HLA-DRB1*11 is a strong risk factor for acquired thrombotic thrombocytopenic purpura in children

Joly, Bérangère S.; Loiseau, Pascale; Darmon, Michaël; Leblanc, Thierry; Chambost, Hérvè; Fouyssac, Fanny; Guigonis, Vincent; Harambat, Jérôme; Stépanian, Alain; Coppo, Paul; Veyradier, Agnès

doi:10.3324/haematol.2019.241968

Cited by 9 publications

(1 citation statement)

References 16 publications

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“…The pathogenesis of an autoimmune disorder is considered to be multifactorial and a strong association between the HLA region, the generation of autoantibodies against self-antigens, and autoimmune diseases has been described [30]. In children, HLA-DRB1*11 may be a susceptibility factor for iTTP, while HLA-DRB1*04, when not associated with HLA-DQB1*03, may be protective [31]. Pediatricians should also be aware of the occurrence of another systemic autoimmune disease many years following remission [13,32].…”

Section: Prognosismentioning

confidence: 99%

The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review

Joseph

Joly

Picod

et al. 2023

JCM

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Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy (TMA) related to a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cleaving protease. This deficiency is often immune-mediated (iTTP) and related to the presence of anti-ADAMTS13 autoantibodies that enhance its clearance or inhibit its VWF processing activity. iTTP management may be challenging at extreme ages of life. International cohorts of people with TTP report delayed diagnoses and misdiagnoses in children and elderly people. Child-onset iTTP shares many features with adult-onset iTTP: a female predominance, an idiopathic presentation, and the presence of neurological disorders and therapeutic strategies. Long-term follow-ups and a transition from childhood to adulthood are crucial to preventing iTTP relapses, in order to identify the occurrence of other autoimmune disorders and psychosocial sequelae. In contrast, older iTTP patients have an atypical clinical presentation, with delirium, an atypical neurological presentation, and severe renal and cardiac damages. They also have a poorer response to treatment and prognosis. Long-term sequelae are highly prevalent in older patients. Prediction scores for iTTP diagnoses are not used for children and have a lower sensitivity and specificity in patients over 60 years old. ADAMTS13 remains the unique biological marker that is able to definitely confirm or rule out the diagnosis of iTTP and predict relapses during follow-ups.

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Section: Prognosismentioning

confidence: 99%

The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review

Joseph

Joly

Picod

et al. 2023

JCM

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Familial autoimmunity and risk of developing immune thrombocytopenia and Evans syndrome

Lozano Chinga,

Bussel,

Fluchel

et al. 2024

Pediatric Blood & Cancer

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BackgroundImmune thrombocytopenia (ITP) and Evans syndrome (ES) are manifestations of immune dysregulation. Genetic variants in immune‐related genes have been identified in patients with ITP and especially ES. We aimed to explore familial autoimmunity in patients with ITP and ES to understand possible contributions to chronicity.ProcedureWe assessed family history in two ways: via patient report for ITP and ES and by population‐based analysis using the Utah Population Database (UPDB) for ITP. A total of 266 patients with ITP and 21 patients with ES were identified via chart review, and 252 of the 266 patients with ITP were also identified in the UPDB.ResultsChart review showed familial autoimmunity in 29/182 (15.9%) and 25/84 (29.8%) of patients with newly diagnosed+persistent (nd+p) ITP and chronic ITP (cITP), respectively, (p = .009). The UPDB analysis revealed that autoimmunity in relatives of patients with nd+pITP was higher than in relatives of controls (odds ratio [OR]: 1.69 [1.19–2.41], p = .004), but was not significantly increased in relatives of patients with cITP (OR 1.10 [0.63–1.92], p = .734). Incomplete family history in medical records likely contributed to the observed discrepancy.ConclusionsThe findings suggest that familial autoimmunity may have a stronger association with the development of ITP rather than its duration. Twelve (57.1%) patients with ES reported autoimmunity in their relatives. UPDB analysis was omitted due to the small number of patients with ES. The use of population databases offers a unique opportunity to assess familial health and may provide clues about contributors to immune dysregulation features within families.

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The impact of HLA-DRB1 alleles in a Hellenic, Pediatric-Onset Multiple Sclerosis cohort: Implications on clinical and neuroimaging profile

Skarlis,

Markoglou,

Gontika

et al. 2024

Neurol Sci

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HLA-DRB1*11 is a strong risk factor for acquired thrombotic thrombocytopenic purpura in children

Abstract: HLA-DRB111 is a strong risk factor for acquired thrombotic thrombocytopenic purpura in children. Running head: HLA-DRB111 and acquired TTP in children.

Cited by 9 publications

References 16 publications

The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review

The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review

Familial autoimmunity and risk of developing immune thrombocytopenia and Evans syndrome

The impact of HLA-DRB1 alleles in a Hellenic, Pediatric-Onset Multiple Sclerosis cohort: Implications on clinical and neuroimaging profile

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HLA-DRB1*11 is a strong risk factor for acquired thrombotic thrombocytopenic purpura in children

Abstract: HLA-DRB1*11 is a strong risk factor for acquired thrombotic thrombocytopenic purpura in children. Running head: HLA-DRB1*11 and acquired TTP in children.

Cited by 9 publications

References 16 publications

The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review

The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review

Familial autoimmunity and risk of developing immune thrombocytopenia and Evans syndrome

The impact of HLA-DRB1 alleles in a Hellenic, Pediatric-Onset Multiple Sclerosis cohort: Implications on clinical and neuroimaging profile

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Abstract: HLA-DRB111 is a strong risk factor for acquired thrombotic thrombocytopenic purpura in children. Running head: HLA-DRB111 and acquired TTP in children.