Hematology Flashcards
What 3 proteins make up plasma?
albumin, globulins, fibrinogen
blood is made up of what 3 cell types
Erythrocytes, leukocytes, platlets
Chief function of blood (5)
Carry O2 (RBC)
Delivery of substances needed for cellular metabolism
Removal of wastes
Defense against microorganisms and injury
Maintenance of acid-base balance (bicarbonate)
How much blood is in the body?
6 quarts
Plasma: Function of each
Albumins
Globulins
Fibrinogen
Albumins
Function as carriers (eg: medication) and control the plasma oncotic pressure
Globulins
Carrier proteins and immunoglobulins (antibodies)
Clotting factors
Mainly fibrinogen
Erythrocytes (RBCs) composition function structure life cycle
Floating bags of hemoglobin
Most abundant cell in the body
Responsible for tissue oxygenation
Biconcavity (increase surface area) and reversible deformity(can be squeezed)
120-day life cycle (3-4 month), do not go through cell devision
Erythropoietin (EPO): function location of function location of production what triggers production
EPO makes RBCs, work in the bone marrow
Produced by peritubular cells of the kidney (electrolyte, fluid, pH balance)
Hypoxic state in the kidney triggers production
Leukocytes (WBCs)
Granulocytes vs Agranulocytes
what type of WBC are each
function of each
Granulocytes:
phagocytes (neutrophils, eosinophils, and basophils).
Granules in cytoplasm contain enzymes that kill antigens.
Agranulocytes:
monocytes, macrophages, and lymphocytes—contain relatively fewer granules than granulocytes. Carry out inflammatory and immune functions, remove debris
.
Granulocytes:
Eosinophils- function
Eosinophils ingest antigen-antibody complexes
Induced by IgE hypersensitivity for immune fighting in parasitic infections
Histaminase to that help control inflammatory processes
Granulocytes:
Basophils- function
Central cell in inflammation, release histamine
Basophils = in blood
Mast cells = vascularized connective tissue (not WBC)
Lymphocytes are what 3 cell types?
T cells
B cells
Natural killer (NK) cells
Platlets
Structure
function
(Thrombocytes)
Disk-shaped cytoplasmic fragments
Essential for blood coagulation and control of bleeding
Thrombopoietin (TPO): function location of production location of function what triggers production LIfespan
- Hormone that stimulates the production and differentiation of megakaryocytes and is the main regulator of the circulating platelet numbers.
- TPO is primarily produced by the liver and induces platelet production in the bone marrow.
- Release b/c of trauma.
- Platelets circulate for 10 days before losing their functional capacity
Primary lymphoid organs
Secondary lymphoid organ
Bone marrow and thymus
Spleen, lymph nodes
All of the lymphoid organs link the hematologic and immune systems in that they:
Link hemo to immuno because of masses of lymphoid tissue containing macrophages, T cells, B cells.
Another way to say it: are sites of residence, proliferation, differentiation, or function of lymphocytes and mononuclear phagocytes (monocytes and macrophages)
Spleen:
function
Largest secondary lymphoid organ
filters blood
Phagocytosis of old, damaged, and dead blood cells are removed
Blood storage
Lymph nodes
function
Facilitates maturation of lymphocytes
Transports lymphatic fluid back to the circulation
Cleanses the lymphatic fluid of microorganisms and foreign particles
MPS
what cells is it made up of
function
organs (2)
(Mononuclear Phagocyte System)
Consists of monocytes/macrophages that differentiate without dividing and reside in the tissues for months or perhaps years
Cells of the MPS ingest and destroy microorganisms and foreign material
The MPS is mostly the liver and spleen
Hematopoiesis
the process of Erythrocytes (blood cell) production in the bone marrow
Two stages of Hematopoiesis
1) mitotic division (i.e., proliferation)
2) maturation (i.e., differentiation) into mature hematologic cells
what are Pluripotent stem cells
mother cells continuously going though cell division. Daughter cells mature into RBC (EPO); WBC/ Plat. (TPO)
Bone marrow
Proper name
red vs yellow
location
Called myeloid tissue
Red (active) and yellow bone marrow (fat storage)
Adult active bone marrow found in flat bones
Marrow located in: Pelvic bones, vertebrae, cranium and mandible, sternum and ribs, humerus, and femur
3 examples of when Hematopoiesis is stimulated
-Stimulus to increase WBC production: infectious exposure
WBC are getting used up
-Stimulus to increase plat. production: trauma and blood loss (TPO)
-Stimulus to increase RBC production: anemia reflected through hypoxia (EPO)
Erythropoiesis
What it is
what stimulates it
what occurs internally
the maturation of RBCs.
