Figure 7.

Images of pathologic patterns of five forms of inherited platelet disorder (IPD) obtained by confocal microscopy.Immunofluorescence microscopy. In Glanzmann’s thrombasthenia (GT), platelets show almost no GpIIb/IIIa surface receptor (green). In BSS, the signal corresponding to GpIb/IX (green) on the surface is lacking, and only some spots inside the platelets are recognizable, showing the altered protein which cannot be transferred to the membrane. In GFI1B-RT, the stem cell antigen CD34 (green) persists on the platelet surface. Instead of a typical nice ring (controls), the cytoskeleton marker β1-tubulin (green) appears twisted in Wiskott-Aldrich syndrome (WAS), and resembles a ball of wool in TUBB1-RT. The healthy controls are shown in the upper pictures.