Diagnostic criteria for hematopoietic stem cell transplant-associated microangiopathy: Results of a consensus process by an International Working Group

Tapani Ruutu, Giovanni Barosi, Richard J. Benjamin, Richard E. Clark, James N. George, Alois Gratwohl, Ernst Holler, Massimo Iacobelli, Karim Kentouche, Bernhard Lämmle, Joel L. Moake, Paul Richardson, Gerard Socié, Zella Zeigler, Dietger Niederwieser, Tiziano Barbui

Research output: Contribution to journalArticlepeer-review

Abstract

Background and Objectives: There are no widely accepted criteria for the definition of hematopoietic stem cell transplant -associated microangiopathy (TAM). An International Working Group was formed to develop a consensus formulation of criteria for diagnosing clinically significant TAM. Design and Methods: The participants proposed a list of candidate criteria, selected those considered necessary, and ranked those considered optional to identify a core set of criteria. Three obligatory criteria and four optional criteria that ranked highest formed a core set. In an appropriateness panel process, the participants scored the diagnosis of 16 patient profiles as appropriate or not appropriate for TAM. Using the experts' ratings on the patient profiles as a gold standard, the sensitivity and specificity of 24 candidate definitions of the disorder developed from the core set of criteria were evaluated. A nominal group technique was used to facilitate consensus formation. The definition of TAM with the highest score formed the final proposal. Results: The Working Group proposes that the diagnosis of TAM requires fulfilment of all of the following criteria: (i) >4% schistocytes in blood; (ii) de novo, prolonged or progressive thrombocytopenia (platelet count 9/L or 50% or greater reduction from previous counts); (iii) sudden and persistent increase in lactate dehydrogenase concentration; (iv) decrease in hemoglobin concentration or increased transfusion requirement; and (v) decrease in serum haptoglobin. The sensitivity and specificity of this definition exceed 80%. Interpretation and Conclusions: The Working Group recommends that the presented criteria of TAM be adopted in clinical use, especially in scientific trials.

Original languageEnglish
Pages (from-to)95-100
Number of pages6
JournalHaematologica
Volume92
Issue number1
DOIs
Publication statusPublished - Jan 2007

Keywords

  • Bone marrow transplantation
  • Hematopoietic stem cell transplantation
  • Thrombotic microangiopathy
  • Thrombotic thrombocytopenic purpura
  • Transplant-associated microangiopathy

ASJC Scopus subject areas

  • Hematology

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