Stimulated by EPO during times of hypoxia.
In each step the quantity of hemoglobin increases and the nucleus decreases in size.
Erythrocytes
a red blood cell that (in humans) is typically a biconcave disc without a nucleus. Erythrocytes contain the pigment hemoglobin, which imparts the red color to blood, and transport oxygen and carbon dioxide to and from the tissues.
Hemoglobin
function
how many per RBC
Function of the Two pairs of protein subunits (globins)
Oxygen-carrying protein of the erythrocyte
A single erythrocyte contains as many as 300-400 million hemoglobin molecules
Each subunit contains iron-protoporphyrin complex (heme)
each heme carries 4 oxygen molecules
Nutritional requirements for hemoglobin synthesis
deficiency can lead to what?
Vitamins
B12, B6, B2, E, and C; folic acid; pantothenic acid; and niacin
(deficiency in B12 and Folate leads to decreased life span of RBC)
Destruction of Aged RBCs
who does the destroying
where is it done (primary and secondary)
Aged red cells (senescent) are destroyed by macrophages of the MPS
Primarily done in the spleen
The liver takes over if the spleen is nonfunctioning/absent
when Porphyrin (heme) are broken down, what happens
Porphyrin is reduced to bilirubin (pigmented- yellow/green wast from the breakdown of heme), transported to the liver, and secreted in the bile
Leukopoeisis: Leukocytes and Agranulocytes
where do each come from
when are they released?
Leukocytes arise from stem cells in the bone marrow
Leukocytes mature in the bone marrow
Agranulocytes are released into the bloodstream before they fully mature
Thrombopoeisis
is the development of platelets. Platelets (thrombocytes) are derived from stem cells that differentiate into megakaryocytes. During thrombopoiesis, the megakaryocyte nucleus enlarges and becomes extremely polyploidy without cellular division.
Endomitosis:
The megakaryocyte undergoes the nuclear phase of cell division but fails to undergo division (googlie eyes)
The megakaryocyte expands due to the doubling of the DNA and breaks up into fragments (platelets)
Hemostasis means:
the arrest of bleeding by formation of blood clots at sites of vascular injury.
Hemostasis requires (5):
1- Platelet plug "the gum in the hole" 2-Clotting factors (leads to the production of fibrin "the cement over the gum") 3- Blood flow and shear forces 4- Endothelial cells 5- Fibrinolysis
Hemostasis: Result of Vascular Spasm/Vasoconstriction in response to injury
Spasm induces vasoconstriction that inhibits amount of blood flow through the damaged area and blood loss
Plateplet plug Process (5)
Platelet activation (Calcium is essential for activation)
Adhesion to damaged vascular wall
von Willebrand factor (vWF) makes platelets sticky
Platelet degranulation
Aggregation adherence increases
Platelets to wall and each other
Activation of the clotting system and development of an immobilizing meshwork of platelets and fibrin
the 4 most important proteins that make up the Common Pathway of Coagulation and how they effect each other
Factors X and V activate prothrombin into thrombin. Thrombin then converts fibrinogen into fibrin, which becomes a fibrin clot.
Clot retraction
what it is
what facilitates this
end result
Fibrin strands shorten, become denser and stronger to approximate the edges of the injured vessel and site of injury
Facilitated by large numbers of platelets within the clot and actin-like contractile proteins in the platelets
Pulls wound/tissues together to facilitate repair
Lysis of blood clots = Fibrinolytic system (3)
(Fibrinolysis)
Plasminogen and plasmin breakdown)
Fibrin degradation products
D-dimers (bi-product)
Hematology Evaluation (testing) (3)
bone marrow function Blood tests (main way to test for hemo. issues) Hematocrit: ratio of RBC to overall volume
-cytosis=
Erythrocytosis
Leukocytosis
Thrombocytosis
-cytopenia (-penia)=
erythrocytopeniaa
leukocytopenia
thrombocytopeni
(increased cell count, too many)
1- erythrocytosis (polycythemia, the disorder)= too many RBC, changes viscosity making blood more thick= hypercoagulation
2- leukocytosis= too many WBC, not worrisome sign of normal protective response. Insanely high and abnormal is concerning b/c poss leukemia
3- thrombocytosis (thrombocythemia)= too many platelets, greater risk of forming clots= hypercoagulation
(deficiency in the cell, not enough)
1- erythrocytopenia= anemia leading to tissue hypoxia
2- leukocytopenia= immune suppression (most often decrease in neutrophils)
3- thrombocytopenia= won’t be able to make a clot, increased bleeding risk
What is Anemia?
