Diagnosis and management of acquired thrombotic thrombocytopenic purpura in southeast China: a single center experience of 60 cases

Front Med. 2016 Dec;10(4):430-436. doi: 10.1007/s11684-016-0492-5. Epub 2016 Dec 23.

Abstract

Acquired thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening thrombotic microangiopathy. This study aimed to provide a profile of the diagnosis and management of patients with acquired TTP collected in 10 years in a single center in southeast China. A total of 60 patients diagnosed with acute acquired TTP from March 2005 to August 2015 were enrolled. Among the 60 patients, 52 patients presented with their first episodes, and eight patients had two or more episodes. The median age at presentation was 49 (range, 17 to 78) years with a female predominance (male:female ratio, 1:1.60). ADAMTS 13 activity were analyzed in 43 patients, among whom 33 (76.7%) patients had a baseline level of < 5%. Mortality was 30%. Plasma exchange (PEX) was performed in 62 of 69 (89.9%) episodes. Corticosteroids were administered in 54 of 69 (78.3%) episodes. Other immunosuppressants (e.g., vincristine, cyclosporine, and cyclosporin) were used in 7 of 69 (10.1%) episodes. Rituximab was documented in 4 patients with refractory/relapsed TTP for 5 episodes, showing encouraging results. In conclusion, the diagnosis of TTP depended on a comprehensive analysis of clinical data. Plasma ADAMTS13 activity assay helped confirm a diagnosis. PEX was the mainstay of the therapy, and rituximab can be used in relapsed/refractory disease.

Keywords: ADAMTS 13; plasma exchange; thrombotic thrombocytopenic purpura.

MeSH terms

  • ADAMTS13 Protein / immunology
  • Adolescent
  • Adult
  • Aged
  • Antibodies, Monoclonal / blood
  • China
  • Cyclosporine / therapeutic use
  • Disease Management
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Male
  • Middle Aged
  • Plasma Exchange
  • Purpura, Thrombotic Thrombocytopenic / epidemiology*
  • Purpura, Thrombotic Thrombocytopenic / mortality
  • Purpura, Thrombotic Thrombocytopenic / therapy*
  • Recurrence
  • Rituximab / therapeutic use
  • Vincristine / therapeutic use
  • Young Adult

Substances

  • Antibodies, Monoclonal
  • Immunosuppressive Agents
  • Rituximab
  • Vincristine
  • Cyclosporine
  • ADAMTS13 Protein
  • ADAMTS13 protein, human

Supplementary concepts

  • Thrombotic thrombocytopenic purpura, acquired