Inherited platelet function disorders are associated with a significant bleeding risk during surgery, a retrospective, multicenter study shows.

“The frequency of excessive bleeding assessed by one of three criteria at surgery is pretty remarkable because we had rates ranging from 13% to about 19% depending on the assessment criteria,” Dr. Paolo Gresele said at the annual congress of the European Hematology Association.

Any excessive bleeding by any criteria took place in about 23% of patients.

Although conventionally considered rare, inherited platelet function disorders (IPFD) are more frequent than previously thought, and recent advances in their diagnosis have expanded the forms and number of reported patients.

Only a handful of case reports or small case series have evaluated the bleeding risk associated with surgery in patients with IPFD. As a result, the exact bleeding risk and most appropriate management options in IPFD are not known, Dr. Gresele of University of Perugia (Italy) said.

He reported on a retrospective study involving patients with IPFD enrolled at 42 centers from 16 countries. The median age of the 205 patients was 35 years, and 56% were women; 89 had Glanzmann thrombasthenia, 46 had primary secretion defect, 14 had combined alpha-delta granule deficiency, 11 had Hermansky-Pudlak syndrome, 9 had gray platelet syndrome, and 36 had other forms of IPFD.

Data were collected from 389 procedures, of which 54.5% were surgeries, 30% were dental procedures, and 15.5% were invasive procedures. The most frequent surgeries were otolaryngologic, abdominal, orthopedic, gynecologic, urologic, and dermatologic.

The frequency of excessive bleeding at surgery was about 19.7% by the Bleeding Academic Research Consortium (BARC) bleeding classification, 17% by subjective evaluation by the surgeon or patient, and 13% based on duration of bleeding at surgery of at least 6 or more hours, Dr. Gresele said.

The World Health Organization (WHO) bleeding scale before surgery was quite predictive of excessive bleeding at surgery, with patients at grade 3 and 4 having an odds ratio for excessive bleeding of 9.22 and 28, respectively, compared with patients at grade 0, he said.

Somewhat unexpectedly, the most serious bleeders based on a BARC of at least 2 or more at surgery were patients with Hermansky-Pudlak syndrome, Glanzmann thrombasthenia, and gray platelet syndrome. Those with primary secretion defect and combined alpha-delta granule deficiency had a relatively low risk of bleeding at surgery, he said.

The procedures associated with more excessive bleeding were urologic surgery, gynecologic surgery, and abdominal surgery.

Preparation for surgery was made in 82% of procedures and included prophylactic platelet transfusions in 38.5%, desmopressin (DDAVP) in about 19%, antifibrinolytic agents in about 12%, factor VIIa in less than 10%, and other interventions such as cryoprecipitate, fibrinogen, fresh frozen plasma, intravenous immunoglobulin, suture, and local hemostatic agent in less than 5%.

Generally all measures had a good ability to prevent excessive bleeding, bringing rates down to about 20% or less, Dr. Gresele said. The two exceptions were no prophylaxis and “other” interventions, where the bleeding rate reached a high of about 45% for the latter. In contrast, bleeding risk was significantly reduced when any prophylaxis (odds ratio, 0.38) or DDAVP (OR, 0.24) were used.

In 87 cases, however, emergency treatment for excessive bleeding was required, he said. Platelet transfusions were the most common treatment, given in roughly half of patients, followed by antifibrinolytic agents. The success rate was about 80% for all measures, except for “other” interventions, which had a success rate of about 65%.

“Treatment of excessive bleeding is made mostly with platelet transfusions or antifibrinolytic agents and is able to stop bleeding in many, but not all cases,” Dr. Gresele concluded.