Low RBCs
Anemia classifications based on Morphology and Hemoglobin concentrations
-CYTIC/ -CHROMIC
Size (Morphology):
Identified by terms that end in -CYTIC
Macrocytic (too big in size), microcytic (too small), normocytic (just right in size)
Hemoglobin content (how much hemoglobin):
Identified by terms that end in -CHROMIC
Normochromic (normal hemoglobin) and hypochromic (not enough hemoglobin). Cannot have “too much”!
You can have: Macro Normo; Micro Hypo; Normo Normo
Anemia: Anisocytosis vs Poikilocytosis
Red cells are present in various sizes (rare)
Red cells are present in various shapes
Describing sickle cell
Anemia:
Physiologic manifestations
Classic anemia symptoms
Physiologic s/s: Reduced oxygen-carrying capacity
Symptoms: Fatigue, weakness, dyspnea (SOB, turning up res. response), and pallor (pale)
What breaks down a clot
Plasmin
What is the bi-product of platelet break down?
D-dimer
Macrocytic-Normochromic Anemias (3)
what they are
Pernicious, megaloblastic, folate deficiency
The cell is too big but have an abundance of hemoglobin
Macrocytic-Normochromic: Pernicious anemia cause Results in Symptoms Treatment
Can be caused by a lack of intrinsic factor from the gastric parietal cells (most common)
Required for vitamin B12 absorption
Results in vitamin B12 deficiency due to absorption not often a lack of B12 in diet (B12 obtained from red meat and eggs)
Typical anemia symptoms with possible neurologic manifestations. Nerve demyelination (poss. neuropathy)
Others: Loss of appetite, abdominal pain, beefy red tongue (atrophic glossitis), icterus, and splenic enlargement
Treatment: Parenteral or high oral doses of vitamin B12
Macrocytic-Normochromic: megaloblastic anemias
cause
Characterized by
Caused by deficiencies in vitamin B12 or folate (these vitamins gives RBC their life span, decrease will shorten life span)
Characterized by defective DNA synthesis
Macrocytic-Normochromic: Folate deficiency anemia
cause
Symptoms
Treatment
Not dependent on any other factor
Absorption of folate occurs in the small intestine
Similar symptoms to pernicious anemia except neurologic manifestations generally not seen
Treatment requires daily oral administration of folate
Microcytic-Hypochromic Anemias (3)
what it is
Characterized by red cells that are abnormally small and contain reduced amounts of hemoglobin (sm. b/c not make enough hemoglobin)
1) Iron deficiency (Disorders of iron metabolism)
2) Sideroblastic (Disorders of porphyrin and heme synthesis, mitochondrial disfunction)
3) Thalassemia (Disorders of globin synthesis, genetic)
Microcytic-Hypochromic: Iron deficiency
symptoms
Most common type of anemia worldwide
Nutritional iron deficiency
Metabolic or functional deficiency
causes: Brittle, thin, coarsely ridged, and spoon-shaped nails. Glossitius (A red, sore, and painful tongue)
Koilonychia
Brittle, thin, coarsely ridged, and spoon-shaped nails. Caused by iron deficiency
Microcytic-Hypochromic: Sideroblastic anemia
cause
Characterized by
Altered mitochondrial metabolism causing ineffective iron uptake and resulting in dysfunctional hemoglobin synthesis
Ringed sideroblasts within the bone marrow are diagnostic
Ringed sideroblasts
Sideroblasts are erythroblasts that contain iron granules that have not been synthesized into hemoglobin
Microcytic-Hypochromic: Thalassemia
what is it
cause
Characterized by
Genetic disorder that produces malformed hemoglobin chains
Originated in the Mediterranean region
Autosomal recessive trait
Estimated ~80 million carriers worldwide
Produce less hemoglobin and have low number of RBCs (life long)
Normocytic-Normochromic (5)
what it is
Characterized by red cells that are relatively normal in size and hemoglobin content but insufficient in number of them
How you get it on following slides
Aplastic, Posthemorrhagic, Hemolytic, Sickle cell, chronic inflammation
Normocytic-Normochromic: Aplastic
Pancytopenia- (“pan” encompasses all) all blood cells are deficient (bone marrow problem)
Pure red cell aplasia- Due to low EPO (thus a disfunction in the kidneys)
Normocytic-Normochromic: Posthemorrhagic
Acute blood loss (probably due to trauma)
Normocytic-Normochromic: Hemolytic
Over destruction of red blood cells: due to spleen enlargement.
