Hematological Diseases Flashcards
What is anemia?
A deficiency in the number of erythrocytes (RBCs), quantity or quality of hemoglobin (Hgb), or volume of packed RBCs (hematocrit)
Anemia is not a specific disease but a manifestation of a pathologic process.
What are the general clinical manifestations of anemia?
anemia
Caused by body’s response to tissue hypoxia and vary based on:
* Rate of evolution
* Severity
* Coexisting diseases
Hgb levels are often used to determine severity.
What are the common integumentary manifestations of anemia?
Pallor, jaundice, pruritus
Pallor is due to decreased blood flow and hemoglobin; jaundice results from increased serum bilirubin.
What are the common cardiopulmonary manifestations of anemia?
Increased heart rate and stroke volume to maintain cardiac output
These changes occur as the heart and lungs attempt to provide adequate oxygen to tissues.
What are the common nursing diagnoses for patients with anemia?
Fatigue, impaired nutritional status, ineffective tissue perfusion
Interventions must be tailored to the specific needs and causes of anemia.
What is iron-deficiency anemia?
Most common nutritional disorder in the world, particularly affecting
* Very young
* Women in reproductive years
* Individuals with poor diets or malabsorption
* Those experiencing blood loss
It results from inadequate dietary intake, malabsorption, or blood loss.
What are the clinical manifestations of iron-deficiency anemia?
Pallor, glossitis, cheilitis, abnormal laboratory findings (Hgb, Hct, MCV, MCH, MCHC, reticulocytes, serum iron, TIBC, bilirubin, platelets)
Stool occult blood tests and endoscopy may be used for diagnosis.
What is the goal of interprofessional and nursing management for iron-deficiency anemia?
Treat underlying causes and replace iron through nutritional therapy, oral supplements, or transfusions
Continuous monitoring for liver problems is essential with lifelong therapy.
What is thalassemia?
A group of diseases involving inadequate production of normal hemoglobin, resulting in decreased RBC production due to absent or reduced globulin protein
Thalassemia has an autosomal recessive genetic basis.
What causes megaloblastic anemias?
Impaired DNA synthesis leading to the presence of large RBCs (megaloblasts), primarily due to deficiencies in
* Cobalamin (vitamin B12)
* Folic acid
These deficiencies affect RBC formation and maturation.
What is the treatment for cobalamin deficiency?
Parenteral or intranasal administration of cobalamin (B12) is the treatment of choice
Without treatment, patients may die in 1 to 3 years; long-standing complications may not be reversible.
What is aplastic anemia?
Characterized by pancytopenia (decrease in all blood cell types) and hypocellular bone marrow
It can range from moderate to very severe and is potentially fatal.
What are the common causes of anemia of chronic disease?
Cancer, autoimmune and infectious disorders, chronic inflammation, heart failure, bleeding episodes
It is associated with underproduction of RBCs and can be normocytic, normochromic, and hypoproliferative.
What is hemolytic anemia?
Destruction or hemolysis of RBCs at a rate that exceeds production due to intrinsic or extrinsic problems
Intrinsic forms are usually hereditary, while extrinsic forms are caused by external factors.
What is sickle cell disease?
A group of inherited, autosomal recessive disorders characterized by abnormal hemoglobin in RBCs
It significantly affects quality of life and is usually identified during routine neonatal screening.
What triggers sickling episodes in sickle cell disease?
Low oxygen tension in blood, with infection being the most common precipitating factor
Sickle cell crisis can lead to severe capillary hypoxia and tissue necrosis.
What are the common nursing considerations for patients with megaloblastic anemia?
Early detection and treatment, ensuring safety, and possibly physical therapy
Diminished sensations to heat and pain may necessitate protective measures.
What is the management for acute blood loss anemia?
Identify the source and stop bleeding, provide supplemental iron as needed
Hypovolemic shock is a major complication of acute blood loss.
What is a major cause of morbidity and mortality in sickle cell disease?
Infection
Infection is particularly severe due to compromised spleen function from sickled RBCs.
What is autosplenectomy?
A result of scarring from sickled RBCs leading to compromised spleen function
What is the most common type of pneumonia associated with sickle cell disease?
Pneumococcal pneumonia
What are the lung complications associated with sickle cell disease?
- Pneumonia
- Tissue infarction
- Fat embolism
What are the key symptoms of acute chest syndrome?
- Fever
- Chest pain
- Cough
- Lung infiltrates
- Dyspnea
What diagnostic studies are used for sickle cell disease?
- Peripheral blood smear
- Hemoglobin electrophoresis
- Skeletal x-rays
- MRI
- Doppler studies
- Chest x-ray
What is the management focus for sickle cell disease?
- Preventing sequelae from disease
- Alleviating manifestations from complications
- Minimizing end-organ damage
- Continuously assessing and treating serious sequelae
What are common clinical manifestations of thrombocytopenia?
