[Acquired bleeding disorders - diagnostic approach]. / Abklärung einer erworbenen Blutungsneigung.

BACKGROUND: Patients with acquired bleeding disorders are often a diagnostic challenge in internal medicine. OBJECTIVES: Which diagnostic work up is necessary in these patients and which coagulation tests are useful? MATERIAL AND METHODS: Analysis and diagnostic workup in the daily practice are discussed according to review publications in this field and existing clinical evidence. RESULTS: First important step is the bleeding history of the patient. The different signs of bleeding are important hints for the cause. The global assays Quick (PT) and PTT in combination with platelet count and function reveal a primary knowledge of the kind of the acquired bleeding disorder. In addition special coagulation tests normally lead to the exact diagnosis of the cause of bleeding. CONCLUSIONS: Patients with newly developed bleeding symptoms should be examined for acquired coagulation disorders. The clinical sings in combination with the special coagulation tests allow the correct diagnosis.

Bleeding disorders seen in the dental practice.

Dentists may encounter patients with various types of bleeding disorders in their daily practice. Initial recognition of such bleeding disorders and their possible systemic causes, as well as knowing when to refer those cases to secondary care, plays a crucial and important role in reducing potential complications and negative side-effects. This article will give an account of the most common bleeding disorders that dentists might find in their daily dental practice. This will be followed by another article that will cover the management of congenital and acquired disorders found in the dental practice.

The accuracy of medical history information in referral letters.

BACKGROUND: Accurate medical history information is essential for good patient care and should be notified in the letter of referral. The aim of this study was to investigate the subjective opinion that the medical information in a large number of referrals is either inaccurate or non-existent. METHODS: Medical histories from 54 patients with positive medical history findings upon taking the medical history at the initial consultation appointment were compared to the medical information supplied in the referral letter. RESULTS: Overall, medical information was only 58.8% complete with dental referrals being 55.2% complete and medical referrals 62.4%. The majority of referral letters (70.4%) missed at least one relevant finding and only 29.6% of referrals were 100% complete. CONCLUSIONS: The results of this study suggest that the standard of referral letters needs to be improved as the received referrals were generally incomplete and contained inaccurate information. This highlights the need for each and every practitioner to take their own detailed medical history and not rely on the information supplied in the referral.

Spectrum of inherited bleeding disorders in southern Iran, before and after the establishment of comprehensive coagulation laboratory.

The objective of the present study was to determine the pattern of inherited bleeding disorders in southern Iran and evaluate the effect of a comprehensive coagulation laboratory and related efforts. A total of 545 patients with inherited bleeding disorders were evaluated during 1992-2007 by a cross-sectional study. Data were collected by a data-gathering form. Statistical analysis was done using Statistical Package for the Social Sciences version 15. A P value less than 0.05 was considered statistically significant. Overall 411 patients had common bleeding disorders including 326 hemophilia A, 46 hemophilia B, and 39 von Willebrand disease. Seventy-nine patients had rare coagulation disorders including deficiency of factor VII (n = 26), factor X (n = 18), factor XIII (n = 9), factor I (n = 9), factor XI (n = 7), factor V (n = 4), combined factor VIII and factor V (n = 4), and combined factor X and factor VII (n = 2). Fifty-five patients had platelet disorders including 23 with Glanzmann's thrombasthenia, 15 with Bernard-Soulier syndrome, and 17 with other platelet disorders, most of which (45) were diagnosed after the establishment of the comprehensive coagulation laboratory. Annual mean number of new diagnosed patients with common and rare bleeding disorders increased from 29 +/- 4 to 38 +/- 17. The ratio of the patients diagnosed with rare bleeding disorders to common bleeding disorders significantly increased after the establishment of the comprehensive diagnosis laboratory (P < 0.001).It seems that implementation of collaborative projects by the Shiraz Hemophilia Society and the establishment of the comprehensive coagulation laboratory and treatment centers have been successful in increasing diagnosis of the inherited bleeding disorders and consequently better management of the patients.

Familial multiple coagulation factor deficiencies - chance associations and distinct clinical disorders.

The familial multiple coagulation factor deficiencies (FMCFDs) are a group of rare haemostatic disorders of genetic origin in which there is reduced plasma activity of more than one coagulation factor. FMCFDs may arise from co-incidental inheritance of separate coagulation factor deficiencies or from a single genetic or cytogenetic defect. All the FMCFDs present significant challenges in diagnosis and management yet there is little systematic evidence with which to guide clinical practice. This review summarizes the historical literature that describes the FMCFDs and introduces a refined classification of these disorders. The clinical and laboratory characteristics of the most common FMCFDs are considered in detail.

Laboratory issues in bleeding disorders.

Selected laboratory issues critical for the appropriate diagnosis of haemophilia A and B, von Willebrand's disease (VWD) and more rare bleeding disorders (RBD) are discussed from a worldwide perspective. The overall picture that emerges is on the whole reassuring. Even in non-Western countries like Latin America, most cases of haemophilia are appropriately diagnosed. Moreover, national and international laboratory training workshops are further improving the diagnostic capabilities also in less severe disorders. Most of the RBD can be appropriately diagnosed with relatively simple tests wherever a high clinical suspicion is present. Moreover, minimal requirements for a useful clinical diagnosis are not too far from the capabilities of majority of non-Western countries. The most needed areas concern VWD and platelet function disorders, which suffer from inadequate diagnostic standardization, hampering widespread diagnostic capability in both Western and non-Western countries.

[Quantitative bleeding time].

Bleeding time, which reflects the interaction of the platelets with the damaged vessel wall and the subsequent formation of the primary hemostatic plug, has been widely used in the diagnosis of bleeding disorders, especially platelet abnormalities. We have developed a computerized method to measure the bleeding pattern and the amount of blood loss from the bleeding time incision (quantitative bleeding time). In 87 normal subjects (51 males and 36 females), the bleeding time was 389 +/- 137 sec and the amount of blood loss was 15.7 +/- 7.2 microliters (mean +/- S.D.). The bleeding pattern was classified into four types (I-IV). Type II showed the prolongation of the bleeding time, continuous constant bleeding, and considerably large amount of blood loss from the incision. This type which includes severe von Willebrand disease and serious thrombocytopenia is related to the severe bleeding tendency. Type III exhibited the prolongation of the bleeding time keeping a trace of blood loss from the incision. In type III patients, the bleeding tendency was generally mild despite the prolonged bleeding time as in patients with moderate thrombocytopenia or aspirin ingestion. Measurement of bleeding pattern and blood loss will provide a useful information to evaluate the defect of primary hemostasis.