Patients aﬀected by bleeding disorders present a
wide spectrum of clinical symptoms that vary from a mild or
moderate bleeding tendency to signiﬁcant episodes. Women
with inherited bleeding disorders are particularly disadvantaged
since, in addition to suﬀering from general bleeding symptoms,
they are also at risk of bleeding complications from regular
haemostatic challenges during menstruation, pregnancy and
Bài giảng Hội chứng xuất huyết (Bleeding disorder) với mục tiêu giúp sinh viên nêu được các nguyên nhân gây xuất huyết; trình bày được triệu chứng lâm sàng của xuất huyết. Hy vọng tài liệu là nguồn thông tin hữu ích cho quá trình học tập và nghiên cứu của các bạn. Mời các bạn cùng tìm hiểu và tham khảo nội dung thông tin tài liệu.
Underlying Systemic Diseases that Cause or Exacerbate a Bleeding Tendency Acquired bleeding disorders are commonly secondary to, or associated with, systemic disease. The clinical evaluation of a patient with a bleeding tendency must therefore include a thorough assessment for evidence of underlying disease. Bruising or mucosal bleeding may be the presenting complaint in liver disease, severe renal impairment, hypothyroidism, paraproteinemias or
amyloidosis, and conditions causing bone marrow failure.
Factor XI Deficiency: Treatment The treatment of FXI deficiency is based on the infusion of FFP at doses of 15–20 mL/kg to maintain trough levels ranging from 10 to 20%. Because FXI has a half-life of 40–70 h, the replacement therapy can be given on alternate days. The use of antifibrinolytic drugs is beneficial to control bleeds, with the exception of hematuria or bleeds in the bladder. The development of a FXI inhibitor was observed in 10% of severely FXI-deficient patients who received replacement therapy.
Epistaxis is a common symptom, particularly in children and in dry climates, and may not reflect an underlying bleeding disorder. However, it is the most common symptom in hereditary hemorrhagic telangiectasia and in boys with vWD. Clues that epistaxis is a symptom of an underlying bleeding disorder include lack of seasonal variation and bleeding that requires medical evaluation or treatment, including cauterization. Bleeding with eruption of primary teeth is seen in children with more severe bleeding disorders, such as moderate and severe hemophilia.
von Willebrand Disease vWD is the most common inherited bleeding disorder. Estimates from laboratory data suggest a prevalence of approximately 1%, but data based on symptomatic individuals suggest that it is closer to 0.1% of the population. vWF serves two roles: (1) as the major adhesion molecule that tethers the platelet to the exposed subendothelium; and (2) as the binding protein for FVIII, resulting in significant prolongation of the FVIII half-life in circulation.
Harrison's Internal Medicine Chapter 110. Coagulation Disorders
Coagulation Disorders: Introduction
Deficiencies of coagulation factors have been recognized for centuries. Patients with genetic deficiencies of plasma coagulation factors exhibit lifelong recurrent bleeding episodes into joints, muscles, and closed spaces, either spontaneously or following an injury. The most common inherited factor deficiencies are the hemophilias, X-linked diseases caused by deficiency of Factor (F) VIII (hemophilia A) or Factor IX (FIX, hemophilia B).
(BQ) Part 2 book "BRS Genetics" presents the following contents: Chromosomal morphology methods, cytogenetic disorders, genetics of metabolism, genetics of hemoglobinopathies, genetics of bleeding disorders, genetics of development, genetics of cancer, genetics of cancer, consanguinity.
Posterior Ischemic Optic Neuropathy This is an infrequent cause of acute visual loss, induced by the combination of severe anemia and hypotension. Cases have been reported after major blood loss during surgery, exsanguinating trauma, gastrointestinal bleeding, and renal dialysis. The fundus usually appears normal, although optic disc swelling develops if the process extends far enough anteriorly. Vision can be salvaged in some patients by prompt blood transfusion and reversal of hypotension.
Optic Neuritis This is a common inflammatory disease of the optic nerve.
Harrison's Internal Medicine Chapter 51. Menstrual Disorders and Pelvic Pain
Menstrual Disorders and Pelvic Pain: Introduction
Menstrual dysfunction can signal an underlying abnormality that may have long-term health consequences. Although frequent or prolonged bleeding usually prompts a woman to seek medical attention, infrequent or absent bleeding may seem less troubling, and the patient may not bring it to the attention of the physician. Thus, a focused menstrual history is a critical part of every female patient encounter.
