Lecture 10: HEMOPHILIA AND VON WILLEBRAND DISEASE
Hemophilia is a sex-linked recessive genetic disorder caused by defective or deficient
coagulation factor. The two major forms of hemophilia, which can occur in mild to
severe forms, are hemophilia A and hemophilia B.
Von Willebrand disease is a related disorder involving a deficiency of the von Willebrand
Replacement of deficient clotting factors is the primary means of supporting a patient
with hemophilia. In addition to treating acute crises, replacement therapy may be given
before surgery and before dental care as a prophylactic measure.
Home management is a primary consideration for the patient with hemophilia because the
disease follows a progressive, chronic course.
The patient with hemophilia must be taught to recognize disease-related problems and to
learn which problems can be resolved at home and which require hospitalization.
DISSEMINATED INTRAVASCULAR COAGULATION
Disseminated intravascular coagulation (DIC) is a serious bleeding and thrombotic
It results from abnormally initiated and accelerated clotting. Subsequent decreases in
clotting factors and platelets ensue, which may lead to uncontrollable hemorrhage.
DIC is always caused by an underlying disease or condition. The underlying problem
must be treated for the DIC to resolve.
It is important to diagnose DIC quickly, stabilize the patient if needed (e.g., oxygenation,
volume replacement), institute therapy that will resolve the underlying causative disease
or problem, and provide supportive care for the manifestations resulting from the
pathology of DIC itself.
Neutropenia is a reduction in neutrophils, a type of granulocyte, and therefore is
sometime referred to as granulocytopenia. The neutrophilic granulocytes are closely
monitored in clinical practice as an indicator of a patient’s risk for infection.
Neutropenia is a clinical consequence that occurs with a variety of conditions or diseases.
It can also be an expected effect, a side effect, or an unintentional effect of taking certain
drugs. Occasionally the cause of the neutropenia can be easily treated (e.g., nutritional
deficiencies). However, neutropenia can also be a side effect that must be tolerated as a
necessary step in therapy (e.g., chemotherapy, radiation therapy). In some situations the
neutropenia resolves when the primary disease is treated (e.g., tuberculosis).
The nurse needs to monitor the neutropenic patient for signs and symptoms of infection
and early septic shock.
Myelodysplastic syndrome (MDS) is a group of related hematologic disorders
characterized by a change in the quantity and quality of bone marrow elements. Although
it can occur in all age groups, the highest prevalence is in people over 60 years of age.
Supportive treatment consists of hematologic monitoring, antibiotic therapy, or
transfusions with blood products. The overall goal is to improve hematopoiesis and
ensure age-related quality of life.
Leukemia is the general term used to describe a group of malignant disorders affecting
the blood and blood-forming tissues of the bone marrow, lymph system, and spleen.
Classification of leukemia can be done based on acute versus chronic and on the type of
WBC involved, whether it is of myelogenous origin or of lymphocytic origin.
o The onset of acute myelogenous leukemia (AML) is often abrupt and dramatic.
AML is characterized by uncontrolled proliferation of myeloblasts, the