Level of Awareness of Acquired Hemophilia A Among Physicians in Türkiye: A Survey Study

Abstract

This survey study aimed to evaluate the level of awareness and knowledge of acquired hemophilia A (AHA) among physicians from various specialties. Data were collected by administering a questionnaire containing questions about two patient profiles to evaluate the approaches of physicians from different specialties. The study included a total of 945 physicians. The most common suspected diagnosis for the clinical patient profile was immune thrombocytopenia, followed by hemophilia. For the laboratory patient profile, the most common suspected diagnosis was hemophilia. While only 9.4% of the physicians stated that they had good knowledge of this disease, only 21.6% wanted to learn more about AHA. Most of the participating physicians do not rank AHA first in the differential diagnosis of a patient with clinical and laboratory findings of AHA. Appropriate educational activities to raise awareness of AHA will help reduce disease-related morbidity and mortality.

Introduction

Acquired hemophilia A (AHA) is a rare, potentially fatal coagulation disorder, which is usually seen in elderly individuals without a history of bleeding disorders and is associated with autoantibodies against factor VIII (FVIII) [1,2]. In half of these patients, autoimmune disorders, hematologic malignancies, solid tumors, respiratory system disorders, skin conditions, pregnancy, drug reactions, infections, and vaccinations have been reported to coincide with AHA [1,2,3,4,5,6].

AHA should be included in the differential diagnosis of any patient with a history of unexpected, new-onset bleeding and consistent physical findings. In cases where the platelet count, prothrombin time (PT), and thrombin time (TT) are normal but the activated partial thromboplastin time (aPTT) alone is prolonged, a mixing test (inhibitory screening test) is required to confirm the diagnosis.

Morbidity and mortality rates are high among symptomatic, untreated patients. The rareness of the disease, low levels of awareness among physicians, and the complexity of the laboratory assessments usually lead to delayed diagnosis [7,8]. Thus, raising awareness of AHA among physicians of patients with a high potential of having this disease is of utmost importance to ensure their immediate referral to an expert for appropriate management [7,9].

This survey study evaluated the levels of awareness and knowledge of AHA among physicians from various specialties.

Materials and Methods

Physicians from different specialties with a high potential of encountering patients with AHA were invited to take part in this cross-sectional survey study. The study was approved by the local ethics committee. All of the participating physicians provided their written informed consent. A sample size with a 95% confidence interval and 3% margin of error was estimated to include at least 940 participants. The study group consisted of specialists recruited from 6 different fields of medicine (obstetrics and gynecology, rheumatology, emergency medicine, internal medicine, medical oncology, and adult hematology) according to the distribution rate of specialties in Türkiye.

The questionnaire used in this survey study consisted of the 13 questions provided in Table 1. Physicians were given two patient profiles, one with clinical findings (Table 2) and the other with laboratory findings (Table 3) of AHA, and were asked to comment on the probable diagnoses of the patients and how they would approach and manage each case. Statistical analyses were performed using IBM SPSS Statistics 21.0 with a statistical significance level of 0.05. Numerical variables were expressed as means and standard deviations, while categorical variables were expressed as frequencies and percentages. Comparisons among subgroups were performed using the chi-square test.

Table 1. Questionnaire.

Table 2. Profile 1.

Table 3. Profile 2.

Results

A total of 945 physicians (mean age: 36.7±9.6 years; range: 24-70 years) working in different departments of hospitals were included. Over 66% of the physicians reported having seen cases with clinical profiles indicating AHA among a total of 7819±5497 (median: 7000) patients within the past 12 months (Table 4).

Table 4. Number of physicians who encountered patients matching the provided profiles in the last 12 months.

