Literature DB >> 25576335

Does corticosteroid treatment cause prolonged recovery and increased total bilirubin level in severe ADAMTS-13-deficient TTP patient?

Zeynel Abidin Sayiner1, Didar Yanardag Acik2, Mehmet Yilmaz2, Salih Subari3, Ayse Ozlem Mete4, M Sinan Dai5.   

Abstract

A 41-year-old female patient complaining of fatigue, headache, mild confusion, and rush on her lower extremities was admitted to our emergency department. Laboratory tests revealed that he had anemia, thrombocytopenia, and increased levels of indirect bilirubin and lactic dehydrogenase (LDH) in blood tests. Direct and indirect Coombs tests were negative, and fragmented erythrocytes were observed in peripheral blood smears. The patient was diagnosed with thrombotic thrombocytopenic purpura (TTP). The best supportive care was provided. Therapeutic plasma exchange (TPE) and 1 mg/kg methylprednisolone treatments were administered. On the 10th day of treatment, LDH level and fragmented red blood cells in peripheral blood smear were decreased, but his direct and indirect bilirubin levels increased despite the fact that he was treated with 1 mg/kg methylprednisolone and TPE. The patient had severe ADAMTS-13 deficiency. After discontinued steroids treatment, his bilirubin level normalized within 4 days. On the 4th day after bilirubin level normalized, vincristine treatment was administered. TPE was also continued. There was no consensus about the optimal schedule for discontinuing plasmapheresis therapy, and also we observed total bilirubin level improvement with discontinued corticosteroid treatment. In this case, corticosteroid treatment was linked with the increase of total bilirubin level in severe ADAMTS-13-deficient TTP patient.

Entities:  

Keywords:  Corticosteroid treatment; Hemolysis; Severe ADAMTS-13 deficiency; TPE; TTP

Mesh:

Substances:

Year:  2015        PMID: 25576335     DOI: 10.1007/s00508-014-0691-5

Source DB:  PubMed          Journal:  Wien Klin Wochenschr        ISSN: 0043-5325            Impact factor:   1.704


  17 in total

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Authors:  Sarah L Allford; Beverley J Hunt; Peter Rose; Samuel J Machin
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Review 2.  Thrombotic microangiopathies.

Authors:  Joel L Moake
Journal:  N Engl J Med       Date:  2002-08-22       Impact factor: 91.245

Review 3.  ADAMTS-13 assays in thrombotic thrombocytopenic purpura.

Authors:  F Peyvandi; R Palla; L A Lotta; I Mackie; M A Scully; S J Machin
Journal:  J Thromb Haemost       Date:  2010-01-21       Impact factor: 5.824

4.  Improved survival with plasma exchange in patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.

Authors:  P N Lara; T L Coe; H Zhou; L Fernando; P V Holland; T Wun
Journal:  Am J Med       Date:  1999-12       Impact factor: 4.965

Review 5.  HUS and TTP: variable expression of a single entity.

Authors:  G Remuzzi
Journal:  Kidney Int       Date:  1987-08       Impact factor: 10.612

6.  Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group.

Authors:  G A Rock; K H Shumak; N A Buskard; V S Blanchette; J G Kelton; R C Nair; R A Spasoff
Journal:  N Engl J Med       Date:  1991-08-08       Impact factor: 91.245

7.  Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients.

Authors:  W R Bell; H G Braine; P M Ness; T S Kickler
Journal:  N Engl J Med       Date:  1991-08-08       Impact factor: 91.245

Review 8.  Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura.

Authors:  P Ruggenenti; M Noris; G Remuzzi
Journal:  Kidney Int       Date:  2001-09       Impact factor: 10.612

9.  Twice-daily plasma exchange for patients with refractory thrombotic thrombocytopenic purpura: the experience of the Oklahoma Registry, 1989 through 2006.

Authors:  Loan Nguyen; Xiaoning Li; Deanna Duvall; Deirdra R Terrell; Sara K Vesely; James N George
Journal:  Transfusion       Date:  2007-11-19       Impact factor: 3.157

Review 10.  How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome.

Authors:  Marie Scully; Tim Goodship
Journal:  Br J Haematol       Date:  2014-01-06       Impact factor: 6.998

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