(F) L1: RBC Disorders (Part 1: Anemia and Polycythemia) Flashcards
- A condition characterized by an elevated number of red blood cells
- It signifies an excess of RBCs which can result in increased blood viscosity and related complications
Polycythemia
- A condition that is characterized by a lower than normal amount of red blood cells or reduced amount of hemoglobin in the blood
- Reflects as a deficiency of RBCs leading to oxygen carrying capacity problems
Anemia
TOF: Anemia and Polycythemia are related
True (both involved in the imbalance of RBC number but in OPPOSITE directions)
- The process by which the RBCs are produced in the body (in the bone marrow)
- It begins with hematopoietic stem cells which are considered multipotent (can differentiate into various cell types)
Erythropoiesis
Hematopoietic stem cells differentiate into proerythroblasts under the influence of what hormone which is produced where?
Erythropoietin (in the kidneys)
Note: This is in response to LOW oxygen levels
At what red cell stage does hemoglobin accumulation begin?
Note: Hgb enables RBCs to carry oxygen
Erythroblast
As erythroblasts mature, their nuclei (swell/shrink) and are eventually (expelled/binded to the cell)
- Shrink
- Expelled (it allows for more space for Hgb)
Red cells without a nucleus but contain residual organelles and ribosomes are called?
Reticulocytes
How many days do reticulocytes circulate in the bloodstream before they lose their remaining organelles and mature into fully functional RBCs?
1-2 days
Polycythemia or Anemia?
↑ Hgb, Hct, and RBC count
Polycythemia
Polycythemia or Anemia?
↓ Hgb, Hct, and RBC count
Anemia
Refers to the TRUE increase/decrease of Hgb and Hct
Absolute
Refers to the FALSE increase/decrease of Hgb and Hct
Relative
- The condition wherein the increase of RBC concentration is due to a false increase in the number of the red blood cells
- A result of decreased plasma volumes due to dehydration and other factors
- The actual RBC volume is not increased but Hct is, so it leads to the appearance of this condition
Relative Polycythemia
Relative polycythemia is aka “stress polycythemia” or what syndrome?
Gaisbock Syndrome
Causes of Relative Polycythemia
- This is one of the most common causes of relative polycythemia
- Refers to when the body loses excessive amounts of fluids through sweating or not consuming enough water leading to a reduced plasma volume
- It causes RBCs to become more concentrated
Dehydration
Causes of Relative Polycythemia
- This is caused by diarrhea, vomiting, or severe bleeding which contributes to decreased plasma volume leading to false increased RBC concentration
Fluid Loss
Causes of Relative Polycythemia
- These are medications that are frequently recommended to patients suffering from heart failure and hypertension
- It can increase urine output leading to dehydration and decreased plasma volume
Diuretics
Familiarize yourself with the 6 causes of relative polycythemia
- Dehydration
- Fluid loss
- Burns
- Diuretic use
- Alcohol and smoking
- Prolonged tourniquet application
Characterized by a true increase in the number of RBCs wherein the bone marrow produces too many RBCs leading to a higher hematocrit and hemoglobin level
Absolute Polycythemia
What is the other name for Primary Polycythemia?
Polycythemia Vera
- This is a rare, chronic myeloproliferative disorder in which the BM overproduces RBCs, WBCs, and platelets
- Often caused by a mutation of JAK2 gene (a JAK2 V617F mutation)
- These mutated cells are continuously produced even in the absence of the body’s normal regulatory signals
- It results in a true increase in RBC number
Polycythemia Vera/Primary Polycythemia
Polycythemia Vera/Primary Polycythemia
This is a gene that provides instruction for producing kinase
Janus Kinase (JAK2)
Polycythemia Vera/Primary Polycythemia
This mutation is characterized by a single point mutation in which valine is replaced by phenylalanine at position 617 of the JAK2
JAK2 V617F Mutation
Valine (V) at the 617th position replaced by Phenylalanine (F)
Polycythemia Vera/Primary Polycythemia (S&S)
Blood hyperviscosity can lead to conditions like deep vein thrombosis or pulmonary embolism which is caused by the formation of what?