Polycystic Ovarian Syndrome: Treatment The major abnormality in patients with PCOS is the failure of regular, predictable ovulation. Thus, these patients are at risk for the development of dysfunctional bleeding and endometrial hyperplasia associated with unopposed estrogen exposure. Endometrial protection can be achieved with the use of oral contraceptives or progestins (medroxyprogesterone acetate, 5–10 mg, or prometrium, 200 mg daily for 10–14 days of each month).
Clinically, hemophilia A and hemophilia B are indistinguishable. The disease phenotype correlates with the residual activity of FVIII or FIX and can be classified as severe (25% of normal, the disease is discovered only by bleeding after major trauma or during routine presurgery laboratory tests. Typically, the global tests of coagulation show only an isolated prolongation of the aPTT assay.
The clinical diagnosis of inhibitor is suspected when patients do not respond to factor replacement at therapeutic doses. Inhibitors increase both morbidity and mortality in hemophilia. Because early detection of an inhibitor is critical to a successful correction of the bleeding or to eradication of the antibody, most hemophilia centers perform annual screening for inhibitors. The laboratory test required to confirm the presence of an inhibitor is an aPTT mixed with normal plasma. In most hemophilia patients, a 1:1 mix with normal plasma completely corrects the aPTT.
Approach to the Patient: Bleeding and Thrombosis
Clinical Presentation Disorders of hemostasis may be either inherited or acquired. A detailed personal and family history is key in determining the chronicity of symptoms and the likelihood of the disorder being inherited and it provides clues to underlying conditions that have contributed to the bleeding or thrombotic state.
The central mechanism of DIC is the uncontrolled generation of thrombin by exposure of the blood to pathologic levels of tissue factor (Fig. 110-3). Simultaneous suppression of physiologic anticoagulant mechanisms and abnormal fibrinolysis further accelerate the process. Together these abnormalities contribute to systemic fibrin deposition in small and mid-sized vessels. The duration and intensity of the fibrin deposition can compromise the blood supply of many organs, especially the lung, kidney, liver, and brain, with consequent organ failure.
Differential Diagnosis The differential diagnosis between DIC and severe liver disease is challenging and requires serial measurements of the laboratory parameters of DIC. Patients with severe liver disease are at risk for bleeding and manifest laboratory features including thrombocytopenia (due to platelet sequestration, portal hypertension, or hypersplenism), decreased synthesis of coagulation factors and natural anticoagulants, and elevated levels of FDP due to reduced hepatic clearance. However, in contrast to DIC, these laboratory parameters in liver disease do not change rapidly.
Coagulation Disorders Associated with Liver Failure The liver is central to hemostasis because it is the site of synthesis and clearance of most procoagulant and natural anticoagulant proteins and of essential components of the fibrinolytic system. Liver failure is associated with a high risk of bleeding due to deficient synthesis of procoagulant factors and enhanced fibrinolysis. Thrombocytopenia is common in patients with liver disease and may be due to congestive splenomegaly (hypersplenism), or immune-mediated shortened platelet life span (primary biliary cirrhosis).
At the beginning, specialists tried to diagnose the stage of periodontal disease depending
on the relation between the clinical appearances and the presence of some specific cell
populations or specific matrix components (Havemose-Poulsen & Holmstrup, 1997;
Romanos et al., 1993).
Internal Medicine Essentials for Clerkship Students is a
collaborative project of the American College of
Physicians (ACP) and the Clerkship Directors in
Internal Medicine (CDIM), the organization of individuals
responsible for teaching internal medicine to
medical students. The purpose of IM Essentials is to
provide medical students with an authoritative educational
resource that can be used to augment learning
during the third year internal medicine clerkship.
There are a number of compelling reasons for all physicians to possess knowledge
and skill in the behavioral and social sciences. Perhaps most important is
that roughly half of the causes of mortality in the United States are linked to
social and behavioral factors. In addition, our nation’s population is aging and
becoming more culturally diverse. Both of these trends highlight the need for
enhanced physician capabilities in the behavioral and social sciences.