When physicians were asked about the diagnosis of the patient given in Table 2 using an open-ended question, the most common response was immune thrombocytopenia (ITP) (23.4%), followed by hemophilia (18.3%). The percentages of physicians suspecting hemophilia were significantly higher among hematologists (27.5%) and rheumatologists (29.5%) (Table 5). The rate of physicians who included AHA in the differential diagnosis was 31.1%. Almost all hematologists (93.8%) indicated that they would treat the patient with the given profile, while the majority of non-hematologists (81.2%) stated that they would rather refer the patient to a different specialty, mainly to hematology (87.0%).

Table 5. Number of physicians stating or choosing “Hemophilia” as the most likely diagnosis for the patients described in the profiles.

When the same question was asked in a multiple-choice format (Table 1, Q6), ITP (39.6%) and hemophilia (32.0%) were the most frequently selected choices. Nearly 50% of the hematologists associated the given patient profile with hemophilia. Hemophilia was significantly less considered among physicians aged ≥41 years compared to younger age groups (65.7% vs. 83.5% and 84.4%, p=0.001). Only 40.2% of the physicians and 59.7% of the hematologists who suspected hemophilia further associated the clinical profile with AHA (Table 1, Q6a).

More than one-third of the physicians reported having seen cases consistent with the second profile (cases with laboratory findings indicative of AHA) within the past 12 months (Table 3). Hemophilia ranked first among the probable diagnoses for this profile, being suspected by 27.8% of the physicians (Table 5). AHA was considered (Table 1, Q6a) by 21.3% of hematologists and only 3.5% of all physicians. Hemophilia and AHA were indicated by 53.5% and 67.7% of the physicians, respectively, when given as multiple-choice options.

Only 9.4% of the physicians stated that they had a high level of knowledge regarding AHA and an additional 21.6% expressed their interest in learning more about AHA (Table 6). This interest was significantly higher in the age group of ≤30 years (p=0.003).

Table 6. Physicians’ self-evaluation of their level of knowledge of acquired hemophilia.

Discussion

Increased awareness of AHA is essential to prevent delays in the diagnosis and treatment of this highly fatal disease [10]. In our study, 18.3% of the physicians listed AHA among the probable diagnoses for the given clinical patient profile. In a recent study with similar results, Taher et al. [5] suggested that the awareness of healthcare professionals should be raised and their level of knowledge regarding AHA should be improved. One-third of the physicians in our study indicated ITP as the most probable diagnosis even when hemophilia was also given as an option. Misdiagnosis is a global issue that is primarily associated with the rareness of the disease and probably also related to the underrepresentation of AHA in the medical education curricula, both of which contribute to low levels of awareness regarding the disease. A survey study conducted among non-hematologists working at tertiary medical centers in the Arabian Gulf showed that 42% of the responders were not aware of AHA [11].

Our study showed that physicians in the higher age groups had significantly lower levels of knowledge of AHA. This might be explained by the recent activities aiming to raise awareness of acquired hemophilia, which mainly target younger physicians in the field.

A great majority of the participants (90.6%) reported having limited or low levels of knowledge regarding the disease. Despite this, overall interest in learning more about AHA remained low with the exception of physicians aged ≤30 years, which might suggest that younger physicians have a higher motivation for learning.

Studies indicate that hematologists should play a major role in creating policies to increase physicians’ awareness of AHA and provide guidance on its appropriate diagnosis and treatment [5]. In an expert opinion paper recently published by Dolan et al. [12], 10 key principles are listed for the management of AHA. Considering the importance of the issue, it seems to be crucial that these principles be globally implemented by integrating AHA into the training programs of medical schools and the curricula of both specialty training and continuing medical education activities to ensure an accurate and timely diagnosis and appropriate management.

Conclusion

The results of our study demonstrate that the level of awareness of AHA among physicians from various specialties in Türkiye is not adequate for the appropriate and timely diagnosis and treatment of the disease. Raising awareness of AHA, particularly among non-hematologists, would contribute significantly to early diagnosis and help reduce the morbidity and mortality rates associated with AHA. We hope that our study will inspire the development of strategies and national education projects to improve countrywide awareness of AHA.