Clot
Polycythemia Vera/Primary Polycythemia (S&S)
- This condition causes the blood flow to become impaired in the circulatory system, so the oxygen supply to the various tissues and organs becomes insufficient
- The patient may experience low fatigue and dizziness
Hyperviscosity of blood
Polycythemia Vera/Primary Polycythemia (S&S)
Blood hyperviscosity causes our spleen to (shrink/enlarge) which contributes to the sequestration or trapping of the blood cells
Enlarge
Polycythemia Vera/Primary Polycythemia (S&S)
This occurs after exposure to warm water
Itching (Pruritus)
Polycythemia Vera/Primary Polycythemia (S&S)
This condition is accompanied by reddish/bluish coloration of the hand
Burning pain in hand
Polycythemia Vera/Primary Polycythemia (Lab Findings)
- (High/Low) blood count
- (High/Low) ferritin
- (Increased/Decreased) ESR
- (High/Low) LDH
- (Hyper/Hypo)-uricemia
- (High/Low) Vit. B12
- (High/Low) EPO
- (Hyper/Hypo)-plastic bone marrow
- High blood count
- Low ferritin
- Decreased ESR
- High LDH
- Hyperuricemia
- High Vit. B12
- Low EPO
- Hyperplastic BM
- This occurs in response to external factors that stimulate the body to produce more RBCs
- The primary mechanism of this is the body’s attempt to increase the oxygen carrying capacity in response to low oxygen levels (hypoxia)
Secondary Polycythemia
Causes of Secondary Polycythemia
When oxygen is reduced at high locations, the body is triggered to produce more RBCs to compensate
High altitudes
Causes of Secondary Polycythemia
Conditions such as emphysema or COPD can result to inadequate oxygenation of the blood leading to secondary polycythemia
Chronic Lung Disease
Causes of Secondary Polycythemia
Conditions such as congenital heart defects can reduce oxygen delivery to the tissues stimulating the production of more RBCs
Heart Disease
Causes of Secondary Polycythemia
Due to exposure to carbon monoxide, it can lead to reduced oxygen carrying capacity of the blood leading to increased RBC production
Smoking
Causes of Secondary Polycythemia
There is production of tumors that can produce EPO leading to secondary polycythemia
Erythropoietin-producing tumors
- A condition characterized by the deficiency of RBCs or a reduced amount of hemoglobin in the blood
- Results in the decreased ability of the blood to carry oxygen
Anemia
Anemia (S&S)
Most common sign and symptom of anemia
Fatigue
Anemia (S&S)
This is due to a drop in BP
Dizziness and Lightheadedness
Anemia (S&S)
Due to insufficient oxygen supply to the brain
Headache
Anemia (S&S)
Due to poor circulation in the extremities
Cold hands and feet
Anemia (S&S)
- Refers to the tendency to eat non-nutritive and non-food substances like ice, soap, chalk, clay, and metal objects
- Most common in children, pregnant women, and those with certain mental health conditions
- May be linked to nutritional deficiencies and psychological factors
Pica
Familiarize yourself with the 12 signs and symptoms of anemia
- Fatigue
- Pale skin
- Shortness of breath
- Weakness
- Dizziness and lightheadedness
- Headache
- Cold hands and feet
- Chest pain
- Cognitive and concentration problems
- Brittle nails
- Pale/yellow conjunctiva
- Pica
Tests for Anemia
- The initial and most common test to diagnose anemia
- It measures various components of the blood
Complete Blood Count
Tests for Anemia
What RBC index can you use to classify the anemia as micro, normo, or macrocytic (acc. to size)?
Mean Cell Volume (MCV)
Tests for Anemia
What RBC index can you use to determine the average amount of Hgb concentration in the RBCs?
