Patients with a bleeding diathesis remain a diagnostic challenge in medicine. Many healthy individuals consider their bleeding and bruising excessive, whereas patients with mild to moderate abnormalities may not recognize subtle symptoms as abnormal. Distinguishing between these two groups of patients requires skill and experience and often cannot be done with certainty. On the other hand, patients with profound coagulation disorders and obviously abnormal bleeding symptoms may not volunteer information unless specifically questioned. A few standardized bleeding assessment instruments and disease-specific scales have been developed, but few attempts have been made to assess their clinical usefulness and hence are not widely used. In addition, laboratory tests for screening for hemostatic abnormalities lack sensitivity and specificity. The purpose of this study is to describe the initial approach, consultation behavior and outcome in patients with a suspected bleeding diathesis in a tertiary care center.
A 5-year retrospective analysis of adult patients with new and/or unexplained bleeding history referred to the hematology service of the Henry Ford Hospital. Patients were excluded it they were younger than 18 years, had a previous diagnosis of a bleeding disorder, or had been referred because of an abnormal hemostatic screening test without a history of bleeding. Data were collected for demographics, presenting symptoms, initial work up prior to consultation, hematologist’s work up and final diagnosis.
A total of 103 patients were included in the study. 75.7% (78) were female, and the median age of presentation was 41 years (range 18 to 85). The most common bleeding symptom was easy bruising (62.7%), followed by menorrhagia (37.3%), bleeding after tooth extraction or a surgical procedure (15.6%), and epistaxis (13.6%). The initial work up was mostly done by the primary care physician and in a few cases by the gynecologist. In 58% of cases the initial work up consisted of a complete blood count and a PT/PTT; in 32.3% no work up was done; in 5.7% the work up included a PFA-100; and in 4% a von Willebrand screening test was carried out. Work up by the consulting hematologist consisted of PFA-100, von Willebrand screen (and associated specialized tests), platelet aggregation studies, and electron microscopy when appropriate. Von Willebrand disease was diagnosed in 36.4% of patients, a platelet function disorder was found in 8.4%, a coagulation factor deficiency was found in 3.9% (2 patients with factor iX, 1 patient with factor VIII and 1 patient with factor VII). After complete work up, a bleeding diathesis could not be confirmed in 36.4% of patients, while in 14.9% of patients the etiology was secondary to a systemic illness or medication related. Among patients diagnosed with von Willebrand disease, 76.9% were female, with menorrhagia being reported in 70% of them, followed by easy bruising in 44.4%. In cases where a bleeding diathesis could not be confirmed, 79.5% were female. Of those, menorrhagia was reported in 31.25% of cases.
Easy bruising was the most common presenting symptom for which patients were referred to hematology. Overall, a third of patients complained of menorrhagia, and we found that it was the most common complaint among those with von Willebrand disease. Our study describes the difficulty in attaining a definite diagnosis in patients with a suspected bleeding diathesis since a diagnosis could not be confirmed in about one third of the patients. This addresses the limitations of current diagnostic assays. Additionally, should these patients truly not have a bleeding diathesis, it stresses the need for standardized bleeding scores and instruments to discriminate between healthy individuals and patients with a true bleeding diathesis.
No relevant conflicts of interest to declare.
Asterisk with author names denotes non-ASH members.