Unit 3_ Hematological System

Question 1

What is the branch of science that studies the form, structure, and function of blood and blood-forming tissues?
plasma
formed elements
- erythrocytes, or red blood cells (RBCs)
- leukocytes, or white blood cells (WBCs)
- Platelets (thrombocytes)

Answer 1

Hematology

Question 2

What can alter the oxygen-carrying capacity of the blood and the constituents, structure, consistency, and flow of the blood?
- Hypo-coagulopathy or hyper-coagulopathy
- Increased work of the heart and breathing
- Impaired tissue perfusion
- Increased risk of thrombus

Answer 2

Hematologic conditions

Question 3

What includes a blood test panel or complete blood count (CBC) measures:
- Red blood cells (RBCs)
- White blood cells (WBCs) immunity
- Hemoglobin (Hb) oxygen-carrying protein
- Hematocrit (amount/proportion of red blood cells)
- Platelets (clotting)

Answer 3

Hematologic conditions

Question 4

What are general signs and symptoms of hematologic conditions?

Answer 4

Edema
Congestion
Infarction (brain, heart, GI tract, etc.)
Thrombosis
Splenomegaly
Embolism
Shock
- Rapid, weak pulse
- Hypotension
- Cool, moist skin
- Pallor

Question 5

When can the following occur?
- Decrease in RBC and lymphocyte production
- Slightly decreased hemoglobin
- Decreased differentiation –> increased innate immunity and reduced adaptive immunity
- Increased cytokine production –> chronic inflammation
- Comorbidities/malnutrition may contribute to hematological pathology

Answer 5

Aging

Question 6

What are reasons for blood transfusions?

Answer 6

Surgery
- Elective or repair after trauma
Trauma
Childbirth
Liver disorders
Hematological disorders such as hemophilia and anemia
Chemotherapy
Kidney failure

Question 7

What is the most common transfusion-related reaction? Causes headache, nausea/vomiting, hypertension, tachycardia.

Answer 7

Febrile nonhemolytic reaction

Question 8

What occurs due to volume overload from a rapidly infused transfusion? Results in shortness of breath, red face, tachycardia, hypertension, headaches, and seizures typically begin within 6 hours of transfusion.

Answer 8

Transfusion-associated circulatory overload (TACO)

Question 9

What is the most common cause of transfusion-related death? Neutrophil-specific antibodies in donor plasma are directed against recipient leukocytes in the pulmonary vasculature. Cause fever, hypotension, hypoxia, and pulmonary edema.

Answer 9

Transfusion-related acute lung injury (TRALI)

Question 10

What can result from giving a person the wrong blood or blood is mislabeled manifests as fever and flank pain?

Answer 10

Acute/delayed hemolytic transfusion reaction

Question 11

What can occur when a mild allergic reactions (such as pruritus and urticaria) are common (1%-3% of transfusions); anaphylaxis reaction is rare?

Answer 11

Allergic reaction

Question 12

What can occur from bacterial contamination of blood products?

Answer 12

Septic reactions

Question 13

What are the roles of a therapist when a patient is receiving blood transfusion?

Answer 13

Patients are not usually exercising or being treated by the physical therapist during a blood transfusion.

Exercise or therapy of any kind is not advised during the first 30 minutes (possibly up to 60 minutes) of the transfusion.

Monitor for adverse reactions
- Most occur during the first 15 minutes (e.g., fever, chills, urticaria, acute respiratory distress, transfusion-related acute lung injury)
- Signs of orthostatic hypotension

Consider
Why is the patient receiving a blood product?
What is the underlying medical condition and the goals of therapy for that day/week?
What is in the best interest of the individual’s health and safety?
- Will mobilizing the person during a blood transfusion be beneficial or detrimental?
- Can treatment be held (postponed) until a later time either later in the transfusion process or later in the day or the next day?

Question 14

What is hypoxia detected by kidneys?

• Stimulates release of erythropoietin (EPO).
• EPO promotes the production of RBCs in bone marrow
• An increase in RBC circulation increases available oxygen
• Hypoxia resolved –> EPO suppressed

Answer 14

Erythropoiesis

Question 15

What is a hereditary disorder characterized by excessive iron absorption by the small intestine?

• Excess iron is slowly deposited in cells, particularly in the liver, heart, pancreas, and, to a lesser extent, other endocrine glands (e.g., the pituitary gland).
• Weakness, chronic fatigue, myalgias, joint pain (particularly the second and third metacarpophalangeal joints [MCPs])

Answer 15

Hereditary hemochromatosis (HH)

Question 16

What therapist role includes:
Arthropathy occurs in 40% to 60% of individuals with hemochromatosis and can be the first manifestation of the disease.
- Not reversible and often continues to progress even with effective medical intervention.
- Twinges of pain on flexing the small joints of the hand, especially the second and third metacarpophalangeal joints. Involvement of these joints often helps to distinguish hemochromatosis-related arthropathy from osteoarthritis.
- Can progress to large joints, including the hips, knees, and shoulders resulting in disability

Therapeutic intervention:
- ROM, strength, and proper alignment to promote function, prevent falls, and prevent the loss of independence in activities of daily living.
- Evaluating the need for compensatory techniques, assistive devices, orthotics, and splints toward these goals.