Autoimmune hemolytic anemias (eg: lupus)
Jaundice: wast of bilirubin from breakdown of RBC
Normocytic-Normochromic: Sickle cell
Autosomal recessive disorder that distorts hemoglobin proteins Causes RBC to “sickle” in shape Most often in times of hypoxia No biconcave disc! Risk of CVA
Normocytic-Normochromic: chronic inflammation
Mild to moderate anemia seen in:
AIDS, rheumatoid arthritis, lupus erythematosus, hepatitis, renal failure, and malignancies
Polycythemia
Too many red blood cells
Relative polycythemia
Result of dehydration (b/c it lowers the plasma volume)
Fluid loss results in relative increases of red cell counts and Hgb and Hct values
Absolute polycythemia
cause
too many RBC
Causes:
Abnormality of stem cells in the bone marrow making too many RBC
Polycythemia vera (PV)
or
Increase in erythropoietin (EPO) in response to chronic hypoxia (at the kidney). An inappropriate response to erythropoietin-secreting tumors
Leukocytosis
Leukocytosis is a normal protective physiologic response to stressors
Leukopenia
Leukopenia is not normal and not beneficial
A low white count predisposes a patient to infections
Infectious Mononucleosis (mono)
Acute, self-limiting infection of B-lymphocytes transmitted by saliva through personal contact
Commonly caused by:
Epstein-Barr virus (EBV) (85%)
Cytomegalovirus (CMV), hepatitis, influenza, HIV
Serious complications are infrequent
Fever, sore throat, swollen cervical lymph nodes, increased lymphocyte count
Splenic rupture is the most common cause of death
Leukemia
Cancers of excessive accumulation of leukemic cells in bone marrow
Lymphocytic (lymphoblastic)
Myelogenous
Acute leukemia (early maturation)
Chronic leukemia (late maturation)
were going to be lymphocytes (T, B, NKC)
were goingt to be RBCs, platelets, and granulocytes
- Presence of undifferentiated or immature cells (appear like stem cells), usually blast cells. Early in differation
- Predominant cell is more differentiated but does not function normally
ALM pediatrics
AML/ CML (chromosome 9, older adults)
Lymphadenopathy
Local lymphadenopathy
General lymphadenopathy
Enlarged (swollen) lymph nodes that become palpable and tender
Local lymphadenopathy
Drainage of an inflammatory lesion located near the enlarged node
General lymphadenopathy
Occurs in the presence of malignant or nonmalignant disease (lymphoma)
Lymphomas
Two major categories: Hodgkin lymphoma, Non-Hodgkin lymphomas
Malignant transformation of lymphocytes
Hodgkin lymphoma
Characterized by presence of Reed-Sternberg (RS) cells (googly eye cells)
Non-Hodgkin lymphomas
B-Cell Neoplasms
T & NK cell Neoplasms
Burkitt lymphoma
Most common non-Hodgkin lymphoma in children
Fast-growing tumor of the jaw and facial bones
Lesions in submandibular lymph nodes
Associated with EBV or HIV infection
Viral
Thrombocytopenia
What can it cuase
Platelet count <150,000/mm3
1) hemorrhage from minor trauma
2) spontaneous bleeding
3) severe bleeding (spontaneous bleeding, potentially fatal)
von Willebrand factor deficiency:
what is causes
used to adhere clot, cause increased bleeding risk
Vitamin K deficiency
what it is used for
what is causes
Vitamin K is necessary in the liver for synthesis and regulation clotting factors and anticoagulants
may not be about to make a clot, decreased coagulation
Vit K: dark leafy greens
Liver disease
what is causes
Liver disease causes a broad range of hemostasis disorders
Don’t make clotting factors, Decrease in TPO
Defects in coagulation, fibrinolysis, and platelet number and function
Hemophilia A Hemophilia B what is it how it is caused what is causes
Hemophilia A: deficiency in Clotting factor VIII
Hemophilia B: deficiency in Clotting factor IX
Christmas Disease
Transmission of A & B is via recessive X linked inheritance
1/3 of cases occur due to new mutations after birth, not inherited
All forms of hemophilia are relatively rare.
Cannot make a clot.
Disseminated Intravascular Coagulation (DIC) “Death Is Coming”
widespread clotting within vessels (leading to ischemia), activation of clotting factor to breakdown clots leading to possible hemorrhage
Complex highly fatal disorder in which clotting and hemorrhage simultaneously occur
Endothelial damage is the primary initiator of DIC
Most often from sepsis
Fibrin degradation product (FDP) and D-dimer levels increase
Stem cells
cells with the potential to develop into many different types of cells in the body. They serve as a repair system for the body.
Zymogen
-zyme= enzyme; -ogen= inactive protein
an inactive protein which is converted into an enzyme when activated by another enzyme
