnancy complications in women with hereditary thrombotic thrombocytopenic purpura

2020

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Stroke and myocardial infarction in hereditary thrombotic thrombocytopenic purpura: similarities to sickle cell anemia

Abstract: Key Points Stroke is common in hereditary thrombotic thrombocytopenic purpura (TTP) patients, occurring in 25% to 31% of patients. In hereditary TTP patients, the age of occurrence of first stroke (median, 19 years) is similar to that for sickle cell anemia patients.

Cited by 19 publications

References 19 publications

Recognizing and managing hereditary and acquired thrombotic thrombocytopenic purpura in infants and children

Recognizing and managing hereditary and acquired thrombotic thrombocytopenic purpura in infants and children

Diagnosis of Hereditary TTP Caused by Homozygosity for a Rare Complex ADAMTS13 Allele After Salmonella Infection in a 43-Year-Old Asylum Seeker

Frequency and severity of pregnancy complications in women with hereditary thrombotic thrombocytopenic purpura

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