Mean Cell Hemoglobin Concentration (MCHC)
Tests for Anemia
- This should be examined under the microscope to visually inspect the size, shape, and appearance of the RBCs
- It can also help differentiate various types of anemia such as megaloblastic, hemolytic, and iron-deficiency
Peripheral Blood Smear
Tests for Anemia
- Helps assess the bone marrow’s response to anemia by measuring the young and immature RBCs
- Performed for anemia diagnosis
- A relatively accurate reflection of the amount of effective RBC production taking place in the bone marrow
Reticulocyte Count
Tests for Anemia
Increased RBC destruction and blood loss results to (increased/decreased) reticulocyte count
Increased
Tests for Anemia
In cases where there is defective hemoglobin synthesis, replacement of the normal marrow by tumors, or failure of the bone marrow to produce cells, the reticulocyte count is (increased/decreased)
Decreased
Tests for Anemia
This type of Iron Study:
- Measures the amount of iron in the blood
Serum Iron
Tests for Anemia
This type of Iron Study:
- Measures the capacity of the blood to bind iron
Total Binding Capacity (TBC)
Tests for Anemia
This type of Iron Study:
- Is calculated by dividing the serum iron by the total binding capacity and can also indicate how much iron is bound to transferrin
Transferrin Saturation
Tests for Anemia
This type of Iron Study:
- Measures the body’s iron stores
Serum Ferritin
Tests for Anemia
What is the normal percentage of nucleated RBCs being of erythroid origin (M:E ratio)?
20-35%
Tests for Anemia
What is the normal Myeloid:Erythroid (M:E) ratio?
4:1
Tests for Anemia
When bone marrow biopsy is performed in the case of anemia, an iron stain should be done to determine the percentage of what cells?
Sideroblasts
Note: The presence/absence of ring sideroblasts must be noted
Tests for Anemia
An estimation of the iron stores may be determined by performing iron staining using what stain?
Prussian blue
Tests for Anemia
This analyte indicates destruction of RBCs found in hemolytic anemias
Bilirubin
Tests for Anemia
- This may also be performed but it is less specific
- Increased levels signify hemolytic anemia and in some cases, ineffective erythropoiesis (megaloblastic anemia)
Serum LDH
Anemia According to Cause
Identify if acc. to:
A. Decreased/Impaired RBC Production
B. Increased RBC Destruction
- Bone marrow disorders
- Nutritional deficiencies
- Decreased EPO
- Globin synthesis defect
- Iron overload
- Ineffective erythropoiesis
A. Decreased/Impaired RBC Production
Anemia According to Cause
Identify if acc. to:
A. Decreased/Impaired RBC Production
B. Increased RBC Destruction
- Intrinsic RBC defect
- Non-immune acquired hemolytic anemia
- Immune hemolytic anemia
B. Increased RBC Destruction
What is the #1 cause of anemia which can be seen in bone marrow disorders?
Decreased/Impaired RBC Production
These types of anemia are characterized by abnormal bone marrow function leading to ineffective RBC production
Clue: 2 answers
Aplastic anemia or Myelodysplastic anemia
If there is iron deficiency anemia, there is inadequate intake or absorption of what?
Iron
In this type of anemia, there is a lack of folate which may hinder the DNA synthesis in the RBC precursors leading to megaloblastic anemia
Folate Deficiency Anemia
A decrease in this usually causes hormonal imbalances or conditions that can affect the endocrine system and can interfere with the body’s ability to produce RBC
Erythropoietin
Thalassemia, sickle cell anemia, or congenital erythropoietic anemia are all classified under this kind of defect which can result in impaired RBC production
Defects in Globin Synthesis
Hemolytic anemia can be categorized as intrinsic or extrinsic depending on whether the destruction originated where?
