Erythroblastic Synartesis in a Patient With Low Grade B Cell Lymphoma

Abstract

Erythroblastic synartesis is a very rare form of acquired dyserythropoiesis. It is characterized by ineffective erythropoiesis due to abnormal aggregates of erythroid precursors in the bone marrow. An autoimmune etiology has been implicated with several cases having an IgG kappa paraprotein. The first case was described in 1973 and a total of 9 cases have been reported to date. There appears to be an association with lymphoproliferative and autoimmune disorders and treatment with steroids, rituximab and other immunosuppressants have been utilized with varying degrees of benefit. We here describe a new case of erythroblastic synartesis in a 59-year African-American man who presented with an IgG kappa paraprotein and an associated low grade B-cell lymphoma. The condition in this patient has proved relatively treatment refractory over the course of almost four years, necessitating multiple sequential lines of therapy with steroids, rituximab, erythropoietin and cyclosporine. A review of the previously published reports will be provided in addition to the current case presentation.




J Hematol. 2014;3(1):13-18
doi: http://dx.doi.org/10.14740/jh113w