Successful treatment with rituximab for acute refractory thrombotic thrombocytopenic purpura related to acquired ADAMTS13 deficiency: A pediatric report and literature review

Harambat, Jer ome; Lamireau, Delphine; Delmas, Yahsou; Ryman, Anne; Llanas, Brigitte; Brissaud, Olivier

doi:10.1097/pcc.0b013e3181e89f8f

Cited by 16 publications

(13 citation statements)

References 25 publications

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“…ADAMTS-13 functional activity was assessed by full von Willebrand Factor length technique as reported [17]. Activity <5 % was defined as a severe deficiency (activity >50 % is considered normal).…”

Section: Methodsmentioning

confidence: 99%

“…Activity <5 % was defined as a severe deficiency (activity >50 % is considered normal). ADAMTS-13 inhibitor was determined using a neutralizing inhibitor approach as previously described [17] and anti-ADAMTS-13 IgG was determined by ELISA (TECHNOZYME ADAMTS-13 INH, Technoclone, Austria). Our laboratory cut-off for a positive value was >35 IU/mL.…”

Section: Methodsmentioning

confidence: 99%

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Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease

Delmas

Helou

Chabanier

et al. 2015

BMC Pregnancy Childbirth

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BackgroundThrombotic thrombocytopenic Purpura (TTP) defined as ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin type 1 domain 13) activity <10 % is a rare aetiology of thrombocytopenia during pregnancy, although the precise incidence is unknown. During pregnancy, the diagnosis of TTP is crucial as it has high feto-maternal morbidity-mortality and requires urgent plasma exchange. The purpose of this study was to assess the incidence of TTP retrospectively and to describe case presentations and follow-up.MethodsA monocentric retrospective study (2008–2009) was conducted among pregnant women followed in a tertiary care obstetrical unit who experienced at least one episode of severe thrombocytopenia (platelets ≤75 G/L) during 2008 and 2009. In cases of uncertain aetiology of thrombocytopenia, ADAMTS-13 activity was assessed by the full length technique.ResultsAmong 8,908 deliveries over the 2 year period, 79 women had a platelet count nadir ≤75 G/L. Eighteen had a known aetiology of thrombocytopenia and 11 were lost to follow-up. Among 50 remaining patients, ADAMTS-13 activity was undetectable (<5 %) in 4, consistent with the diagnosis of TTP. Platelet count spontaneously normalized in 3 patients after delivery. None presented focal cerebral involvement. Three of the four, who were primipara patients, had a sustained severe deficiency in the absence of anti-ADAMTS-13 antibodies, and ADAMTS-13 gene sequencing indicated a constitutive deficiency. The fourth, a multipara patient, had an acquired, auto-immune TTP. Placental pathology in the three primipara patients showed severe and non-specific ischemic lesions. Two patients lost their babies shortly after birth. In subsequent pregnancies in these two patients, prophylactic plasma infusion initiated early with increasing volume throughout pregnancy prevented TTP relapse, improved placental pathology, and led to normal delivery.ConclusionsThe prevalence of TTP among thrombocytopenic pregnant women is high, up to 5 % in a tertiary unit. Platelet count normalization after delivery does not eliminate TTP. Clinicians should be aware of TTP during pregnancy, and, even if assessed retrospectively, ADAMTS-13 assessment is of particular importance for identifying patients with congenital TTP. In these patients, preventive plasma infusion and/or exchange can dramatically improve foetal prognosis, resulting in successful childbirth.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease

Delmas

Helou

Chabanier

et al. 2015

BMC Pregnancy Childbirth

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show abstract

“…Recently, there have been many successful cases [67][68][69], and to date, no significant adverse effects have been reported. In our registry, only one childhood TTP patient (7 years old) with acquired TTP with ADAMTS13:INH was successfully treated with PE followed by rituximab, as shown in table II.…”

Section: Treatment Of Acquired Thrombotic Thrombocytopenic Purpuramentioning

confidence: 94%

Paradigm shift of childhood thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency

2012

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“…Acquired autoimmune TTP very rarely occurs in children and adolescents and only case and short series reports have been published on this subject [82][83][84][85][86]. Like in adults, TPE (usually associated with steroids) is the reference curative treatment [85].…”

Section: Management Of Acquired Autoimmune Thrombotic Thrombocytopenimentioning

confidence: 96%

Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura

Coppo

Veyradier

2012

La Presse Médicale

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Successful treatment with rituximab for acute refractory thrombotic thrombocytopenic purpura related to acquired ADAMTS13 deficiency: A pediatric report and literature review

Cited by 16 publications

References 25 publications

Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease

Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease

Paradigm shift of childhood thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency

Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura

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