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

From the Department of Medicine, Helsinki University Central Hospital, Helsinki, Finland (TR); Laboratory of Clinical Epidemiology, IRCCS Policlinico S. Matteo, Pavia, Italy (GB); American Red Cross-New England Region, Dedham, MA, USA (RJB); Department of Haematology, Royal Liverpool University Hospital, Liverpool, UK (REC); Hematology-Oncology Section, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA (JNG); Division of Hematology, University Hospital Basel, Basel, Switzerland (AG); Abteilung Hämatologie/Internal Onkologie, Klinikum der Universität Regensburg, Regensburg, Germany (EH); Gentium, Villa Guardia, Como, Italy (MI); Klinik für Kinder-und Jugendmedizin, Friedrich-Schiller-Universität, Jena, Germany (KK); Department of Hematology and Central Hematology Laboratory, University Hospital Bern, Bern, Switzerland (BL); Baylor College of Medicine, Houston, TX, USA (JLM); Harvard Medical School and Dana-Farber Cancer Institute, Boston, MA, USA (PR); Department of Hematology, Hôpital Saint-Louis, Paris, France (GS); Institute for Transfusion Medicine, Pittsburgh, PA, USA (ZZ); Division of Hematology and Oncology, University of Leipzig, Leipzig, Germany (DN); Division of Hematology, Ospedali Riuniti di Bergamo, Bergamo, Italy (TB)

Correspondence: Tapani Ruutu, M.D., Helsinki University Central Hospital, Department of Medicine, Division of Hematology POB 340, FIN-00029 HUS, Helsinki. Finland. E-mail: tapani.ruutu{at}hus.fi

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 <50x109/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.

Key words: bone marrow transplantation, hematopoietic stem cell transplantation, thrombotic microangiopathy, thrombotic thrombocytopenic purpura, transplant-associated microangiopathy.

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