Steroids: What role in TTP?

Gorini, M; Luliri, P.A.trizia

doi:10.1016/0955-3886(92)90124-y

Cited by 3 publications

(2 citation statements)

References 37 publications

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“…However, there remain some patients who do not respond to plasma-exchange, even when combined with drugs which were known or supposed to be active in T T Pi.e., steroids, antiplatelet drugs, prostacyclin (4,5,6). Therefore, alternative and/or complementary therapies to plasmapheresis are needed.…”

Section: Introductionmentioning

confidence: 99%

Vincristine sulfate for the treatment of thrombotic thrombocytopenic purpura refractory to plasma‐exchange

Bobbio-Pallavicini¹,

Porta

Centurioni³

et al. 1994

European J of Haematology

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Among all the patients treated by the Italian Cooperative Group for TTP, we retrospectively reviewed the results obtained using vincristine (VCR) in 8 TTP patients (4 men and 4 women, average age: 39.25 years, range: 23–48) who did not respond to combined apheretic and pharmacologic treatment. All patients, after failing to respond to treatment, were started on VCR at the dose of 2 mg, i.v., once a week. Despite this treatment, 4 patients (50%) died 1, 7, 12 and 25 days after the first VCR dose, respectively. The other 4 patients who received VCR achieved complete remission 24, 30, 40 and 50 days from the beginning of the treatment. Total doses of VCR ranged from 2 to 6 mg in the deceased group, and from 6 to 14 mg in the cured patients. In our experience, VCR is a promising agent to treat TTP patients resistant to conventional plasma‐exchange and pharmacologic therapy.

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Section: Introductionmentioning

confidence: 99%

Vincristine sulfate for the treatment of thrombotic thrombocytopenic purpura refractory to plasma‐exchange

Bobbio-Pallavicini¹,

Porta

Centurioni³

et al. 1994

European J of Haematology

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“…During the years, an autoimmune hypothesis for TTP pathogenesis has often been proposed, 3 based on several clinical observations, including: (1) the superiority of plasma exchange over plasma infusion in the treatment of TTP, 4 which suggests the possibility that removing a possible humoral pathogenetic factor may be more effective than administering a factor that possibly is lacking; (2) the anecdotal finding of immune complexes and several autoantibodies [5][6][7][8][9][10][11][12] (Table 1) in TTP patients, which were considered as putative pathogenetic factors (reviewed by Porta et al 13 ); (3) the proven therapeutic efficacy of corticosteroids 14 and other immunosuppressant agents (e.g., vincristine 15 and azathioprine 16 ), which seem to be able to cure at least some TTP patients; and (4) the observation that TTP may occur in association with several autoimmune diseases, especially systemic lupus erythematosus (SLE; reviewed by Fessler 17 ).…”

Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

Autoimmunity in Thrombotic Thrombocytopenic Purpura

Porta

Caporali

Montecucco

2005

Semin Thromb Hemost

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In the last few years, an autoimmune hypothesis for the pathogenesis of thrombotic thrombocytopenic purpura (TTP) has been proposed often, with variable success because of inconsistent supporting data. We are now aware that at least one subgroup of TTP patients does present with pathogenic autoantibodies (i.e, anti-ADAMTS13); this group consequently is a putative candidate for a curative treatment including plasma exchange (still the cornerstone of TTP treatment), together with corticosteroids or other immunosuppressants. Furthermore, for patients not responding to or relapsing following plasma exchange, the use of stronger immunosuppression (e.g., with the use of the anti-CD20 monoclonal antibody rituximab) should be considered as appropriate. Conversely, few data are actually available regarding the complex relationship between TTP and the antiphospholipid (aPL) syndrome, as well as other autoimmune diseases. Indeed, a correct differential diagnosis should be done on the basis of both different clinical presentations and autoantibody profile. At present, the presence of aPL antibodies should give evidence against a diagnosis of TTP, even though we cannot exclude that aPL antibodies may, in a minority of patients, be associated with a primary endothelial damage ultimately resulting in overt TTP.

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Microangiopathies in pregnancy

Elayoubi

Donthireddy

Nemakayala³

2018

BMJ Case Reports

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Thrombotic thrombocytopenic purpura (TTP) is a potentially reversible, life-threatening medical emergency. We present a case of a 21-year-old female with evidence of haemolytic anaemia based on the presence of positive markers of haemolysis. Negative Coomb's test, thrombocytopenia and placental infarcts raised suspicion for a thrombotic microangiopathy. She was diagnosed with TTP and managed with emergency plasma exchange. Her recovery was immediate.A presumptive diagnosis of TTP should be based on the presence of microangiopathic haemolytic anaemia with thrombocytopenia and plasma exchange should be initiated while complete work up is pending. Using the regular pentad solely for diagnosis of TTP will lead to underdiagnosis of many cases and should be avoided.Several microangiopathies can be seen during pregnancy including TTP/atypical haemolytic uraemic syndrome, HELLP syndrome, pre-eclampsia, disseminated intravascular coagulopathy and antiphospholipid antibody syndrome. Distinction between each type will be the focus of our discussion as treatment decisions differ accordingly.

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Steroids: What role in TTP?

Cited by 3 publications

References 37 publications

Vincristine sulfate for the treatment of thrombotic thrombocytopenic purpura refractory to plasma‐exchange

Vincristine sulfate for the treatment of thrombotic thrombocytopenic purpura refractory to plasma‐exchange

Autoimmunity in Thrombotic Thrombocytopenic Purpura

Microangiopathies in pregnancy

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