140 Questions
What is the primary function of hemoglobin in red blood cells?
To carry oxygen from lungs to tissues and organs
Which of the following is NOT a general cause of anemia?
Overproduction of bone marrow
What is the estimated number of people suffering from anemia worldwide?
2.5 billion
Which of the following is a common risk factor for anemia?
Having a poor diet
What is the name of the protein that carries oxygen from the lungs to tissues and organs?
Hemoglobin
In which part of the body are red blood cells, white blood cells, and platelets produced?
Bone marrow
What is the most common type of white blood cell?
Neutrophil
Which of the following is a possible consequence of anemia?
Less ability to remove CO2 from tissues
What is the first step in the lab evaluation of anemia?
Start with a complete blood count (CBC)
Which of the following is a type of anemia caused by a lack of iron?
Iron-deficiency anemia
What is Hematocrit (Hct) in the context of blood?
The ratio of the volume of red blood cells to the total volume of blood
What is the primary cause of a decreased Red Cell Distribution Width (RDW)?
Very little variation in size of RBCs
What is the primary characteristic of Microcytic anemia?
Small RBCs
Which of the following is a cause of Megaloblastic anemia?
All of the above
What is the primary function of Vitamin B12 in the body?
Maintenance of neurological health
What is the primary difference between B12 deficiency and folate deficiency?
Homocysteine levels are high in folate deficiency
What is the primary purpose of iron studies in anemia?
To diagnose iron deficiency anemia
What is the primary function of transferrin in the body?
To bind with iron in the blood
What is the primary meaning of a low ferritin level?
Iron deficiency
What is the primary purpose of checking methylmalonic acid (MMA) levels in anemia?
To diagnose Vitamin B12 deficiency
What is the primary function of a reticulocyte count?
To check if the bone marrow is producing the right amount of red blood cells
What is the primary cause of megaloblastic anemia?
Folic acid deficiency
What is the significance of a low haptoglobin level?
It indicates hemolytic anemia
What is the primary function of the Coombs test?
To diagnose autoimmune hemolytic anemia
What is the significance of a high LDH level in the blood?
All of the above
What is the primary function of serum ferritin?
To determine if a person has an iron deficiency or not
What is the primary cause of anemia in elderly people with iron deficiency?
Colon cancer
What is the primary treatment for iron deficiency anemia?
Oral iron supplements
What is the primary purpose of checking for signs of anemia in a patient?
To determine if the anemia is severe or not
What is the primary approach to diagnosing anemia?
Check for obvious signs of anemia
What is the primary function of hemoglobin inside red blood cells?
To carry oxygen from the lungs to tissues and organs in the body and carry carbon dioxide back to the lungs
What is the primary site of blood production in adults?
Bone marrow of bones
Which of the following is a common risk factor for anemia?
Having a poor diet
What is the effect of anemia on the body's ability to deliver oxygen to tissues?
Less ability to deliver oxygen
What is the primary cause of anemia in people with iron deficiency?
Lack of iron
Which of the following is a type of white blood cell?
Lymphocyte
What is the effect of anemia on the body's ability to remove CO2 from tissues?
Less ability to remove CO2
Which of the following is a general cause of anemia?
Bleeding
What is the prevalence of anemia in the world's population?
30%
Which of the following is a type of anemia caused by a lack of nutrients or vitamins?
All of the above
What is the primary reason for a decrease in Red Cell Distribution Width (RDW) in anemia?
Very little variation in size of RBCs
What is the primary difference between Microcytic and Megaloblastic anemia?
Size of RBCs
What is the primary cause of Spurious Megaloblastic anemia?
Cells sticking together
What is the primary treatment for Vitamin B12 deficiency in patients with intrinsic factor deficiency?
Intramuscular Vitamin B12 injections
What is the primary purpose of checking Homocysteine levels in anemia?
To differentiate between Vitamin B12 and folate deficiency
What is the primary meaning of a high Transferrin saturation level?
Iron overload
What is the primary function of Ferritin in the body?
To store iron
What is the primary reason for checking Total Iron Binding Capacity (TIBC) in anemia?
To diagnose iron deficiency
What is the primary difference in the treatment of Megaloblastic anemia caused by Vitamin B12 deficiency versus folate deficiency?
