mod 4

Question 1

Anemia

Answer 1

hemoglobin is less than 10 or 11 g/dl

Question 2

Iron-Deficiency Anemia

Answer 2

Predictable at developmental periods

In premature infants, due to low fetal supply

At 12 to 36 months, due to ingestion of large amounts of cow’s milk and diet

In adolescents, due to rapid growth and poor eating habits

Question 3

Sickle Cell Crisis

Answer 3

Precipitating factors

Anything that increases the body’s need for oxygen or alters the transport of oxygen
Trauma
Fever, infection
Physical and emotional stress
Increased blood viscosity due to dehydration
Hypoxia

Types
Vasoocclusive (VOC) thrombotic
Splenic sequestration
Aplastic crises

Question 4

Sickle Cell Vasoocclusive (VOC) thrombotic

Answer 4

Vasoocclusive (VOC) thrombotic
Most common type of crisis and is very painful
Stasis of blood with clumping of cells in the microcirculation leads to ischemia and then infarction
Signs are fever, pain, and tissue engorgement

Question 5

Sickle Cell Splenic sequestration

Answer 5

Life-threatening type; death can occur within hours
Blood pools in the spleen
Signs are profound anemia, hypovolemia, and shock

Question 6

Sickle Cell Aplastic crises

Answer 6

Diminished production and increased destruction of RBCs
Triggered by viral infection or depletion of folic acid
Signs include profound anemia and pallor

Question 7

Diagnosis of Sickle Cell Anemia

Answer 7

Cord blood in newborns
Newborn screening
Genetic testing to identify carriers and children who have the disease
Sickle turbidity test
Quick screening in children over 6 months of age
Hemoglobin electrophoresis

Question 8

Thalassemia (Cooley Anemia)

Answer 8

Anemia results from defective synthesis of hemoglobin, structurally impaired RBCs, and a shortened life of RBCs

Inherited blood disorders of hemoglobin synthesis
Classified by the hemoglobin chain affected and by the amount of effect
Autosomal recessive disorder with varying expressivity
Both parents must be carriers to have offspring with the disease

RBC changes are often seen by 6 weeks of age

Question 9

beta-Thalassemia

Answer 9

Four types
Thalassemia minor: Asymptomatic silent carrier
Thalassemia trait: Mild microcytic anemia
Thalassemia intermediate: Moderate to severe anemia plus splenomegaly
Thalassemia major (Cooley anemia): Severe anemia requiring transfusions for survival

Greeks, Italians, and Syrians

Question 10

alpha-Thalassemia

Answer 10

Occurs in Chinese, Thai, African, and Mediterranean people

Question 11

Medical Management of Thalassemia

Answer 11

Blood transfusion to maintain normal hemoglobin levels
Side effect is hemosiderosis
Treat with iron-chelating drugs such as deferoxamine (Desferal)
Binds excess iron for excretion by the kidney

Question 12

Aplastic Anemia

Answer 12

Aplastic Anemia

In hypoplastic anemia, there is profound depression of RBCs but normal levels of white blood cells (WBCs) and platelets

idiopathic

Question 13

Hemophilia A

Answer 13

Classic hemophilia (deficiency of factor VIII)
Accounts for 80% of cases of hemophilia

Question 14

Hemophilia B

Answer 14

Christmas disease (deficiency of factor IX)

Question 15

von Willebrand disease (vWD)

Answer 15

Deficiency, abnormality, or absence of vWF and factor VIII

Affects both males and females

Question 16

Etiology of Hemophilia A

Answer 16

Males are affected

Females may be carriers

Question 17

Manifestations of Hemophilia

Answer 17

Bleeding tendencies range from mild to severe
Symptoms may not occur until 6 months of age

Hemarthrosis
Bleeding into joint spaces of the knee, ankle, or elbow leads to impaired mobility and, eventually, bony changes and disability
Symptoms include warmth, pain, bruising, and decreased movement

Epistasis

Bleeding in the gastrointestinal tract

Question 18

Hemophilia Diagnostic

Answer 18

diagnosed through amniocentesis

Genetic testing of family members

low levels of factor VIII or IX and a prolonged partial thromboplastin time (PTT)

Platelet count, prothrombin time (PT), and fibrinogen levels are normal

Question 19

Management of Hemophilia

Answer 19

Desmopressin (DDAVP)
IV
Increases factor VIII activity by two to four times
Used for mild hemophilia

Question 20

Interventions for Hemophilia

Answer 20

Shave only with an electric razor

For superficial bleeding, apply pressure for at least 15 minutes and ice to promote vasoconstriction

If significant bleeding occurs, transfusion for factor replacement

Question 21

Managing Hemarthrosis

Answer 21

During bleeding episodes, elevate and immobilize the joint

Range-of-motion exercises after the bleeding stops will help to prevent contractures

Avoid obesity to minimize joint stress

Question 22

Idiopathic Thrombocytopenic Purpura

Answer 22

An acquired hemorrhagic disorder characterized by

Thrombocytopenia (excessive destruction of platelets)

Purpura (discoloration caused by petechiae beneath the skin, with no other signs of bleeding)
NORMAL bone marrow

Occurs in two forms
Acute, self-limiting (usually following a viral illness)
Chronic (lasting longer than 12 months)

Diagnostic evaluation
Platelet count is less than 20,200/mm
Rule out other diseases and conditions

Question 23

Wiskott-Aldrich Syndrome

Answer 23

X-linked recessive disorder with triad of
Thrombocytopenia (usually at birth)
Eczema
Immunodeficiency of select B and T lymphocytes

Question 24

Blood transfusions

Answer 24

Monitor vital signs
Baseline, initially; 15 minutes after initiation and completion
Use an appropriate filter and saline flush
Use blood within 30 minutes of arrival
Infuse a full unit over 4 hours maximum