Synovial tumefactive extramedullary hematopoiesis associated to polycythemia vera.
The case of a 66-year-old male patient with a chronic myeloproliferative type polycythemia vera disorder, who after 2 years of evolution is developing a tumefactive extramedullary hematopoiesis (TEH) located in the synovial of the articulation in the right knee, is described. The tumor histologically consists of a relatively lax and edematous synovial structure diffusely infiltrated by mature and semimature hematopoietic cellular population. The simultaneous study of the bone marrow reveals medullar spaces full of hematopoietic cellularity, with a predominance of megakaryocytic and red series, and with the addition of severe reticulin fibrosis, facts that suggest a progression toward myelofibrosis. The TEH developed in tissues without a reticulum endothelial system is very uncommon. We provide data about the first case located in the synovial membrane and we review the literature regarding this pathologic entity.
Alvarez-Argüelles Cabrera
Hugo
H
Department of Pathology, Canary University Hospital, Tenerife, Canary Islands, Spain.
Carrasco Juan
José Luis
JL
García Castro
María Candelaria
MC
González Gaitano
Manuel
M
Bonilla Arjona
Alfonso
A
Díaz-Flores
Lucio
L
D000368:Aged; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D008297:Male; D009372:Neoplasms, Connective Tissue / Q000473:pathology*; D011087:Polycythemia Vera / Q000150:complications*; D055728:Primary Myelofibrosis / Q000209:etiology / Q000473:pathology; D013583:Synovial Membrane / Q000473:pathology*