Type of medication
What is the primary reason for checking Methylmalonic acid (MMA) levels in anemia?
To diagnose Vitamin B12 deficiency
What is the primary reason for conducting iron studies in anemia?
To determine the body's iron stores and saturation
What is the implication of a high serum ferritin level in a patient with anemia?
The patient is not iron deficient
What is the primary difference between iron deficiency anemia and B12 deficiency anemia?
B12 deficiency anemia is characterized by larger red blood cells
What is the significance of a low haptoglobin level in a patient with anemia?
The patient has hemolytic anemia
What is the primary purpose of the Coombs test in anemia?
To determine the presence of autoimmune hemolytic anemia
What is the implication of a high LDH level in a patient with anemia?
The patient has hemolytic anemia or tissue damage
What is the primary cause of anemia in elderly people with iron deficiency?
Colon cancer
What is the primary approach to diagnosing anemia in a patient?
Check for signs of anemia and then investigate the cause
What is the significance of a reticulocyte count in anemia?
It determines the body's production of red blood cells
What is the primary treatment for iron deficiency anemia?
Oral iron supplementation
What is the primary component of healthy clotting that is responsible for adhering to the site of vascular injury?
Von Willebrand Factor (vWF)
What is the term for the formation of a clot that persists in an unbroken vessel?
Thrombus
What is the primary cause of hypercoagulability in patients with cancer?
Reactive disorder
What is the term for the movement of a clot that is freely moving in the bloodstream and can get stuck in a vessel over time?
Embolus
What is the primary cause of stasis in patients with polycythemia or leukostasis?
High hematocrit or elevated WBCs
What is the term for the combination of stasis, hypercoagulability, and vascular injury that contributes to clot formation?
Virchow's triad
What is the primary sign of bleeding too much?
Increased/prolonged menstrual bleeding
What is the primary cause of vascular injury in patients with atherosclerosis?
Vascular disease
What is the primary purpose of the Mixing Study in coagulation disorders?
To determine the presence of a clotting factor deficiency or inhibitor
What is the implication of a high D-Dimer level in a patient?
The patient has a clot somewhere in the body
What is the primary characteristic of Polycythemia Vera?
An increase in red blood cell production
What is the significance of a low ATIII level in a patient?
The patient is experiencing clot formation
What is the primary treatment for Polycythemia Vera?
Phlebotomy and aspirin
What is the implication of a prolonged aPTT in a patient?
The patient has a clotting factor deficiency
What is the primary function of Factor V in the coagulation cascade?
To act as a cofactor for factor X
What is the primary characteristic of Thrombocytosis?
An increase in platelet production
What is the primary effect of the mutation in Factor V Leiden?
Removal of the cleavage site used by activated Protein C
What is the risk of a first VTE in compound heterozygotes with G20210A and FVL?
20
What is the primary function of Protein C?
To inactivate Factor V and Factor VIII
What is the classification of a DVT that is below the knee and past the popliteal vein?
Distal DVT
What is the next step in the evaluation of a patient with a low probability of DVT and a positive d-dimer?
Obtain an ultrasound
What is the primary difference in the treatment of proximal and distal DVTs?
Proximal DVTs require long-term anticoagulation, while distal DVTs do not
What is the primary purpose of the PERC rule?
To rule out pulmonary embolism in patients with low probability
What is the next step in the evaluation of a patient with low or intermediate risk of pulmonary embolism and a positive PERC rule?
Send a d-dimer
What is the primary indication for considering systemic lytic therapy or catheter-directed thrombolysis in patients with pulmonary embolism?
Severe presentation of PE
What is the recommended duration of anticoagulation therapy for patients with unprovoked PE?
Long-term anticoagulation
What is the primary purpose of ordering troponin values in patients with pulmonary embolism?
To evaluate right heart strain
What is the primary indication for using IVC filters in patients with pulmonary embolism?
Patients who cannot tolerate anticoagulation
What is the primary risk factor for VTE in hospitalized patients?
All of the above
What is the primary mechanism of Heparin-Induced Thrombocytopenia (HIT)?
