Hemotologic System Flashcards
What are the components of blood?
Plasma and formed elements
What are the formed elements of blood?
Erythrocytes (RBCs)
Leukocytes (WBCs)
Thrombocytes (platelets)
What are the blood forming tissues?
Liver
Bone marrow
Thymus
Spleen
Lymph nodes
Where are plasma proteins formed?
In the liver
What blood components are formed in the red bone marrow?
Erythrocytes, leukocytes, and thrombocytes
Where are leukocytes formed?
Red bone marrow
Thymus
Spleen
Lymph nodes
As adults, are many leukocytes formed in the thymus?
No, bc the thymus is pretty much non existing by the time we reach 20 yo
What is hematopoiesis?
Erythrocytes formation in red bone marrow
Where is there red bone marrow?
Vertebrae, sternum, ribs, skull, scapulae, pelvis, proximal limb bones
All parts of the hematologic system come from what cells?
Hematopoietic cells
Hematopoietic cells becomes either ________ or ________
Common myeloid progenitor, common lymphoid progenitor
Does the hematologic system change much with age?
Nope
T/f: blood composition changes little with age
True
What about the hematologic system changes with age?
The % of marrow space occupied by hematopoietic tissue declines progressively
T/f: hematocrit and hemoglobin levels should remain WNL as you age
True
T/f: low values of blood counts should be considered pathological with the elderly
True
Changes in blood levels in the elderly is pathological and associated with what things?
Nutritional deficiencies and other medical conditions
The s/s of hematologic disorders manifest as disorders of the______ system
Circulatory
Circulatory disorders can be characterized by …
Edema and congestion
Infarction
Thrombosis and embolism
Lymphedema
Bleeding and bruising
Hypotension and shock
When does shock occur?
When the circulatory system is unable to maintain adequate pressure to perfuse organs
What are the common clinical signs of shock?
Tachycardia
Tachypnea
Cool extremities
Decreased pulses
Decreased urine output
Altered mental status
What are the three types of shock?
Hypovolemic shock
Cardiogenic shock
Vasoplegic shock
What is hypovolemic shock?
Shock from loss of blood volume
What are the causes of hypovolemic shock?
Hemorrhage
Vomiting
Diarrhea
Dehydration (from decreased fluid intake, diabetes, or inadequate rehydration)
What is cardiogenic shock?
Sudden loss of the hearts ability to maintain cardiac output
What are some causes of cardiogenic shock?
Arrhythmias
Acute vascular dysfunction
Acute MI
Severe CHF
Cardiomyopathy
Obstructive valvular disease
Cardiac tumor
Sepsis
What is vasoplegic shock?
Large decrease in vascular resistance
What are some causes of vasoplegia?
Bacteremia
SCI (any vessel below level of injury will dilate)
Pain
Trauma
Vasodilator drugs
Burns
Pancreatitis
Anaphylaxis
Liver failure
If we increase the diameter of vessel, what happens?
Decrease in BP
What is involved in the medical management of hematologic deficiencies?
Blood transfusions
Erythropoietin stimulating agents
Do we usually do whole blood transfusions or just components of blood?
We usually only transfuse the components needed
What blood components may we transfuse?
Platelets
RBCs
Specific clotting factors
What are some severe rxns to blood transfusions?
Transfusion related acute lung injury (TRALI)
Acute hemolytic transfusion rxn
What is the most severe blood transfusion rxn?
Acute hemolytic transfusion rxn
What is transfusion related acute lung injury?
When the donor antibodies attack the host leukocytes in the pulmonary vasculature
T/f: transfusion related acute lung injury (TRALI) can cause death and usually requires intubation and ventilation but often is reversible
True
What is acute hemolytic transfusion rxn
A rare but severe reaction to a transfusion when there is an ABO mismatch (wrong blood type given) causing the donor cells to destroy the host cells
What are the moderate blood transfusion rxns?