- Mucosal bleeding
- Cutaneous bleeding
- Petechiae
- Purpura
- Ecchymoses
What are the causes of thrombocytopenia?
- Inherited
- Acquired
- Immune thrombocytopenia purpura (ITP)
- Thrombotic thrombocytopenia purpura (TTP)
- Heparin-induced thrombocytopenia (HIT)
What is the primary nursing diagnosis for a patient with thrombocytopenia?
Risk for bleeding
What is the typical platelet count that indicates thrombocytopenia?
Less than 150,000/µL
What can be a serious complication of untreated thrombotic thrombocytopenia purpura (TTP)?
Irreversible renal failure and death
What is the first step in the management of heparin-induced thrombocytopenia (HIT)?
Stop all heparin including flushes
What is the genetic cause of hemophilia?
X-linked recessive genetic disorder
What are the two major types of hemophilia?
- Hemophilia A
- Hemophilia B
What is the deficiency associated with von Willebrand disease?
Deficiency of von Willebrand coagulation factor (Factor VIII)
What is a serious bleeding and thrombotic disorder characterized by abnormal clotting?
Disseminated Intravascular Coagulation (DIC)
What is a specific marker for the degree of fibrinolysis in DIC?
D-dimer
What are common clinical manifestations of hemophilia?
- Slow, persistent bleeding
- Delayed bleeding after minor injuries
- Uncontrollable hemorrhage after dental procedures
- Epistaxis
- GI bleeding
- Hematuria
What is blood component therapy primarily used for?
Managing hematologic diseases
What is the most common cause of hemolytic transfusion reactions?
Transfusion of ABO-incompatible blood
True or False: Blood component therapy permanently resolves the underlying problem.
False
What should be done immediately after obtaining blood from the blood bank?
Positive identification of the donor blood and recipient must be made
What is autotransfusion?
Removing whole blood from a person and transfusing it back into the same person
What are the three main deficiencies in anemia?
Number of erythrocytes (RBCs), quantity or quality of hemoglobin (Hgb), and volume of packed RBCs (hematocrit).
How is anemia diagnosed?
Through complete blood count (CBC), reticulocyte count, and peripheral blood smear.
What are the integumentary manifestations of anemia?
Pallor (decreased Hgb and blood flow), jaundice (increased bilirubin), and pruritus (increased bile salts).
What are common cardiopulmonary responses to anemia?
Increased heart rate and stroke volume to compensate for reduced oxygen transport.
What is the most common nutritional disorder worldwide?
Iron-deficiency anemia.
What are major causes of iron-deficiency anemia?
Inadequate dietary intake, malabsorption, and blood loss.
What are key clinical manifestations of iron-deficiency anemia?
Pallor (most common), glossitis (inflamed tongue), and cheilitis (inflamed lips).
How is iron-deficiency anemia managed?
Treating underlying cause, nutritional therapy, oral iron supplements, and blood transfusions if severe.
What is the primary cause of thalassemia?
Absent or reduced globulin protein leading to decreased RBC production and hemolysis.
What are the two major types of thalassemia?
Thalassemia minor (one gene affected) and thalassemia major (two genes affected).
What deficiencies cause megaloblastic anemia?
Cobalamin (vitamin B12) and folic acid deficiency.
How is cobalamin deficiency managed?
Parenteral or intranasal administration of cobalamin, as oral absorption is impaired.
What are causes of anemia of chronic disease?
Cancer, autoimmune disorders, chronic inflammation, and infections like HIV and hepatitis.
What is aplastic anemia?
Pancytopenia (decreased RBCs, WBCs, and platelets) due to hypocellular bone marrow.
What is the major complication of acute blood loss anemia?
Hypovolemic shock due to rapid blood loss.
What triggers sickling episodes in sickle cell disease?
Low oxygen tension, often caused by infection, stress, dehydration, or altitude changes.
What are complications of sickle cell disease?
Pain crisis, stroke, organ damage, acute chest syndrome, and infection.
What is the most common cause of thrombocytopenia?
Immune thrombocytopenia purpura (ITP), where the immune system destroys platelets.
What are key symptoms of thrombocytopenia?
Petechiae (small red dots), purpura (bruising), and prolonged bleeding.
What is Disseminated Intravascular Coagulation (DIC)?
A serious disorder causing excessive clotting followed by bleeding due to depleted clotting factors.
What are the primary treatments for DIC?
Treating the underlying cause, blood transfusions, and anticoagulants if thrombosis is present.
What are signs of a hemolytic transfusion reaction?
Fever, chills, back pain, dark urine, and hypotension due to ABO-incompatible blood.
What is the purpose of autotransfusion?
To collect and reinfuse a patient’s own blood to avoid reactions and disease transmission.