Antibodies that form to heparin-platelet complex and cause platelet aggregation
What is the primary criterion for diagnosing Vaccine-Induced Thrombotic Thrombocytopenia (VITT)?
All of the above
What is the primary recommendation for patients with suspected VITT?
Perform immediate CBC with platelet count and imaging for thrombosis
What is the primary approach to diagnosing VITT?
Draw blood prior to any therapies for PF4-ELISA
What is a common finding in patients with HHC?
Hyperpigmentation and liver cirrhosis
What is the estimated percentage of affected males who develop cirrhosis due to iron overload?
10%
What is a characteristic radiologic finding in HHC arthropathy?
Squared-off bone ends in the MCP joints
What is the primary treatment for hemochromatosis?
Phlebotomy
What is the amount of iron contained in 500mL of whole blood?
200-250mg
What is the primary endocrine abnormality in hemochromatosis?
Hypogonadism
What is the goal of treatment in hemochromatosis?
Achieve ferritin levels
What is the primary function of the quaternary structure of hemoglobin in red blood cells?
To carry oxygen for transport within red blood cells
What is the characteristic of hemoglobin A2?
It has two alpha and two delta chains
What is the primary cause of thalassemia?
Mutations that alter beta globin chains
What is the result of a production problem in anemia?
Marrow not producing red blood cells
What is the significance of elevated LDH, AST, and bilirubin levels in anemia?
Indicates hemolysis
What is the significance of a low haptoglobin level in anemia?
Indicates hemolysis
What is the primary difference between alpha and beta thalassemia?
Alpha thalassemia is caused by deficient alpha chains
What is the primary function of the tetramer structure of hemoglobin?
To carry four oxygen molecules
What is the result of hemolysis in anemia?
Red blood cells breaking down
What is the primary characteristic of hemoglobin A?
It has two alpha and two beta chains
What is the recommended dose of morphine for an opioid-experienced patient weighing over 50kg?
5-10mg IV
What is the purpose of transfusing leukoreduced RBCs in a patient with hemoglobin S disease?
To increase oxygenation
How much will 10ml/kg of leukoreduced RBCs typically raise the hemoglobin level?
2gm/dl
What is the goal of partial exchange transfusion in a patient with hemoglobin S disease?
To decrease hyperviscosity
How much will one unit of PRBCs decrease the hemoglobin S level?
30%
What is the recommended rate of transfusion in a patient with severe anemia to avoid volume overload?
1 unit over 3-4 hours
What is the benefit of phlebotomizing 500ml of blood before transfusing 2 units of PRBCs?
Decreases iron burden
What is the primary mechanism by which sickle cells adhere to the endothelium?
Interaction between VLA-4 and VCAM-1
What is the effect of hypoxia on nitric oxide in patients with sickle cell disease?
Decreases nitric oxide availability
What is the goal of transfusion in a patient with hemoglobin S disease?
To increase oxygenation
What is the recommended target hematocrit level in a patient with hemoglobin S disease on chronic transfusion?
35%
What is the primary reason for the formation of a gel-like substance containing Hb crystals in deoxygenated conditions?
Marked decrease in solubility and increased viscosity of Hb
What is the primary function of the spleen in patients with sickle cell disease?
To create lymphocytes for the destruction and recycling of old RBCs
What is the effect of increasing hematocrit level above 30% in a patient with hemoglobin S disease?
Creates hyperviscosity
What is the primary consequence of recurrent sickling in patients with sickle cell disease?
Irreversibly sickled RBCs
What is the primary mechanism by which free Hb contributes to vasoconstriction in sickle cell disease?
By decreasing nitric oxide availability
What is the primary characteristic of sickle cells that makes them sticky?
Expression of VLA-4 on the surface
What is the primary cause of avascular necrosis in patients with sickle cell disease?
Vaso-occlusive crisis
What is the primary effect of hydroxyurea on the spleen in patients with sickle cell disease?
Preserves the function of the spleen
What is the primary consequence of hemolysis in patients with sickle cell disease?
Increased levels of LDH
What is the primary reason for the development of hepatosplenomegaly in patients with Beta Thalassemia Major?
Hemolysis and anemia driving increased EPO and bone marrow hyperplasia
What is the characteristic of Hemoglobin H disease?
