Literature DB >> 25332586

Cryosupernatant and Immunosuppression as Effective Alternative Therapies for TTP in Three Pediatric SLE Patients.

Pragathesh Palaniappan1, Arul Premanand Lionel1, Indira Agarwal1, Sarah Mathai1, Leni Mathew1, Sanwar Agarwal2, Purva Keni2, Raju Khubchandani2, Sathish Kumar1.   

Abstract

Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) very rarely present simultaneously and pose a diagnostic and therapeutic dilemma to the physician. Prompt diagnosis and management with plasma exchange and immunosuppression is life-saving. To describe the effectiveness of cryosupernatant and steroids in pediatric SLE with TTP. We describe three children aged 12-14 years with SLE who were diagnosed with TTP based on fever, CNS manifestations, ANA, anti-dsDNA, anti-sm positivity, hypocomplementemia, and microangiopathic anemia with thrombocytopenia. All three children were managed with cryosupernatant and steroids without plasmapheresis. All children improved with cryosupernatant and steroids. All attained remission within 10 days. They were doing well at last follow up without relapse or flare. Cryosupernatant and steroids may be an effective therapy for Thrombotic thrombocytopenic purpura with systemic lupus erythematosus.

Entities:  

Keywords:  Cryosupernatant; Pediatric systemic lupus erythematosus; Thrombotic thrombocytopenic purpura

Year:  2014        PMID: 25332586      PMCID: PMC4192230          DOI: 10.1007/s12288-014-0338-8

Source DB:  PubMed          Journal:  Indian J Hematol Blood Transfus        ISSN: 0971-4502            Impact factor:   0.900


  14 in total

1.  Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias.

Authors:  Sarah L Allford; Beverley J Hunt; Peter Rose; Samuel J Machin
Journal:  Br J Haematol       Date:  2003-02       Impact factor: 6.998

2.  Rituximab for the treatment of thrombotic thrombocytopenic purpura in systemic lupus erythematosus.

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Journal:  Lupus       Date:  2008-01       Impact factor: 2.911

Review 3.  Thrombotic thrombocytopenic purpura: recognition and management.

Authors:  Joseph E Kiss
Journal:  Int J Hematol       Date:  2010-01       Impact factor: 2.490

4.  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

5.  Thrombotic microangiopathic hemolytic anemia with reduction of ADAMTS13 activity: initial manifestation of childhood-onset systemic lupus erythematosus.

Authors:  Eyal Muscal; Rachel M Edwards; Debra L Kearney; John M Hicks; Barry L Myones; Jun Teruya
Journal:  Am J Clin Pathol       Date:  2011-03       Impact factor: 2.493

6.  Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood.

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Journal:  Arthritis Rheum       Date:  1999-11

7.  Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study.

Authors:  S K Kwok; J H Ju; C S Cho; H Y Kim; S H Park
Journal:  Lupus       Date:  2009-01       Impact factor: 2.911

Review 8.  Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus.

Authors:  F Musio; E M Bohen; C M Yuan; P G Welch
Journal:  Semin Arthritis Rheum       Date:  1998-08       Impact factor: 5.532

9.  Thrombotic microangiopathic hemolytic anemia in a patient with SLE: diagnostic difficulties.

Authors:  Ami A Shah; John P Higgins; Eliza F Chakravarty
Journal:  Nat Clin Pract Rheumatol       Date:  2007-06

Review 10.  Interventions for haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura.

Authors:  Mini Michael; Elizabeth J Elliott; Greta F Ridley; Elisabeth M Hodson; Jonathan C Craig
Journal:  Cochrane Database Syst Rev       Date:  2009-01-21
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  1 in total

Review 1.  Pure lupus podocytopathy first presenting as thrombotic thrombocytopenic purpura-like syndrome.

Authors:  Guillermo Delgado-García; Carlos Cámara-Lemarroy; Adrián Infante-Valenzuela; Perla Colunga-Pedraza; Gabriela Alarcón-Galván; Arian Armenta-González; Héctor Jorge Villarreal-Velázquez
Journal:  Clin Rheumatol       Date:  2015-09-18       Impact factor: 2.980

  1 in total

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