Allergic rxn/anaphylaxis
Septic rxn
Transfusion associated circulatory overload (TACO)
What is a common rxn to blood transfusions, especially in those who receive multiple transfusions?
Allergic rxns
What are septic reactions caused by?
Bacterial contamination
What blood component usually causes septic rxns?
Platelets
What is transfusion associated circulatory overload (TACO)?
When the circulatory system can’t maintain normal fxns with the increased volume of a transfusion
What pts receiving blood transfusions are more susceptible to transfusion associated circulatory overload (TACO)?
Older pts
Pts who have kidney or heart disease
What is the most common and mild rxn to a blood transfusion?
Febrile nonhemolytic rxn
What is a febrile nonhemolytic rxn following a blood transfusion?
Fever during or within 4 hours of receiving a transfusion resulting from the donor blood factors becoming reactive
T/f: febrile nonhemolytic rxn following a blood transfusion is transient
True
Is there evidence that we should or shouldn’t work with transfusion patients?
Nope, many time we will
How long should we avoid working with a pt who has received a blood transfusion?
At least 30 minutes
What is a delayed hemolytic transplant rxn?
A delayed transfusion reaction in those who’ve previously received a blood transfusion with minor mismatches that causes failed rise or fall in hemoglobin after a transfusion
What are erythropoietin stimulating agents?
Meds that replace human EPO to stimulate erythropoiesis in people with reduced RBC production
Erythropoietin stimulating agents are often used in what patients?
Those with anemia of chronic disease
Those with chronic kidney disease
Those with cancer
What is bloodless medicine?
Caring for people with minimal to no use of blood products
What are the goals of care when using bloodless medicine?
To manage chronic anemia, minimize blood loss, and optimize hemostasis
What precautions need to be taken with bloodless medicine?
Iron and folate supplements
EPO
surgical techniques to minimize blood loss
Early intervention for bleeding
Minimize blood draws
Acceptance of lower hemoglobin levels
What are the PT implications of bloodless medicine?
OH, RPE, and symptoms based approach to care
What is anemia?
Reduced quantity or quality of RBCs in the blood leading to reduced oxygen carrying capacity
What are normocytic RBCs?
Normal sized RBCs
What are macrocytic RBCs?
Abnormally large RBCs
What are microcytic RBCs
Abnormally small RBCs
What are normochromic RBCs?
Normal hemoglobin
What are hyperchromic RBCs?
High concentration of hemoglobin in each RBC
What are hypochromic RBCs?
Low concentration of hemoglobin in each RBC
What is anisocytosis?
Various size RBCs?
What is poikilocytosis?
Various shaped RBCs
What is the symptom of any disease that affects the balance of RBCs production or destruction?
Anemia
What causes iron-deficiency anemia?
Blood loss, pregnancy, malabsorption
What population is most likely to have iron deficiency anemia?
Menstruating or pregnant women
Growing children
People with low SES
Older adults
Young female athletes
Why may young female athletes have iron deficiency anemia?
Deficient diet
Iron loss through sweat
Menstruation
Peripheral disruption of RBCs with foot strike
What is the #1 source of iron in the US through food?
Fortified cereals
In older adults, what is likely the cause of iron deficiency anemia?
Malabsorption
In younger people, what tends to be the cause of iron deficiency anemia?
Not enough iron in the diet
Why is folate deficiency anemia so much less common in the US now?
Bc many foods have folate supplementation bc deficiency can cause neural tube defects in gestation
What B vitamin deficiency can cause anemia?
B12 deficiency
What is the most common cause of B12 deficiency anemia?
Pernicious anemia
What is pernicious anemia?
An autoimmune condition in which autoantibodies attack intrinsic factors
What are common causes of B12 deficiency anemia?
Vegetarian/vegan diet
Bariatric surgery
Bacterial growth
Excessive antacid use
Excessive alcohol
Why are there neuro symptoms associated with B12 deficiency anemia?
Bc B12 is essential for myelinating neurons
What are the neurological symptoms associated with B12 deficiency anemia?