Presence of beta 4 tetramers
Why do patients with Sickle Cell Disease have a protective effect against malaria?
Sickled cells halt the infestation of malaria
What is the primary reason for the requirement of transfusion support in patients with Beta Thalassemia Major?
Hemolysis and anemia due to incorrect synthesis of HbA
What is the characteristic of Alpha Thalassemia Trait?
Mild anemia with microcytosis
What is the primary cause of anemia in patients with Beta Thalassemia?
Excess production of other chains hindering normal RBC production
What is the term for the process that leads to many of the complications of Sickle Cell Disease?
Vaso-occlusion
What is the primary difference between HbSS and HbSb0 thalassemia?
HbSS has a more severe anemia than HbSb0
Why do patients with Beta Thalassemia require annual MRI to check liver iron?
To monitor for liver iron overload
What is the primary goal of treatment in Beta Thalassemia?
To manage symptoms and improve quality of life
Study Notes
Anemia
- Definition: Decreased red blood cells, leading to decreased oxygen delivery and increased CO2 levels
- Causes: Bleeding, underproduction, bone marrow issues, lack of nutrients/vitamins, immune destruction, hemolysis, increased needs (pregnancy, medications)
Epidemiology of Anemia
- 30% of the world's population (approx. 2.5 billion people) has anemia
- Iron deficiency makes up about 1 billion people, especially prevalent in lower-income regions (sub-Saharan Africa, South Asia, Caribbean)
- In the USA, approximately 3 million people suffer from anemia
Blood Production
- In adults, blood is made in the bone marrow of bones
- Bone marrow also makes White Blood cells and Platelets
- White blood cells, in order from most common to least common: Neutrophil, Lymphocytes, Monocytes, Eosinophil, Basophil
Risk Factors for Anemia
- Being a menstruating woman
- Having a poor diet
- Having an autoimmune or inflammatory disease
- Having an infection
- Having cancer
- Having liver or endocrine disease
- Having kidney disease
- Having a chronic disease of any type
Lab Evaluation for Anemia
- Start with a Complete Blood Count (CBC)
- Hemoglobin (Hb): protein inside red blood cells that carries oxygen
- Hematocrit (Hct): ratio of the volume of red blood cells to the total volume of blood
- MCV (Mean Corpuscular Volume): measurement of Red Blood Cell Volume (size of RBC)
- RDW: red cell distribution width
- Increase in RDW = variation in size of RBC, which is a normal finding
- Decrease in RDW = very little variation in size, which could be due to anemia (macrocytic anemia)
Classification of Anemias
- Classified based on MCV (size of the cell)
- Microcytic (small) = MCV < 80
- Normocytic (normal) = MCV 80-100
- Macrocytic (large) = MCV > 100
- Megaloblastic (extra large) = MCV > 110
Causes of Microcytic Anemia (MCV < 80)
- Iron deficiency
- Thalassemia
- Sideroblastic anemia
- Anemia of chronic disease
Causes of Megaloblastic Anemia (MCV > 110)
- Folic acid deficiency
- B12 deficiency
- Spurious Megaloblastic anemia (cells sticking together and machine reading them as one)
Treatment of B12 Deficiency
- B12 comes from animal products (meats…)
- Needed for neurological health
- Lack of B12 over long periods can lead to vibratory sensation loss, psychosis, and is not usually reversible once it happens
- If vegetarian, can try B12 supplements by mouth
- If unable to absorb B12 (Intrinsic factor deficiency), must receive medication IM FOR LIFE!!!### Lab Tests for Bleeding Disorders
- CBC – Platelet count and mean platelet volume (MPV)
- PFA (Platelet Function Analysis)
- Partial thromboplastin time (aPTT) – if prolonged, indicates issue with clotting factors or vWF
- Prothrombin time (PT) – if prolonged, indicates issue with clotting factors
- Liver Function Tests – for hepatic causes
- Platelet Antibody Studies, Factor Levels
- Heparin-Induced Thrombocytopenia (HIT) Assay
Disorders that Predispose to Clotting
- Thrombocytosis
