Ten patient stories illustrating the extraordinarily diverse clinical features of patients with thrombotic thrombocytopenic purpura and severe ADAMTS13 deficiency

George, James N.; Chen, Qiaofang; Deford, Cassie C.; Al-Nouri, Zayd L.

doi:10.1002/jca.21248

Cited by 47 publications

(36 citation statements)

References 39 publications

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“…In a cohort of 31 TTP patients with undetectable (<5%) ADAMTS13 activity, 22 patients (71%) had a positive ANA and 3 (9.7%) had a positive anti-dsDNA at the time of diagnosis [14]. According to data from the Oklahoma TTP-HUS Registry, 4 out of 72 patients with severe ADAMTS13 deficiency were diagnosed with SLE, 2 concurrently with TTP, 1 patient 5 months after and 1 6 years after the initial episode of TTP [15].…”

Section: Discussionmentioning

confidence: 99%

A 35-year-old woman with influenza A-associated thrombotic thrombocytopenic purpura

Jonsson

Hammenfors²,

Oppegaard³

et al. 2015

Blood Coagulation &Amp; Fibrinolysis

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A previously healthy 35-year-old woman presented with severe thrombotic thrombocytopenic purpura (TTP) affecting several organs and concomitant influenza A infection. On admission to hospital, haemoglobin was 5.4 g/dl, platelet count 6 × 10/l, Schistocyte count in peripheral blood 5%, and throat swab positive for influenza A RNA. The patient was treated with antiviral medication and transfusions of fresh frozen plasma before plasma exchange therapy with excellent response. Plasmaphereses were attenuated after 5 days, resulting in TTP relapse 3 days later. A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) protein was very low (<0.04 U/l) and anti-ADAMTS13 elevated (>2 U/l), consistent with acquired TTP. Platelet counts normalized after five additional plasma exchanges and oral corticosteroids. Antinuclear antibodies and subgroup anti-Ro/Sjögren's syndrome A antigen (SSA) were detected in serum and have remained borderline-elevated, although evaluation during TTP, recovery and follow-up have lacked clinical manifestations of connective tissue disease. Influenza A infection induced production of ADAMTS13 inhibitor, which resulted in TTP in a patient with circulating antinuclear antibodies, lacking other manifestations of connective tissue disease.

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Section: Discussionmentioning

confidence: 99%

A 35-year-old woman with influenza A-associated thrombotic thrombocytopenic purpura

Jonsson

Hammenfors²,

Oppegaard³

et al. 2015

Blood Coagulation &Amp; Fibrinolysis

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“…ADAMTS-13 sumažėjimas 10 proc. normalaus jo aktyvumo padeda diagnozuoti įgytą TTP, taip pat parodo ligos atkryčio riziką [25,26]. Patofiziologija 1982 m. neįprastai dideli Vilebrando faktoriaus multimerai buvo rasti lėtine įgimta TTP sergantiems pacientams.…”

Section: Trombinė Trombocitopeninė Purpuraunclassified

Trombinių Mikroangiopatijų Sindromų Patogenetiniai Mechanizmai, Diferencinė Diagnostika Ir Gydymas

