Clinical pattern of hemophilia and causes of variability

Maria Eva Mingot-Castellano1

  • 1Department of Hematology, Málaga Regional University Hospital, Malaga, Spain.

: In persons with hemophilia, the severity and spontaneity of bleeding episodes depends on the degree of factor deficiency present in the patients. Severe hemophilia is classically characterized by a factor VIII (FVIII) or factor IX (FIX) coagulant activity below 1% of normal (spontaneous and severe bleeds). Moderate hemophilia is associated with factor activity between 1 and 5% of normal (milder spontaneous and traumatic bleeds). Finally, mild hemophilia has factor activity in excess of 5% of normal (traumatic bleeds in general). Nonetheless, this classification is rather simplistic. It is a known fact that there are subjects with identical clotting factor levels who have different bleeding phenotypes. We will analyze different factors to justify this.

Related Concept Videos

Tail Vein Transection Bleeding Model in Fully Anesthetized Hemophilia A Mice08:13

Tail Vein Transection Bleeding Model in Fully Anesthetized Hemophilia A Mice

The refined tail vein transection (TVT) bleeding model in anesthetized mice is a sensitive in vivo method for the assessment of hemophilic bleeding. This optimized TVT bleeding model uses blood loss and bleeding time as endpoints, refining other models and avoiding death as an...
7.4K
Variability: Analysis01:11

Variability: Analysis

Measures of variability are statistical metrics that reveal the dispersion pattern within a dataset. They are pivotal in biostatistics, providing insights into the heterogeneity within health and biological data. Variability signifies the degree to which data points diverge from one another, helping researchers understand the potential range of values and associated uncertainty within the data.
The range is a simple measure of variability, indicating the difference between the highest and...
448
Random Variables01:09

Random Variables

A random variable is a single numerical value that indicates the outcome of a procedure. The concept of random variables is fundamental to the probability theory and was introduced by a Russian mathematician, Pafnuty Chebyshev, in the mid-nineteenth century.
Uppercase letters such as X or Y denote a random variable. Lowercase letters like x or y denote the value of a random variable. If X is a random variable, then X is written in words, and x is given as a number.
For example, let X = the...
17.4K
Variables Affecting Phosphorescence and Fluorescence01:26

Variables Affecting Phosphorescence and Fluorescence

Fluorescence and phosphorescence are essential phenomena in fields like analytical chemistry, biological imaging, and materials science, where they detect molecular properties and visualize cellular structures. Understanding the variables that influence these luminescent behaviors is crucial for maximizing accuracy and efficiency in their applications. These variables can broadly be grouped into chemical structure, solvent properties, and external conditions, each playing a distinct role in...
1.3K
Work and Energy for Variable Forces01:10

Work and Energy for Variable Forces

When an object is acted upon by a variable force, the amount of work done and the change in energy of the object can be more complex to calculate compared to when a constant force is applied. Work is the product of force and displacement, while energy is the capacity of a system to do work. When a constant force is applied to an object, the work done can be calculated as the product of the force and the distance moved in the direction of the force. However, when a variable force is applied, the...
5.6K
Fixed Action Patterns01:06

Fixed Action Patterns

A fixed action pattern (FAP) is a specific, hard-wired sequence of behaviors that occurs in response to an external stimulus, called a sign stimulus. The behavior is “fixed” because it is essentially unchangeable—proceeding similarly across individuals of a species every time it occurs.
17.5K