- Polycythemia Vera
- Factor V Leiden
- Prothrombin Thrombophilia
- Antiphospholipid Syndrome
- Heparin-Induced Thrombocytopenia
Polycythemia Vera
- Myeloproliferative disorder characterized by increased RBCs, WBCs, and platelets
- Causes hyperviscosity, leading to sludge in the pipes
- Symptoms: headaches, hearing and vision changes, difficulty concentrating, itching/pain, night sweats, weight loss, flushing, cyanosis
- Physical Exam Findings: hypertension, redness or cyanosis of the skin from venous stasis, splenomegaly, signs/symptoms of DVTs, PE
- Lab Findings: elevated counts – WBCs, RBCs, platelets, decreased iron stores (iron, ferritin)
- WHO Criteria: all major criteria or the first 2 + minor
Factor V Leiden
- Cofactor for factor X
- One of the most common inherited causes of hypercoagulability
- Results from a single point mutation that causes resistance to Protein C
- Inherited in an autosomal dominant pattern
- Associated with placental abruption in pregnancy
Other Thrombophilias
- Prothrombin G20210A Mutation
- Protein C and Protein S Deficiencies
- Compound Heterozygotes – G20210A + FVL
DVT and PE
- Presentation: SOB, chest pain, cough, bloody sputum, pleuritic pain, shock/sudden death
- Testing: PERC – Pulmonary Embolism Rule-Out Criteria
- Diagnosis: IF low or intermediate risk but PERC+, send a d-dimer. If the d-dimer is positive, recommend imaging.
- Categories: low-risk, submassive, massive
- Treatment: anticoagulation, possible thrombolysis for massive PE
Prophylaxis and Thrombophilias
- IVC Filters
- DVT Prophylaxis: consider in patients with high risk of VTE, weighed against their bleeding risk
- Thrombophilias: test only if it will change treatment plan
Heparin-Induced Thrombocytopenia
- Caused by antibodies that form to heparin-platelet complex
- STOP heparin if this happens!
- Use alternative anticoagulation!
Vaccine Induced Thrombotic Thrombocytopenia (VITT)
- Diagnosis: must meet all 5 criteria
- Treatment: initiate therapy with non-heparin anticoagulation pending PF4 ELISA### Anemia and Thalassemia
- Anemia: too few circulating red blood cells or too little hemoglobin, resulting in decreased oxygen carrying capacity
- Three main categories of anemia:
- B: Bleeding Acutely (e.g. trauma or GI bleed)
- P: Production Problem (marrow not producing)
- H: Hemolysis (red cell breakdown)
Hemoglobin Structure
- Hemoglobin is the workhorse of RBCs, carrying oxygen for transport
- Hemoglobin A is a tetramer, consisting of two alpha (α) and two beta (β) chains
- Each chain has a heme group, allowing each hemoglobin to carry four oxygen molecules
Thalassemia
- Decreased mean corpuscular volume (MCV)
- Caused by mutations that alter beta globin chains
- Alpha thalassemias: deficient α chains (found later in life, not in newborns)
- More common in Southeast Asian, Indian, Southern Chinese, Middle Eastern, and African descent
Sickle Cell Disease
- Caused by mutations in the beta globin chain gene
- HbS: when valine replaces glutamic acid at Beta 6
- HbC: when lysine replaces glutamic acid at Beta 6
- Sickling begins when Hb S is exposed to deoxygenation, leading to irreversibly sickled RBCs
Pathophysiology of Sickle Cell Disease
- Sickled RBCs have a lifespan of 10-20 days, compared to 120 days for normal RBCs
- Spleen is responsible for destroying and recycling old RBCs
- Early use of hydroxyurea preserves spleen function
Sickle Cell Anemia
- Caused by beta globin mutations
- HbS and HbC are the most common mutations
- Sickling leads to vaso-occlusion, pain crisis, and other complications
Sickle Cell Complications
- Vaso-occlusive pain crisis
- Splenic sequestration
- Infections
- Stroke and central nervous system damage
- Acute chest syndrome
- Pulmonary hypertension
- Priapism
- Bone and joint damage (avascular necrosis)
- Leg ulcers
- Gall bladder and liver disease
- Renal disease
- Thrombosis
- Eye disease
anemia ppt test 6 clin med
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