Relevance of the criteria commonly used to diagnose myeloproliferative disorder in patients with splanchnic vein thrombosis

Authors

  • Yasmine Chait,

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    • *

      These authors contributed equally to this study.

    • Present addresses: Yasmine Chait, Unité d'Hématologie clinique, département d'Oncologie clinique, Centre Hospitalier Intercommunal Le Raincy-Montfermeil, Montfermeil, France.

  • Bertrand Condat,

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    • *

      These authors contributed equally to this study.

  • Dominique Cazals-Hatem,

    1. Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, and INSERM U 481, Hôpital Beaujon (Clichy); and Laboratoire d'Hématologie, Hôtel Dieu (Paris), Assistance Publique-Hôpitaux de Paris, France
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  • Pierre Rufat,

    1. Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, and INSERM U 481, Hôpital Beaujon (Clichy); and Laboratoire d'Hématologie, Hôtel Dieu (Paris), Assistance Publique-Hôpitaux de Paris, France
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  • Sai Atmani,

    1. Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, and INSERM U 481, Hôpital Beaujon (Clichy); and Laboratoire d'Hématologie, Hôtel Dieu (Paris), Assistance Publique-Hôpitaux de Paris, France
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  • Driss Chaoui,

    1. Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, and INSERM U 481, Hôpital Beaujon (Clichy); and Laboratoire d'Hématologie, Hôtel Dieu (Paris), Assistance Publique-Hôpitaux de Paris, France
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  • Françoise Guilmin,

    1. Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, and INSERM U 481, Hôpital Beaujon (Clichy); and Laboratoire d'Hématologie, Hôtel Dieu (Paris), Assistance Publique-Hôpitaux de Paris, France
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  • Jean Jacques Kiladjian,

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    • ‡Jean Jacques Kiladjian, Département d'Hématologie, Hôpital Avicenne, Bobigny, France.

  • Aurélie Plessier,

    1. Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, and INSERM U 481, Hôpital Beaujon (Clichy); and Laboratoire d'Hématologie, Hôtel Dieu (Paris), Assistance Publique-Hôpitaux de Paris, France
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  • Marie Hélène Denninger,

    1. Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, and INSERM U 481, Hôpital Beaujon (Clichy); and Laboratoire d'Hématologie, Hôtel Dieu (Paris), Assistance Publique-Hôpitaux de Paris, France
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  • Nicole Casadevall,

    1. Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, and INSERM U 481, Hôpital Beaujon (Clichy); and Laboratoire d'Hématologie, Hôtel Dieu (Paris), Assistance Publique-Hôpitaux de Paris, France
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  • Dominique Valla,

    1. Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, and INSERM U 481, Hôpital Beaujon (Clichy); and Laboratoire d'Hématologie, Hôtel Dieu (Paris), Assistance Publique-Hôpitaux de Paris, France
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  • Jean B. Brière

    1. Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, and INSERM U 481, Hôpital Beaujon (Clichy); and Laboratoire d'Hématologie, Hôtel Dieu (Paris), Assistance Publique-Hôpitaux de Paris, France
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Jean B. Brière, Hematology Unit Beaujon Hospital, 100 Bd Général Leclerc 92118, Clichy, France.
E-mail: jean.briere@bjn.ap-hop-paris.fr

Summary

Myeloproliferative disorders (MPD) are reported in 25–65% of patients with splanchnic vein thrombosis (SVT). Diagnostic criteria for MPD have not been fully established in this context. Using clusters of abnormal megakaryocytes in bone marrow (BM) biopsy as a reference standard for Philadelphia negative MPD, we assessed the relevance of other criteria currently recommended for the diagnosis of MPD in SVT (128 consecutive SVT patients). First, usual criteria were compared with BM results: endogenous erythroid colony formation (EEC) was strongly correlated with BM results; splenomegaly, blood cell count, total red cell volume, erythropoietin level and cytogenetic were much less accurate. Then, patients were assigned to three groups according to the combination of BM and EEC findings (group I: both present; group II: both absent; group III: other patients); clinical presentation and outcome were compared in each group. At a mean follow-up of 6·09 ± 6·6 years, progression to a severe form of MPD occurred in 7 of 31 group I patients (23%), in 1 of 34 group III patients (3%) and 0 of 63 group II patients. The combination of marked splenomegaly and platelet count >200 × 109/l was restricted to groups I and III. In conclusion, in patients with SVT, BM findings and EEC allowed the diagnosis of MPD at risk of aggravation. Marked splenomegaly in association with platelet counts >200 × 109/l constitute a simple index with high specificity but low sensitivity.

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