9/24a Common Blood Disorders (Biomedical Sciences) Flashcards
(84 cards)
1
Q
what does the hematologic system consist of (big picture)?
A
Blood vessels throughout the entire body
involves multiple organs
Bone Marrow - for creation and maturation
thymus - WBCs and immune system so antigen presentation and antibody formation effect on formation of WBCs
Spleen is the filter of the hematologic system
2
Q
composition of the blood
A
- plasma (water (91%) and proteins (7%) - clotting proteins, albumin for osmotic stability, globulins - immune proteins antibodies
- formed elements - blood cells
1. RBCs are the most common
2. WBC (leukocytes, monocytes, neutrophils, eosinophils, basophils)
3. Platelets
4. Plasma
3
Q
primary function of hematologic system
A
- through red blood cells
- responsible for delivering oxygen to various tissues
- hemoglobin molecules have iron that help bind oxygen to transfer them to tissues in gas exchange
4
Q
functions of the hematologic system
A
- delivery oxygen of substances for cellular function
- removal of wastes
- immune function
- injury repair
- maintain acid/base balance
5
Q
how does waste get removed in the hematologic system?
A
: delivery of waste products to spleen, liver, and kidneys for excretion and filtration
6
Q
what is the purpose of oxygen in the hematologic system?
A
delivery oxygen of substances for cellular function
7
Q
immune function of hematologic system?
A
WBCs are infection fighting cells, leukocytes (neutrophils are the most common and respond to bacterial infections); Lymphocytes respond to viral infections
8
Q
injury repair of hematologic system?
A
: hemostasis and appropriate inflammatory response, gets initiated by platelets
9
Q
how does hematologic system maintain acid/base balance?
A
:CO2 is an acid and the exchange in respiratory and renal systems makes sure the pH balance is maintained
10
Q
Hematopoesis
A
creation of blood cells
- occurs in bone marrow of adults
- embryonic yolk sac, fetal liver and spleen in the fetal development
11
Q
process of hematopoesis
A
- starts with hematopoetic stem cells (pluripotent)
- reside in bone marrow
- differentiate in response to signals that come from other organs (thymus > bone marrow to produce lymphocytes > response to antigen presentation)
12
Q
extramedullary hematopoesis
A
adult liver or adult spleen plays a role in the creation of blood cells in abnormalities/pathology
13
Q
what does it mean to be pluripotent?
A
same stem cell differentiates into a variety of cell types
- Erythrocyte
- Megakaryocyte
- leukocyte
- -B&T Lymphocytes - thymus to bone marrow
- -Monocytes
- -Neutrophils
- -Eosinophils
- -Basophils
14
Q
lymphoid cell vs myeloid cell
A
- Lymphoid cell matures into a BRT lymphocyte
- Myeloid cell becomes a red blood cell, a megakaryocyte, or any kind of white blood cell (in response to cytokine activity and antigens from foreign invaders)
15
Q
Erythrocytes
A
Red Blood Cell
- hemoglobin and hematocrit are used to look at function of RBC
- principle function is to delvier oxygen
- can’t deliver oxygen if there is not a lot of hemoglobin
- bi-concave cell that has flexibility to get through small vessels without lysing open
- circulates for 90-120 days and travels to spleen to remove the iron from RBC
16
Q
Hemoglobin and RBC relationship
A
RBc is the vehicle for hb to function in the delivery of oxygen.
RBC without hb does no good in a clinical perspective
Hb lies on the outside/periphery of the RBC
17
Q
after the RBCs lifespan, what happens to it?
A
circulates for 90-120 days and travels to spleen to remove the iron from RBC
IRON is conserved and recycled - tightly by the spleen
Iron is contained within Hb and it acts to bind with the oxygen
Hb goes to the liver and gets excreted as bile into the GI tract
18
Q
hemoglobin normal values
A
male - 14-18g/dL
female - 12-16 g/dL
newborn - 14-24 g/dL
CRITICAL VALUE - <8.0g/dL
19
Q
RBCs normal values in the body
A
male - 4.7-6.1 million
female - 4.2-5.4 million
20
Q
what does HB require for formation?
A
Iron
B-12
Folic Acid
21
Q
what is hematocrit?
A
percentage of blood volume that is made of red blood cells (hemoglobin x 3)
lab parameter
~45% for most patients
if the value is not in normal range:
too high: RBCs are abnormally large
too low: More white blood cells/plasma than they should
22
Q
if RBC, HGB, and HCT values are low, what is the issue?