Dementia
Ataxia
Neuropathic pain
What is anemia of chronic disease?
Any condition associated with increased inflammatory chemicals that decreases responsiveness to erythropoietin (EPO)
What are the causes of anemia of chronic disease?
Infections, malignancies, inflammation, chronic kidney disease, DM
T/f: chronic kidney disease can cause a decrease in EPO and lead to anemia of chronic disease
True
What are the s/s of anemia?
Tachycardia
OH
Weakness
Fatigue
Dyspnea on exertion
Pallor
Dizziness
Chest pain
Leg cramps with exercise
What are hemoglobinopathies?
Abnormal formation of hemoglobin that affects the oxygen carrying capacity of the blood
What are the two most common hemaglobinopathies?
Sickle cell anemia
Thalassemia
T/f: sickle cell disease is an autosomal recessive disease
True
What is sickle cell disease?
An autosomal recessive disease that causes abnormal hemoglobin formation (sickle shaped RBCs)
What ethnicities are more affected by sickle cell anemia?
African Americans, those from the Mediterranean, Saudi peninsula, and India
What is a theory as to why those from African, the Mediterranean, Saudi peninsula, and India more likely to have sickle cell disease?
Bc it is believes that sickle cell disease protects against malaria
What is sickle cell trait?
Inherited abnormal gene from only one parent for abnormal hemoglobin that is typically asymptomatic
What is the pathogenesis of sickle cell disease?
Hemoglobin becomes deformed when deoxygenated and the RBCs become sickled
What are the clinical manifestations of sickle cell disease?
Tissue ischemia
Thrombus formation
Sclerosis of blood vessels
Chronic anemia
Why is thrombus and ischemia an issue with sickle cell disease?
Bc the sickle shape of the RBCs makes them more likely to stick to the vessel walls and cause a clot/occlusion
What is a sickle cell crisis?
When a large proportion of RBCs are sickled at the same time
What factors may precipitate a sickle cell crisis?
Physiologic stress such as infection, hypoxia, dehydration, extreme temps, and alcohol consumption
What is acute chest syndrome?
A severe life threatening complication of sickle cell disease that causes an embolism to become lodged in the pulmonary vessel
What are the 3 severe life threatening complications of sickle cell disease?
Acute chest syndrome
Stroke
Splenic sequestration
What are the signs of acute chest syndrome.
Severe chest pain
Fever
Congestion
Cough
Dyspnea
Tachypnea
Sternal or costal retractions
Wheezing
Acute chest syndrome is very common in what population
Children with sickle cell disease
What are the signs of a stroke?
Balance
Eyes
Face
Arms
Speech
Time
What is splenic sequestration?
A severe life threatening complication of sickle cell disease where a large ischemic area of the spleen causes necrosis
Why is ischemia of the spleen so life threatening in sickle cell disease?
Bc the spleen is a large storage area of RBCs
What are the s/s of splenic sequestration?
Weakness
Abdominal pain
Fatigue
Dyspnea
Tachycardia
Pallor
Hypotension
What do we do as PTs for sickle cell disease?
Risk prevention
Watch for s/s of acute chest syndrome, stroke, and splenic sequestration
Pain management during episodes
Address kinesthesia
What can we educate pts with sickle cell disease on?
Adequate hydration
Physical activity for pulmonary health
Prophylactic vaccines and meds
How can we manage pain during episodes in sickle cell disease?
MILD heat
Visualization
Relaxation
Biofeedback
Jt protection with slings and positioning
What is thalassemia?
A group of diseases caused by either deletion or mutation of the genes responsible for the alpha and beta globin chains for hemoglobin
How many alpha globin chains are there normally in hemoglobin?
4
Alpha thalassemia results from what?
A deletion of one or more alpha globin genes leading to anemia
T/f: severe anemia is alpha thalassemia often leads to significant cardiac complications and death
True
How is alpha thalassemia treated.
With transfusions
When one alpha gene is deleted in alpha thalassemia, what results?
