Absence of Fibroblast-Induced Fibrin Clot Retraction in a Patient with Glanzmann’s Thrombasthenia and Abnormal Wound Healing

Platelets and fibroblasts from normal individuals induce fibrin clot retraction(FCR). Absence of platelet-induced FCR is a characteristic abnormality of Glanzmann’s thrombasthenia (GT) patients. The purpose of this study vas to evaluate whether fibroblasts from a 16-year old girl with severe GT (Thromb. Haemost. 1976, 36, 286) would induce FCR. Fibroblast culture was established from skin biopsy from the inner arm of the patient; fibroblasts were grown and FCR was evaluated as described previously (Europ. J. Cancer 1976, 12, 823). No clot retraction was observed in the samples containing GT fibroblasts during a 24-hour observation period. In control samples FCR started after about 3 hours and appeared to be completed within 12 hours (70–85%).Since fibrin-fibroblast interaction could play a role in normal tissue repair, the process of healing of the wound produced in this patient by the skin biopsy was followed. Neither haemorrhagic complications nor infections occurred during the first 10 days after the biopsy; at that time stiches were removed and the lips of the wound appeared to be well in line. However, abnormal tissue repair (lips swollen, out of the line and interwoven by recent granulation tissue) was evident 20 days later and persisted till the last outpatient control (6 months after biopsy). These results would suggest that in GT the cellular abnormality, as indicated by absent FCR, is not restricted to platelets. Whether the abnormal platelet and fibroblast interaction with fibrin might be somehow related to defective wound healing following skin biopsy is not yet known.