Erythroblastic synartesis: an auto-immune dyserythropoiesis.
Erythroblastic synartesis is a rare form of acquired dyserythropoiesis, first described by Breton-Gorius et al in 1973. This syndrome is characterized by the presence of septate-like membrane junctions and "glove finger" invaginations between erythroblasts, which are very tightly linked together. This phenomenon, responsible for ineffective erythropoiesis, leads to an isolated severe anemia with reticulocytopenia. In the following report, we describe 3 new cases of erythroblastic synartesis associated with dysimmunity and monoclonal gammapathy. In all cases, the diagnosis was suggested by characteristic morphological appearance of bone marrow smears, and further confirmed by electron microscopy. Ultrastructural examination of abnormal erythroblast clusters showed that these cells were closely approximated with characteristic intercellular membrane junctions. The pathogenesis of the dyserythropoiesis was modeled in vitro using crossed erythroblast cultures and immunoelectron microscopy: when cultured in the presence of autologous serum, the erythroblasts from the patients displayed synartesis, whereas these disappeared when cultured in normal serum. Moreover, synartesis of normal erythroblasts were induced by the patient IgG fraction. Immunogold labeling showed that the monoclonal IgG were detected in, and restricted to, the synartesis. A discrete monoclonal plasmacytosis was also found in the patient bone marrow. The adhesion receptor CD36 appeared to be concentrated in the junctions, suggesting that it might be involved in the synartesis. These experiments indicated that a monoclonal serum immunoglobulin (IgG in the present cases) directed at erythroblast membrane antigen was responsible for the erythroblast abnormalities. Specific therapy of the underlying lymphoproliferation was followed by complete remission of the anemia in these cases.
Cramer
E M
EM
Services d'Hématologie Biologique et Clinique, Hôpital Lariboisière, Paris, France. emcramer@im3.inserm.fr
Garcia
I
I
Massé
J M
JM
Zini
J M
JM
Lambin
P
P
Oksenhendler
E
E
Souni
F
F
Smith
M
M
Flandrin
G
G
Breton-Gorius
J
J
Tobelem
G
G
Casadevall
N
N
D000328:Adult; D000368:Aged; D000742:Anemia, Dyserythropoietic, Congenital / Q000276:immunology* / Q000473:pathology; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases; D004900:Erythroblasts / Q000276:immunology* / Q000473:pathology / Q000648:ultrastructure; D004920:Erythropoiesis / Q000276:immunology*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008854:Microscopy, Electron; D008875:Middle Aged
D001323:Autoantibodies; D007074:Immunoglobulin G