An asymptomatic carrier
When 2 genes are deleted in alpha thalassemia, what results?
Mild anemia (alpha thalassemia trait)
When 3 alpha genes are deleted in alpha thalassemia, what results?
Severe anemia (most serious form)
When all 4 alpha genes are deleted in alpha thalassemia, what results?
Death bc this is incompatible with life
What results in beta thalassemia?
Mutations in the genes responsible for the beta globin chains
The severity of beta thalassemia is dependent on what?
The # and type of mutations
T/f: beta thalassemia results in mild to severe anemia
True
What is the big effect of beta thalassemia other than anemia?
Iron overload
How is beta thalassemia treated?
With transfusions and iron chelation that breaks down excess iron that would otherwise end up building up in body tissues
What are the complications of anemia and iron overload in beta thalassemia?
Endocrinopathies
Osteoporosis
Cardiomyopathy
Liver cirrhosis
What do endocrinopathies cause?
Abnormal hormone production
What is hereditary hemochromatosis?
An autosomal recessive disease that causes increased iron absorption
T/f: symptoms in hereditary hemochromatosis can range from mild to severe
True
What are the clinical manifestations of hereditary hemochromatosis?
Weakness
Chronic fatigue
Myalgias
Joint pain
Abdominal pain
Hepatomegaly
Elevated hemoglobin
Elevated liver enzymes
The progression of hereditary hemochromatosis may lead to what?
Destructive arthritis or acute inflammatory arthritis
What are the PT roles in caring for pts with hereditary hemochromatosis?
Maintaining flexibility
Strength
Proper joint alignment
Promote fxn
Prevent falls
Prevent loss of ADL independence
Ads, orthotics, and/or splints
What is hemostasis?
Arrest of bleeding after blood vessel injury that involves the interaction among the blood vessel wall, the platelets, and the plasma coagulation proteins
What is primary hemostasis?
The process that involves formation of a platelet plug at the site of vascular injury
What is secondary hemostasis?
Process triggered when vascular damage exposes tissue factor and results in formation of a fibrin plug
What is the outcome of primary hemostasis?
Formation of a platelet plug
What is the outcome of secondary hemostasis?
Formation of a fibrin clot
What are the requirements for primary hemostasis?
Normal # of platelets
Normally fxning platelets
Von Willebrand factor
What are the requirements for secondary hemostasis?
Tissue factor
Clotting factor VII
Clotting factor IX
Clotting factor X
When disorder of primary hemostasis is present, what is the presentation?
Easy brushing
Mucosal bleeding
When disorder of secondary hemostasis is present, what is the presentation?
Serious bleeding events
What is a disorder of primary hemostasis?
Von Willebrand disease
What is a disorder of secondary hemostasis?
Hemophilia
What is the pathogenesis of Von Willebrand disease?
Decreased platelet adhesion
What is Von Willebrand disease?
Deficiency/dysfxn of Von Willebrand factor
What is Von Willebrand factor?
Plasma protein that combines with factor VIII to cause platelets to adhere to each other
What are the clinical manifestations of Von Willebrand disease?
Easy bruising
Mucosal bleeding
Continued bleeding after minor trauma
What is hemophilia?
An X linked recessive disease that causes an inability to form a stable clot
Which type of hemophilia is more common and involves a lack of factor XIII?
Hemophilia A
Which type of hemophilia is less common, not as serious, and involves a lack of factor IX?
Hemophilia B
Why is hemophilia rare in females and more common in males?
Bc hemophilia is an X linked disease and females have two X chromosomes, they have a backup if they get an affected X chromosome , but males only have one X chromosome so they have no backup if they get a mutated one
What are the clinical manifestations of hemophilia?
Spontaneous bleeding
Severe bleeding with trauma or surgery
What joint bleeds occur in hemophilia from most to least common?
Knee, hip, ankle, elbow, hip, shoulder, wrist
How are jt bleeds in hemophilia managed in the acute stage?