Čerkauskaitė

Ašakienė

Čerkauskienė

et al. 2015

Medicinos Teorija Ir Praktika

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Reikšminiai žodžiai: trombinės mikroangiopatijos, HUS, aHUS, komplementas, ADAMTS-13. Trombinės mikroangiopatijos (TMA) – tai grupė sindromų, pasireiškiančių trombocitopenija, mikroangiopatine hemolizine anemija ir smulkiųjų kraujagyslių okliuzija. TMA, priklausomai nuo ligą sukėlusios priežasties, skiriamos į hemolizinį ureminį sindromą (HUS), susijusį su Shiga toksinu (šigatoksinu), atipinį hemolizinį sindromą (aHUS), trombinę trombocitopeninę purpurą (TTP) ir kitas ligas, įskaitant metabolizmo, vaistų, krešėjimo sutrikimų sukeltą TMA. Sporadinį ar epideminį HUS sukelianti E. coli, gaminanti Shiga toksiną, yra svarbi vaikų ūminio inkstų pažeidimo priežastis visame pasaulyje, tuo tarpu TTP labiau būdinga suaugusiesiems ir siejama su specifinės Vilebrando faktorių skaldančios proteazės, dar vadinamos ADAMTS-13, stoka. Svarbiausią aHUS patogenezės grandį sudaro alternatyvaus komplemento kelio reguliacijos sutrikimas, dalyvaujant genetiniams ir aplinkos veiksniams. TMA sindromų patogenetinių kelių ir klinikinio pasireiškimo įvairovė ir konvergencija (supanašėjimas) neretai apsunkina pirminę diagnostiką ir diferenciaciją, o besikeičiantys tyrimų rezultatai tampa iššūkiu kuriant tinkamiausias TMA gydymo gaires. Šiame apžvalginiame straipsnyje pateikiama skirtingų TMA sindromų charakteristika, labiausiai atkreipiant dėmesį į molekulinių patologinių mechanizmų ir klinikinių simptomų sąsają, kartu apžvelgiant naujausius literatūroje aprašytus diagnostikos ir gydymo metodus. Vis dėlto ankstyvas TMA gydymas apsiriboja empiriškai taikoma pakaitine plazmos terapija (išskyrus vaikų Shiga toksino sukeltą HUS (STEC-HUS)), komplemento C5 inhibitoriaus ekulizumabo vartojimu aHUS atveju ar inkstų, inkstų/kepenų transplantacija dėl HUS hemodializuojamiems pacientams.

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“…5 Other reports of ADAMTS13 deficiency in non-TMA conditions, for example, fulminant hepatic failure and systemic infections, raise questions regarding the diagnostic utility of ADAMTS13 testing and highlight the importance of interpreting the results within the appropriate clinical context. 17 Functional assays to measure the activity of ADAMTS13 are based on the detection of cleaved VWF multimers or recombinant VWF peptides in the presence of patient plasma. These tests can also be used to identify inhibitory autoantibodies directed against the enzyme.…”

Section: Ttp Pathogenesis and Diagnosismentioning

confidence: 99%

Thrombotic microangiopathy and indications for therapeutic plasma exchange

Adamski

2014

Hematology

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Thrombotic microangiopathy (TMA) is a clinicopathological condition associated with a wide variety of medical conditions. TMA is classically characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombi that cause end-organ damage. The most prominent diagnoses associated with TMA are thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Although TTP and HUS can have similar clinical and laboratory features and are often lumped together as a combined entity referred to as "TTP/HUS," the pathologic processes causing TMA and optimal therapies for these conditions are different. Empiric use of therapeutic plasma exchange (TPE) in the setting of TMA is common. The high risk of morbidity and mortality associated with some causes of TMA justify rapid institution of this relatively low-risk procedure. However, many causes of TMA do not respond to TPE and prolonged courses of exchange in the absence of an underlying diagnosis may cause a detrimental delay in appropriate medical therapy. The American Society of Apheresis has published guidelines for the use of TPE for several distinct conditions associated with TMA. This list is not comprehensive and the use of TPE for other causes of TMA may be considered if the mechanism of the underlying disease process provides a clear rationale for this intervention. Learning Objective• To acquire information and clinical tools to augment clinical judgement regarding the use of TPE in the setting of TMA

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Ten patient stories illustrating the extraordinarily diverse clinical features of patients with thrombotic thrombocytopenic purpura and severe ADAMTS13 deficiency

Cited by 47 publications

References 39 publications

A 35-year-old woman with influenza A-associated thrombotic thrombocytopenic purpura

A 35-year-old woman with influenza A-associated thrombotic thrombocytopenic purpura

Trombinių Mikroangiopatijų Sindromų Patogenetiniai Mechanizmai, Diferencinė Diagnostika Ir Gydymas

Thrombotic microangiopathy and indications for therapeutic plasma exchange

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