Literature DB >> 30042915

A Case Report on Refractory Moschcowitz Syndrome.

Beenish Fayyaz1, Hafiz Jawad Rehman1.   

Abstract

Although completely identified later on, Thrombotic thrombocytopenic purpura was first described by Dr. Moschcowitz in 1924, therefore the name 'Moschcowitz syndrome'. It is a microangiopathy associated with thrombocytopenia and hemolysis which causes organ dysfunction secondary to widespread microvascular thrombosis. The spectrum of the organ dysfunction in thrombotic thrombocytopenic purpura is quite diverse and not limited to nervous system and kidneys as classically described thus making the diagnosis more difficult. Use of plasmapheresis has led to improvement in mortality rates associated with this disease. However, multiple organ failure and presence of shock indicates likelihood of severe disease activity or refractoriness which can be avoided with earlier initiation of immunosuppressive therapy such as Rituximab.

Entities:  

Keywords:  moschcowitz syndrome; multiorgan failure; plasmapheresis; rituximab; thrombotic microangiopathy; thrombotic thrombocytopenic purpura

Year:  2018        PMID: 30042915      PMCID: PMC6054365          DOI: 10.7759/cureus.2664

Source DB:  PubMed          Journal:  Cureus        ISSN: 2168-8184


  7 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

Review 2.  Clinical practice. Thrombotic thrombocytopenic purpura.

Authors:  James N George
Journal:  N Engl J Med       Date:  2006-05-04       Impact factor: 91.245

3.  Various clinical manifestations in patients with thrombotic microangiopathy.

Authors:  Jae C Chang; Satheesh K Kathula
Journal:  J Investig Med       Date:  2002-05       Impact factor: 2.895

4.  von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome.

Authors:  M Furlan; R Robles; M Galbusera; G Remuzzi; P A Kyrle; B Brenner; M Krause; I Scharrer; V Aumann; U Mittler; M Solenthaler; B Lämmle
Journal:  N Engl J Med       Date:  1998-11-26       Impact factor: 91.245

Review 5.  Thrombotic microangiopathies.

Authors:  Michael H Rosove
Journal:  Semin Arthritis Rheum       Date:  2013-11-16       Impact factor: 5.532

Review 6.  Thrombotic thrombocytopenic purpura: from diagnosis to therapy.

Authors:  Eric Mariotte; Agnès Veyradier
Journal:  Curr Opin Crit Care       Date:  2015-12       Impact factor: 3.687

7.  Thrombotic thrombocytopenic purpura with concomitant small- and large-vessel thrombosis, atypical posterior reversible encephalopathy syndrome and cerebral microbleeds.

Authors:  Wong Li Yu; Thomas Leung; Yannie Soo; Jessamyn Lee; Ka Sing Wong
Journal:  Oxf Med Case Reports       Date:  2015-02-03
  7 in total

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