Relevance of the criteria commonly used to diagnose myeloproliferative disorder in patients with splanchnic vein thrombosis.
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.
Chait
Yasmine
Y
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.
Condat
Bertrand
B
Cazals-Hatem
Dominique
D
Rufat
Pierre
P
Atmani
Sai
S
Chaoui
Driss
D
Guilmin
Françoise
F
Kiladjian
Jean Jacques
JJ
Plessier
Aurélie
A
Denninger
Marie Hélène
MH
Casadevall
Nicole
N
Valla
Dominique
D
Brière
Jean B
JB
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
D004921:Erythropoietin