PubMed1975
Ultrastructural studies of the bone marrow of a patient with refractory anemia revealed aberrant erythroblasts with unique cell junctions. Periodic structures linked adjacent processes on the same cell as well as surfaces of neighboring erythroblasts. Inclusions circumscribed by similar complexes were also present in the cytoplasm. The junction appeared in cross sections as two rigidly parallel unit membranes separated by 250-300 A interspace with two regular arrays of facing 70 A particles at intervals of 160-200 A. These intracellular specializations were present between erythroblasts at various stages of maturation and between, mono, bi or multinucleated erythroblasts. Junctions were permeable to lanthanum and many that appeared to be intracellular were demonstrated to be continuous with the extracellular space. The fact that others were not penetrated by lanthanum indicates that detachment of the interdigitating processes from cells of origin could have occurred. In freeze-etched replicas, distribution of membrane particles was random in areas of septate-like junctions, although parallel rows were sometimes observed both on A and B inner hydrophobic faces of the membrane leaflets. Junctional complexes in tissue culture appeared to have been disrupted and were not reestablished; however, inclusions resembling internalized junctions were observed associated with multivesicular bodies. Ineffective erythropoiesis and the resulting refractory anemia appear to be associated with the presence of the described anomalous junctional complexes.