title: Relevance of the criteria commonly used to diagnose myeloproliferative disorder in patients with splanchnic vein thrombosis.
abstract: 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 x 10(9)/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 x 10(9)/l constitute a simple index with high specificity but low sensitivity.
authors.affiliation: Service d'Hématologie Clinique, Service d'Anatomie et de Cytologie Pathologiques, Service d'Immunohématologie, Service d'Hépatologie, Hôpital Beaujon (Clichy), 100 Boulevard Général Leclerc, 92218 Clichy, France.
authors.forename: Yasmine
authors.initials: Y
authors.lastname: Chait
authors.forename: Bertrand
authors.initials: B
authors.lastname: Condat
authors.forename: Dominique
authors.initials: D
authors.lastname: Cazals-Hatem
authors.forename: Pierre
authors.initials: P
authors.lastname: Rufat
authors.forename: Sai
authors.initials: S
authors.lastname: Atmani
authors.forename: Driss
authors.initials: D
authors.lastname: Chaoui
authors.forename: Françoise
authors.initials: F
authors.lastname: Guilmin
authors.forename: Jean Jacques
authors.initials: JJ
authors.lastname: Kiladjian
authors.forename: Aurélie
authors.initials: A
authors.lastname: Plessier
authors.forename: Marie Hélène
authors.initials: MH
authors.lastname: Denninger
authors.forename: Nicole
authors.initials: N
authors.lastname: Casadevall
authors.forename: Dominique
authors.initials: D
authors.lastname: Valla
authors.forename: Jean B
authors.initials: JB
authors.lastname: Brière
mesh_terms: D001854:Bone Marrow Cells / Q000473:pathology; D020732:Cytogenetic Analysis; D004911:Erythrocyte Volume; D015672:Erythroid Precursor Cells / Q000473:pathology; D004921:Erythropoietin / Q000032:analysis; D005500:Follow-Up Studies; D006801:Humans; D008533:Megakaryocytes / Q000473:pathology; D009196:Myeloproliferative Disorders / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D010641:Phenotype; D010976:Platelet Count; D012680:Sensitivity and Specificity; D013152:Splanchnic Circulation; D013163:Splenomegaly / Q000209:etiology; D018709:Statistics, Nonparametric; D020246:Venous Thrombosis / Q000150:complications* / Q000473:pathology
chemical_list: D004921:Erythropoietin