A
anemia
23
Q
Causes of anemia
A
- iron deficiency
- b12 and folate deficiency
- blood loss
- hemolysis
- bone marrow failure
- chronic disease
- dilution
- pregnancy
- genetic
24
Q
pathways that cause anemia
A
- not forming blood cells properly
- either losing or destroying blood cells (after surgery, trauma, genetics)
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High RBC, HGB, or HCT counts are caused by
Erythrocytosis/Polyceythemia
- hypoxic states
- lung disease
- smoking
- high altitudes
- dehydration
- polycythemia vera
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Symptoms to watch for with anemic patients
- dyspnea, tachypnea (elevated RR)
- increased HR
- weakness
- fatigue
- palor
- low blood pressure
- syncope
- watch out for orthostatic blood pressure
- chest pain - biggest reason for transfusion
- light-headedness
- Unique symptoms = pica, craving for non-food items (ice or paint chips)
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cause of anemia
- most common = iron deficiency
- -reason why in the US is because of GI blood loss
- -reason why world wide = dietary
- menstrual breathing
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Treatment for anemia
- oral iron supplements
- intravenous iron infusion
- transfusion of RBCs
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severe/acute anemia
transfuse RBCs
| or intravenous iron formulation
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Vitamin B12 and folate deficiencies
- due to lack of intake or absorption
- both absorbed in GI tract
- interfere with DNA synthesis of RBCs
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causes of B12 and folate deficiencies
- B12 = malabsorption could be why because it is absorbed in the terminal ilium with intrinsic factor from parietal cells
- Malabsorption of folic acid is NOT common b/c it is absorbed everywhere in the GI tract
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Unique Symptoms of B12 and folate deficiency
- neuropathy from B12 deficiency
- if it is not treated early on, the symptoms can become permanent
- balance, proprioception, neuropathy issues
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Blood loss anemia
due to trauma, surgery or other blood loss
-bone marrow does try to keep up by generating increased numbers of immature red blood cells (reticulocytes) because of stem cells
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symptoms of blood loss anemia
more pronounced fatigue, pallor, tachycardic, syncope, orthostasis
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Anemia of chronic disease
```
underlying pathophysiology is from inflammation
common chronic diseases
- anemia of chronic kidney disease
-liver disease
-autoimmune/CT disorders
-malignancies
-thryroid disease
```
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treatment for blood loss anemia
iron supplementation or blood transfusion (avoid until Hb is at ~<=7-8
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treatment of anemia of chronic disease
if patient has had low hemoglobin for years, their bodies are well compensated for the blood loss especially in comparison to those that have acute anemia
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Common chronic diseases that can cause anemia
- Kidney disease on dialysis
- liver disease
- autimmune/CT disorders
- malignancies
- thyroid diseases
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treatment for chronic anemia
Focused on underlying disease
kidney diesease: lack of erythropoetin production
RBCs get formed in response to erythropoetin in response to low circulating oxygen levels
-patients get injections of erythropoetin to produce more RBCs
40
what is the treatment for rheumatologic/CT auto-immune anemia?
-inflammation and inflammatory proteins prevent absorption of B12 and folic acid
41
bone marrow failure
hematopoetic stem cell comes from bone marrow
- aplastic anemia is a bone marrow disorder where there is damage to the stem cell (no production of ANY blood cells)
- -idiopathic for the most part, possible industrial toxins and radiation therapy, and viral illness
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Symptoms of aplastic anemia
fatigue, weakness (from anemia), infection due to lack of WBCs, and hemmorhage due to lack of platelets to lead to hemostasis
--associated with low cell count in all of the various cell lines
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treatment for bone marrow failure
bone marrow transplant to replace hematopoetic stem cell that has been damaged
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Sickle cell anemia
- hereditary cause
- primarily in African patients
- unstable Hb molecule (Hb S instead of Hb A)
- inherited in an autosomal recessive fashion
- had protective effects for malaria endemic areas
- transfers normal RBCs into sickle RBCs in presence of hypoxia or stress.
45
Unique symptoms of sickle cell anemia
1. Vaso-occlusive pain crises:
Getting stuck in vessels because they don't have the elliptical shape, get clumped together, and don't transport oxygen effectively. Patients have acute chest pain and pain in the long bones. If there is chest pain, they have to be treated very urgently. Cells hemolyze very easily.
