Fibers, crystals, and other forms of HbS polymers in deoxygenated sickle erythrocytes.
We have examined by electron microscopy the formation of fibers and crystals from sickle hemoglobin within sickle erythrocytes following deoxygenation during capillary storage from 1 to 132 days. Intracellular fibers were found on the first day and throughout the period of study. The fibers exhibited a diameter (mean +/- SD) of 17.4 +/- 0.62 nm and were aligned in the cell with a fiber-to-fiber spacing of 18.6 nm (x-axis) by 22.7 nm (y-axis). Between 65 and 132 days, extracellular hemoglobin crystals developed, with a lattice periodicity of 9.63 +/- 0.6 nm. Fibers and crystals coexist as separate structures. These results suggest that crystal formation upon storage of packed deoxygenated sickle erythrocytes may proceed via a phase of fiber dissolution followed by hemoglobin reassembly into extracellular crystals, rather than by a progressive alignment and direct fusion of existing fibers.
D000755:Anemia, Sickle Cell / Q000473:pathology; D003460:Crystallization; D004912:Erythrocytes / Q000648:ultrastructure*; D006451:Hemoglobin, Sickle; D006454:Hemoglobins; D006801:Humans; D008854:Microscopy, Electron; D011108:Polymers; D013997:Time Factors; D014961:X-Ray Diffraction
D006451:Hemoglobin, Sickle; D006454:Hemoglobins; D011108:Polymers; C100003:deoxyhemoglobin