Factor replacement
RICE
Pain free movt
NWB/ minimal WB
Pain meds
Splitting and support as appropriate
How are jt bleeds in hemophilia managed in the subacute stage?
Factor replacement (if indicated)
Progressive WB movt and exercises
Weaning from splints and slings
What is hemophilic arthropathy?
Jt bleeds that lead to spontaneous joint fusion
What muscle bleeds are most common in hemophilia?
Iliopsoas
Quads
Gastroc
Forearm flexors
What can muscle bleeds in hemophilia lead to?
Compartment syndrome
What is a medical emergency that can occur following a specific muscle bleed in hemophilia?
Iliopsoas bleed
How are muscle bleeds managed in hemophilia?
Factor replacement
RICE
Progressive movt
Appropriate WB status
Bed rest for Iliopsoas bleeds
What are the s/s of compartment syndrome?
Excessive pain
Tight skin
Swelling
Neuro deficits (numbness, tingling, and weakness in the peripheral nerve distribution)
What are the s/s of Iliopsoas bleed?
Femoral nerve damage
Groin pain
Pain with hip ext
Decreased sensation in L2-4
Weak knee ext
Weak hip flex
What is the role of PT in an acute bleeding in hemophilia?
Pain control
Maintain positioning
Prevent deformity
What is contraindicated in PT for acute bleeds in hemophilia?
Heat!
What is the role of PT in prevention of bleeds in hemophilia?
Progressive strengthening
Muscle re-education
Gait training
Education
What are the characteristics of a level 1 bleed in hemophilia?
Most fragile joints
Target jts
Previously injured muscle
Painful P/AROM
No swelling or bleeding in the last 2 weeks
What are the characteristics of a level 2 bleed in hemophilia?
Muscle/jt with a hx of bleeding
Chronic mild to moderate impairment
No bleeding for the past 6 months
What are the characteristics of a level 3 bleed in hemophilia?
Jts and muscles with a minimal amount of bleeding
No impairments
When can we progress in supervised strength training for hemophilia?
If there are no adverse rxns to the previous week of exercise
What is involved in prophylactic care for hemophilia prior to supervised strength training?
Factor infusion or meds
How do we determine the intensity of supervised strength training for hemophilia?
Using a % of isometric dynamometery testing to assess pounds of weight used or color of TB to be used
If dynamometry reveals a 2.5 lbs weight should be used, what color TB should be used?
Yellow
If dynamometry reveals a 4.5 lbs weight should be used, what color TB should be used?
Red
If dynamometry reveals a 5 lbs weight should be used, what color TB should be used?
Green
If dynamometry reveals a 7.5 lbs weight should be used, what color TB should be used?
Blue
If dynamometry reveals a 9 lbs weight should be used, what color TB should be used?
Black
If dynamometry reveals a 15 lbs weight should be used, what color TB should be used?
Silver
During week 1 of supervised strength training for hemophilia, what are the level 1 parameters?
40% intensity
1x10
During week 2 of supervised strength training for hemophilia, what are the level 1 parameters?
45-50% intensity
2x10-20
During week 3 of supervised strength training for hemophilia, what are the level 1 parameters?
50-60% intensity
3x10-20
During week 4 of supervised strength training for hemophilia, what are the level 1 parameters?
55-60%
3x10-20
During week 5 of supervised strength training for hemophilia, what are the level 1 parameters?
60-70% intensity
3x10-20
During week 6 of supervised strength training for hemophilia, what are the level 1 parameters?
65-75% intensity
3x10-20
During week 1 of supervised strength training for hemophilia, what are the level 2 parameters?
50% intensity
1x10
During week 2 of supervised strength training for hemophilia, what are the level 2 parameters?
55-60% intensity
2x10-20
During week 3 of supervised strength training for hemophilia, what are the level 2 parameters?
60-70% intensity
3x10-20
During week 4 of supervised strength training for hemophilia, what are the level 2 parameters?
65-75% intensity
3x10-20
During week 5 of supervised strength training for hemophilia, what are the level 2 parameters?