2. Avascular necrosis: occurs in the shoulders and hips due to chronic vaso-occlusive crises affecting the bone
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treatment for sickle cell anemia
- pain control: strong narcotics because of chronic pain starting at a young age
- Transfusions (blood or exchange): when vast majority of the RBCs are sickle cells, the blood is removed and restored with normal blood cells
- Oxygen
- Hydroxyurea: chemotherapy that helps prevent normal sickling
- Hydration
- Joint replacement surgeries
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Precautions to take when working with a patient who has sickle cell anemia
- avoid over exertion
- make sure patient is well hydrated
- monitor pulse ox
- limit use of cryotherapy (can cause stress and increase potential for sickling)
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White Blood Cells (differentials)
- principle function: immune and inflammatory responses
- normal range: 5,000-10,000 cells/mcL
- Differential - types of white blood cells (a-e = most common to least common)
a. neutrophil: respond to bacterial infections
b. lymphocytes: respond to viral infections
c. monocytes: innate immune system, act as macrophages
d. eosinophils: respond to parasitic infections and mediate the allergic response
e. basophils: help mediate inflammation
49
Low WBC counts
Leukopenia
- bone marrow failure (aplastic anemia)
- overwhelming sepsis (consumption of all WBCs in process of trying to fight infection)
- viral infections
- malignancy
- medications (chemotherapy, antibiotics - most common)
- genetic (benign ethnic neutropenia, patients always have a relatively low level of WBCs)
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Most common reason of leukopenia
medication response, chemotherapy and antibiotics
51
High WBC counts
Leukocytosis
- bacterial infection (pneumonia, cellulitis, UTI)
- inflammation (after surgery)
- stress
- trauma
- medications (steroids for autoimmune diseases)
- malignancy: leukemias
52
Leukopenia/neutropenia precautions
low WBC count, particularly low on neutrophils (most common) there is increased susceptibility to infection (<1000 neutrophils)
- more strict with infection control
- strict hand hygiene, possibly using gloves
- mask and gown if we are a carrier for anything (even a minor virus)
- sterilized equipment
- single use to patient then thrown out
53
leukemias
four main types:
(acute = overproduction of immature blood cells, chronic = overproduction of mature forms of blood cells
lymphocytic - hematopoetic then effects B and T cell , myeloid- affects neutrophils, basophils, and eosinophils)
1. acute lymphocytic - in children, good prognosis with chemotherapy
2. acute myeloid - older children, pathopneumonic finding, auer rods
3. chronic lymphocytic - most common in adults
4. chronic myeloid - uniqe finding, philadelphia chromosome (BCR ADL gene)
54
When would you see a patient with leukemias?
- general deconditioning
- to improve functional status
- ECOG score that reviews their functional status and whether they can get chemotherapy or not.
55
Lymphomas
- similar to lymphacitic leukemias, but involve lymphatic organs instead of cells in circulation
- --thymus, spleen, and lymph node
- two main types: Hodgkin's and Non-hodgkins
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Hodgkin's disease
- a type of lymphoma
- typically seen in young adults
- good prognosis with chemo and radiation
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platelets
principle function: hemostasis (appropriate clotting) and inflammatory response
- not really cells, just clumps of cells
- come from megakaryocytes
- form plugs that prevent bleeding
- release signals to WBCs and inflammatory mediators in response to inflammation mediation.
- contain growth factors and receptors
58
how long to platelets circulate for and what is the normal range of platelets in the body?
- circulate for 7-10 days
| - normal range: 140,000-400,000
59
Anti-platelet medications
aspirin, binds to platelet irreversibly (for the life of the platelet), thus not playing a role in the platelet plug foundation
-before surgery need to be off of aspirin for ~7 days
60
When is the risk for spontaneous bleeding?
when platelet counts are less than 10-20k
- spontaneous intracranial hemmorhage is the main concern
- patients should only do ADL
61
What are the recommended platelet counts for different procedures?
- if procedure is invasive, platelets should be over 100,000 and they should be able to do passive resistance exercises
- paracentisis (body fluid sampling procedure) platelets should be over 50,000
- if platelet count is 10-20k, it yields spontaneous bleeding and therapy needs to just be ADL
62
Low platelet counts
thrombocytopenia
- consumption: hemmorhage
- destruction: autoimmune processes, decreased production, bone marrow failure
- sequestration: hypersplenism (hyper active spleen)
- dilution
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High platelet counts
thrombocytosis
- essentially, it is idiopathic
- reactive: bleeding, inflammation, infections, exercise
- polycythemia vera: high RBC and high Hb, overproduction of multiple cell lines
64
signs or symptoms of patients with low or high platelet count?