Clue: 2 answers
Inside or Outside the RBC
- This type of hemolysis involves factors within the RBC that causes destruction
- Common causes include hemoglobinopathies, enzyme deficiencies, and membrane disorders
Intrinsic Hemolysis
- This type of hemolysis can occur due to factors from the external RBC environment
- Can be caused by autoimmune hemolytic anemia
Extrinsic Hemolysis
In this type of hemolytic anemia, the immune system frequently produces antibodies that attack and destroy the RBCs
Autoimmune Hemolytic Anemia
TOF: Anemia can be idiopathic or be secondary to conditions like Lupus or Lymphoma
True
What extrinsic factor may lead to hemolysis by directly damaging the RBCs or by triggering an immune response against them?
Infection
Blood loss anemia is also known as what?
Hemorrhage Anemia
- This usually occurs if there is a significant loss of blood from the body leading to reduction in RBCs and decreased hemoglobin levels
- This type of anemia can result from acute or chronic bleeding
Blood Loss
Acute or Chronic Bleeding?
- Injuries, surgeries, or gastrointestinal bleeding
- From ulcers or bleeding disorders
Acute Bleeding
Acute or Chronic Bleeding?
- Inflammatory and bowel diseases
- Menstrual periods or certain cancers
Chronic Bleeding
A. Microcytic/Hypochromic
B. Macrocytic/Normochromic
C. Normocytic/Normochromic
< 80 fL.
A. Microcytic/Hypochromic
A. Microcytic/Hypochromic
B. Macrocytic/Normochromic
C. Normocytic/Normochromic
Greater than 100 fL.
B. Macrocytic/Normochromic
A. Microcytic/Hypochromic
B. Macrocytic/Normochromic
C. Normocytic/Normochromic
80-100 fL.
C. Normocytic/Normochromic
A. Microcytic/Hypochromic
B. Macrocytic/Normochromic
C. Normocytic/Normochromic
- Thalassemia
- Anemia of Chronic Disease
- Iron Deficiency Anemia
- Lead Poisoning
- Sideroblastic Anemia
A. Microcytic/Hypochromic (TAILS)
A. Microcytic/Hypochromic
B. Macrocytic/Normochromic
C. Normocytic/Normochromic
- Megaloblastic Anemia
- Non-megaloblastic Anemia
B. Macrocytic/Normochromic
A. Microcytic/Hypochromic
B. Macrocytic/Normochromic
C. Normocytic/Normochromic
- Increased reticulocyte count
- Decreased reticulocyte count
- Hemoglobinopathies
C. Normocytic/Normochromic
A. Megaloblastic Anemia
B. Non-megaloblastic Anemia
- Vitamin B12 and folic acid deficiency
- Pernicious anemia
A. Megaloblastic Anemia
A. Megaloblastic Anemia
B. Non-megaloblastic Anemia
- Hypoproliferative anemia
- Refractory anemia
- Liver disease
- Hemolytic anemia
- Acute blood loss anemia
B. Non-megaloblastic Anemia
A. Increased Retics.
B. Decreased Retics.
C. Hemoglobinopathies
- Acute blood loss anemia
- Hemolytic anemia
A. Increased Retics.
A. Increased Retics.
B. Decreased Retics.
C. Hemoglobinopathies
- Hyperproliferative anemia
- Myelopathic anemia
B. Decreased Retics.
A. Increased Retics.
B. Decreased Retics.
C. Hemoglobinopathies
- Renal and Endocrine Diseases
- PNH
C. Hemoglobinopathies
In this classification acc. to morphology:
The red cells are smaller than normal and is often associated with a decrease in MCV which measures the average size of the RBC
Microcytic Hypochromic
In this classification acc. to morphology:
- The RBCs are larger than normal and the MCV is increased indicating a larger than average RBC
- Can be seen in megaloblastic anemia and non megaloblastic anemia
Macrocytic Normochromic
In this classification acc. to morphology:
Characterized by RBCs of normal size and an MCV within the expected range
Normocytic Normochromic