70-75% intensity
3x10-20
During week 6 of supervised strength training for hemophilia, what are the level 2 parameters?
75% intensity
3x10-20
During week 1 of supervised strength training for hemophilia, what are the level 3 parameters?
60% intensity
1x10-20
During week 2 of supervised strength training for hemophilia, what are the level 3 parameters?
65-75% intensity
2x10-20
During week 3 of supervised strength training for hemophilia, what are the level 3 parameters?
70-75% intensity
3x10-20
During week 4 of supervised strength training for hemophilia, what are the level 3 parameters?
75% intensity
3x10-20
During week 5 of supervised strength training for hemophilia, what are the level 3 parameters?
75% intensity
3x10-20
During week 6 of supervised strength training for hemophilia, what are the level 3 parameters?
75% intensity
3x10-20
What is thrombocytopenia?
Decrease in platelets <150,000 g/dL
What are some causes of thrombocytopenia?
Decreased production of platelets
Increased destruction of platelets
Splenic sequestration
What are some symptoms of thrombocytopenia?
Mucosal bleeding
Easy bruising
Petechiae (small purple bruising)
What is thrombocytopenia associated with?
Use of nonsteroidal antiinflammatories
Acquired disorders of platelet fxn can occur through use of what?
Aspirin and other NSAIDs
A single dose of aspirin can suppress normal platelet aggregation for how long?
48 hours or longer
What is disseminated intravascular coagulation?
Uncontrolled activation of coagulation and fibrinolysis that causes both clotting and bleeding at the same time
How does disseminated intravascular coagulation cause both clotting and bleeding at the same time?
Bc all the clotting factors go to specific areas causing clotting and the rest of the body has no clotting factors, leading to bleeding
What may cause disseminated intravascular coagulation?
Sepsis, cancer, massive trauma
What are the clinical manifestations of disseminated intravascular coagulation?
Thrombotic events
Bleeding events
What is leukemia?
Malignant neoplasm of the blood-forming cells that replaces the normal bone marrow with a malignant clone of lymphocytic or myelogenous cells
What is the type of leukemia with the worst prognosis?
Acute myeloid leukemia
What are the 4 types of leukemia?
Acute myeloid leukemia
Acute lymphoblastic leukemia
Chronic myeloid leukemia
Chronic lymphoblastic leukemia
What leukemias affect the common myeloid progenitor?
Myeloid leukemia
What leukemias affect the common lymphoid progenitor?
Lymphoblastic leukemia
What are the characteristics of acute leukemia?
Rapid clinical progression
Proliferation of immature blood cells
>20% blast cells in bone marrow
What are the characteristics of chronic leukemia?
Slow clinical progression
Proliferation of mature cells
Some cells can still fxn
Over proliferation of abnormal cells leads to bone marrow overcrowding
Why is chronic leukemia slower progressing and have fewer symptoms?
Bc there are still some functioning cells
What is lymphoma?
Malignant changes of lymphocytes beginning in the lymph nodes or spleen spreading via the lymphatic system
What is Hodgkin lymphoma?
A largely curable lymphoma characterized by Reel-Sternberg cells
What is Non-Hodgkin lymphoma?
A highly variable type of lymphoma
What is multiple myelomas?
A lymphoma affecting the bone marrow balance or osteoclastic activity and osteoblastic activity
What is a serious complication of sickle cell disease?
Acute chest syndrome
T/f: chronic leukemia is a progression of acute leukemia
False
It is a medical emergency when bleeding occurs in which muscle with hemophilia?
Iliopsoas
Thalassemia is a disorder of what?
Hemoglobin
What PT intervention can help to prevent joint or muscle bleeds for people with hemophilia?
Supervised strength training
Which of the following is the most appropriate for a LE jt with an acute hemophilic bleed: gait training, heat application, progressive strengthening, cryotherapy
Cryotherapy
What is the risk of using cryotherapy for someone with sickle cell disease?
Sickle cell crisis