Low: bleeding disorders (typically involving mucous membranes), easy bruising (petiquie - small tiny red dots that can't be blanched - shins or other independent areas), perpera (small subcutaenous capillary bruising), echmotic bruises
High: stroke, heart attack, or other arteriole clotting complication from not getting adequate blood flow, unable to maintain circulation
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Precautions of low platelet counts
- <20k only ADL
- <50k light exertion
- <100k limit heavy exertion and grade, light passive resistance but nothing that would increase the risk of spontaneous bleeding
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Other bleeding disorders
-hemostasis: platelets, then fibrin production through clotting factor proteins from the liver, continues into vessels
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Lab testing for hemostasis issues and determining patient's risk for bleeding
- aPTT: activated partial thromboplastin time, patients on heparin therapy have this monitored
- PT/INR: prothrombin time and international normalized ratio, patients on coumadin therapy or worfarin have this monitored. >4.9m, the higher the INR goes, the more prone the patient is for bleeding disorders
68
von willebrand disease
- genetic condition that causes a bleeding disorder
- lack von-willebrand factor that goes into fibrin for clotting
- asymptomatic unless there is a bleeding challenge (surgery or trauma)
- 2 types
- treatment is with DDAVP (desmopresin), used prophylacticly during surgeries and aminocaproic acid during acute bleeds
69
hemophilia
- genetic recessive condition
- x-linked, effects male patients
- presents at an early age
- deficiency of clotting factors: Factor VIII, hemophilia A; Factor IX, hemophilia B
- joint deterioration and damage because they bleed into the joints (spontaneous or result of very minor trauma)
- hemearthrosis
70
treatment for hemophilia
transfusions of clotting factors they are deficient in
donated by blood donors who have normal levels of clotting factors:
-cryoprecipitate
-fresh frozen plasma
Synthetic clotting factors:
-recombinant clotting factors
-packed red blood cells
-avoid joint replacement, but manage with conservative PT
71
PT in patients with hemophilia
- positive for QOL, pain control, mobility, and joint range of motion
- avoid any sort of grade 5 manipulation, no excessive force or velocity
- aerobic exercise is very good for these patients, but make sure that they are not actively bleeding
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precautions for patients with bleeding disorders?
- ensure the patient is not actively bleeding
- make sure that the patient is adequately treated
- if patient has pain, parasthesias, numbness, swelling, discontinue therapy and refer to physician or PA
- ensure that vital signs are stable
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Clotting disorders
- main concern is embolism
- risk factors through virchow's triad
1. stasis (venous) from prolonged inability if patient is under anesthesia for a while, immobilized through cast/splint or just overall bedridden (not moving every two hours)
2. Vessel wall injury - surgery cuts some capillaries and possibly small veins or arteries, OR traumatic incidence. body wants to prevent bleeding/hemmorrhage, but if it becomes disregulated there can be excessive clotting
3. hypercoagulability - inhertited genetically, estrogen therapies (BC), smoking, acquired disease states (malignancy/cancer), autoimmune diseases, hepatitis
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Presentation of Venous thromboembolism (VTE) and clotting disorders
```
Deep Vein Thrombosis (DVT)
Pulmonary Embolism (PE)
```
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DVT
- calf pain and tenderness - medially and posterior knee, will feel a rope-like texture
- redness
- swelling
- warmth
- ***typically unilateral
- worse with dorsiflextion (maybe because not much evidence)
- +/- palpable cord (really just textbook)
- not life-threatening, but concerned because that's where PEs start
- can be asymptomatic
76
PE
Signs/Symptoms of DVT
- chest pain and shortness of breath (most common)
- tachycardia, tachypnea, hypoxia
* *First vital organ the clot would go to is the lungs!!
- don't want to forcible dorsiflex foot because we can lodge the DVT to the lungs and cause the PE
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diagnosing DVT/PE
- ultrasound for DVT (urgent)
| - Catscan of vasculature in lungs for PE (emergent)
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genetic clotting disorder patients that are at increased risk for clotting disorders and their treatment
1. factor V leiden disorder (20% of americans have a variation) autosomal dominant
a. Treatment includes monitoring until clot has occurred, at that point treat with anticoagulation therapies
b. If patient has two copies of the factor V or is high risk (taking estrogen or have cancer) put on anticoag before clot even occurs
2. protein C and/or Protein S deficiency (regulate clotting pathway). Not having them leads to ongoing clot formation. Higher risk than factor V leiden.
a. Treat with prophylactic anticoagulation before clot has even occurred.
b. Can administer protein C to turn off clotting cascade.
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is mobility therapy safe in patients with DVT?
YES, as long as they are aware of it and have been treated for it.
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is mobility therapy safe for patients with PE?
It is safe to ambulate and participate in light exertion and ambulation as long as their vital signs remain controlled.
want to be really cautious with this
81
Signs and symptoms for patients with clotting disorders
- evidence of clot in leg, calf pain, tenderness, swelling, issues with light dorsiflexion
- ask patient if there is any chest pain or trouble breathing.
- pay particular attention to the vital signs on HR, RR, and oxygen levels. Tachypnea, Tachycardia, or hypoxia
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things to watch out for with PTT
critical value is if the time to clot is greater than 100s
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critical values with PT/INR
patient should be around 2
if they are around 5-6 we will be very cautious with therapy
if they are greater than 6-7 we will hold off with PT
84
post thrombotic syndrome
pain and discomfort after DVT and can continue to cause discomfort and movement is actually prescribed for post-thrombotic syndrome
