Erythrocyte Disorders) Flashcards
In a CBC:
RBC measures
Hemoglobin measures
Hematocrit measures
MCV measures
MCHC measures
RBC = # of red blood cells
Hemoglobin = Amount of hemoglobin in blood (Red color, oxygen carrying)
Hematocrit = Percent of volume that RBCs take up in blood
MCV = Average volume (size) of red blood cells
–>Microcytic, normocytic, Macrocytic
MCHC = Concentration of hemoglobin in an average red blood cell
–> Hypochromic, Normochromic
Anemias are defined on the basis of….
Cell Size (MCV) and amount of Hgb (MCH)
If MCV is less than the lower limit of normal it is what type of anemia?
Normal Range?
Greater than the upper limit?
Microcytic Anemia
Normocytic Anemia
Macrocytic Anemia
If MCH is less than the lower limit of normal, it is what type of anemia?
If MCH is within the normal range?
Hypochromic Anemia
Normochromic Anemia
An means
Emia means
An = Without Emia = blood
This is defined as a reduction in HEMOGLOBIN concentration in circulating blood, resulting in tissue hypoxia due to lack of oxygen-carrying capacity of blood
Anemia
Symptoms of this illness include pale skin and mucous membranes, jaundice (if hemolytic), Tachycardia, Breathlessness, Dizziness, and Fatigue
Anemia
There are three ways to become anemic…
Lose Blood
Destroy Too Much Blood (hemolysis)
Make Too Little Blood
Hemolysis (anemia) can happen for 2 reasons
Extracorpuscular
Intracorpuscular
When the cause of anemia is losing blood, it is what type of anemia?
When the cause is destruction of too much blood, it is what type of anemia?
When the cause is making too little blood, it is what type of anemia?
Blood loss (hemorrhage)
Hemolytic
Bone marrow suppression/nutritional deficiencies
The etiology of blood loss anemia is traumatic, acute blood loss NOT > 20%
At first the hemoglobin level is normal. After 2-3 days, we see what (have ribosomal RNA, and no nucleus)?
Chronic Blood loss is different, and due to…
Reticulocytes
Iron Deficiency Anemia
Hemolytic anemia is due to decreased red cell lifespan. It can be chronic or acute.
This type is inherited and due to red cell defects
This type is aquired
Intracorpuscular
Extracorpuscular
In hemolytic anemia, increased bilirubin, increased LDH, and decreased haptoglobin are signs of….
Destruction
In hemolytic anemia, increased reticulocytes and nucleated red cells in blood are signs of….
Production (of red cells?)
This extracorpuscular cause of hemolytic anemia is due to physical trauma to red cells, schistocytes (fibrin strands that shred RBCs), and triangulocytes. It is important to find out why it is occurring
Microangiopathic Hemolytic Anemia
The causes of this illness include:
Artificial Heart Valves Malignancy Obstetric Complications Sepsis Trauma
Microangiopathic Hemolytic Anemia
This type of anemia is characterized by IgG, the spleen, and spherocytes
Warm Autoimmune Hemolytic Anemia (Warm AIHA)
This type of anemia is characterized by IgM, complement, intravascular hemolysis, and agglutination
Cold Autoimmune Hemolytic Anemia (Cold AIHA)
This is the most common form of AIHA
_____ antibodies bind to RBCs best at 37 degree body temperature (98.6F)
What occurs in the spleen?
Warm AIHA
IgG
Splenomegaly
In Warm AIHA, these RBCs are formed from nibbling by macrophages (Kupfer cells) causing loss of membrane, leading to a spherical shape
Spherocytes
This is the less common AIHA
_____ antibody binds to RBCs at 0-4 degrees celsius, in the ears, hands, and toes in cold weather
It causes extravascular hemolysis via membrane attack complexes in what organs? (2)
Cold AIHA
IgM
Spleen and Liver
In cold AIHA, this is binding of IgM that causes cross linking of red cells, and sludging of blood
What can it ultimately lead to?
Agglutination
Raynaud’s Phenomenon
This is a test for AIHAs, it can detect the antibodies on RBCs using blood samples
Direct Coombs Test/Direct Antiglobulin Test
A determination of warm AIHA is positive for what?
A determination of cold AIHA is positive for what?
IgG
Compliment
Warm AIHA can be secondary to 2 things
It can also be induced by:
Lymphoproliferative Disorder and Autoimmune Disease
Drugs
Cold AIHA can be secondary to 2 things
Infection and Lymphoproliferative Disorder
What is found in the blood film of warm AIHA
Cold AIHA?
Warm: Spherocytes
Cold: Agglutination
This is an intracorpuscular anemia characterized by QUALITATIVE defect in hemoglobin (aka hemoglobinopathy)
Sickle Cell Anemia
This intracorpuscular anemia is characterized by a single AA substitution in the beta chain of hemoglobin
It can be heterozygous or homozygous, which has worse prognosis?
It creates these cells which are fragile (burst easily), and they get stuck in vessels
Sickle Cell Anemia
Homozygous
Sickle Cells
In sickle cell anemia, what kind of mutation occurs in the Beta chain gene, leading to abnormal beta chains?
The cell will aggregate and polymerize upon deoxygenation. They get stuck in vessels and are fragile.
Point Mutation (valine substituted for glutamate)
Sickle Cell Anemia is more prominent in what ethnic group?
It’s severity is variable
Blacks
What 3 things does sickle cell disease cause?
Treatment includes preventing triggers, vaccinating, and blood transfusion
Chronic Hemolysis, vaso-occlusive disease, and increased infections
This intracorpuscular anemia is a hereditary QUANTITATIVE defect in Hb. Not enough alpha or beta chains can be made. It has variable disease severity
Thalassemia
What type of anemia is thalassemia?
Hypochromic, microcytic with increased RBC and target cells
This blood disease causes tons of spherocytes with a spectrin defect.
What cures it?
Hereditary Spherocytosis
Splenectomy
Hereditary Spherocytosis is autosomal ______
It causes a defect in the RBC membrane due to a mutation in ______, a membrane protein
Dominant
Spectrin
Hereditary Spherocytosis causes spheroidal erythrocytes called ______ that are more vulnerable to sequestration/destruction by the spleen
On smears, red cells lack normal central pallor
Also, splenomegaly (enlargement of spleen)
Spherocytes
40-50% of patients with hereditary spherocytosis experience _____
Gall stones (cholelithiasis)
Glucose-6-Phosphate Dehydrogenase Deficiency causes increased ______ which cause cell lysis
Related to exposure to what kind of drug?
G6PD is self limiting
Peroxides
Oxidative Drug
What cells develop in G6PD due to removal of Heinz bodies?
Bite Cells
In G6PD, some patients are asymptomatic
Other have _____________ hemolysis triggered by broad beans (favism-Africa), drugs (antibiotics, aspirin, infections)
It resolves spontaneously
Episodic hemolysis
Look at chart on the role of G6PD on slide 62 related to pentose phosphate pathway
!
G6PD deficient cells die in part due to reduced ______, oxidants attack hemoglobin bonds
This causes heme to break away from globin, globin denatures and sticks to the RBC membrane, forming ______
This organ bites out Heinz bodies, leading to bite cells?
GSH
Heinz Body
Spleen
Anemia associated with making too little blood has too few building blocks, like this type
Or it can be due to too few erythroblasts, like this type
Or there could be not enough room, like this type
Iron Deficiency Anemia
Aplastic Anemia
Myelophthlistic Anemia