Answer 16

hemochromatosis

Question 17

What is the decrease in the number of red blood cells? This is caused by:
- dietary deficiency (anemia caused by folate or vitamin B12 deficiency)
- acute or chronic blood loss (iron deficiency)
- congenital defects of hemoglobin (sickle cell disease)
- exposure to industrial poisons
- diseases of the bone marrow

Answer 17

Anemia

Question 18

What is classified into three main pathophysiologic states:
1. blood loss
2. decreased production of erythrocytes
3. peripheral destruction of erythrocytes

Answer 18

Anemia

Question 19

Signs and symptoms associated with anemia are related to the ______ of anemia.

Answer 19

Severity

Mild anemia –> fatigue

More advanced –> weakness, dyspnea on exertion, easy fatigue, pallor, tachycardia, increased angina in people with preexisting heart disease

Severe, anemia: heart failure, hypoxic damage to liver/kidneys

Question 20

What is the therapist’s role in managing anemia?

Answer 20

Exercise
- Discuss exercise plan with a physician
- Pacing and training that distribute the intensity of the workload over time
- Progress slowly for anyone with decreased exercise tolerance and monitor vital signs closely (are they out of shape or anemic?)

Precautions
- Knowing the underlying cause of the anemia
GI bleed?
- Bleeding under the skin and easy bruising in response to the slightest trauma often occur when platelet production is altered (thrombocytopenia)
- Decreased oxygen delivery to the skin results in impaired wound healing and loss of elasticity

Monitoring Vital Signs
- Many individuals who are anemic are asymptomatic
- Tachycardia, fatigue, generalized weakness, loss of stamina, exertional dyspnea, low diastolic blood pressure

Question 21

What results from malignant neoplasm of the blood-forming cells that replaces the normal bone marrow with a malignant clone?

It manifests as anemia and reduced tissue oxygenation from decreased erythrocytes.

Infection from neutropenia as leukemic cells are functionally unable to defend the body against pathogens.

Bleeding tendencies from decreased platelet production (thrombocytopenia)

Answer 21

Leukemia

Question 22

What’s the therapist’s role in managing Leukemia?

Answer 22

Precautions
- Thrombocytopenia
- Secondary infection prevention

Children
- Bone disease, joint ROM, strength, gross/fine motor performance
- Absence from school/learning/social experiences
- Cognitive deficits from neurotoxicity of radiation/chemotherapy

Question 23

What is an increase in the number of leukocytes in the blood in response to:
- Infection and leukemia
- Hemorrhage (GI bleed, skull fracture, etc.)
- Normal protective response to physiologic stressors such as strenuous exercise, emotional changes, temperature changes, anesthesia, surgery, etc.

Answer 23

Leukocytosis

Question 24

What is a a decrease in any or all white blood cells (neutrophils is the most common)?

It manifests as asymptomatic (and detected by routine tests).

Clinical signs and symptoms consistent with infection, such as sore throat, cough, fever.

Answer 24

Leukopenia

Question 25

What is increased number or proportion of basophils to other leukocytes in the blood or tissue? It includes chronic myeloid leukemia. Allergic and chronic inflammatory reactions or infections manifest.

Answer 25

Basophilia

Question 26

What is an increased number of eosinophils in tissue or blood? Eosinophils are a prominent feature of parasitic infections, allergy, asthma, and pulmonary and vascular disorders.

Answer 26

Eosinophilia

Question 27

What is an elevation in the number of neutrophils in the blood? It's caused by inflammation or tissue necrosis, acute inflammation, cancer.

Answer 27

Neutrophilia

Question 28

What is a reduction in circulating neutrophils and leads to an increased risk for infection?

Answer 28

Neutropenia

Question 29

What is an increase in monocytes, chronic infections, such as tuberculosis, syphilis, and subacute endocarditis, and other inflammatory processes?

Answer 29

Monocytosis

Question 30

What mediates the initial adhesion of platelets (to other platelets and endothelial collagen) at sites of bleeding injuries? It manifests as easy bruising and bleeding.

Answer 30

von Willebrand disease

Question 31

What are the therapist's roles for von Willebrand disease?

Answer 31

Prevention/monitoring - Wounds healing - Secondary infection Fall prevention Education

Question 32

What is mild, moderate, and severe decreases in clotting factors? It manifests as spontaneous bleeding: Joints (knee) and deep muscle leads to degradation chronically. - Chronic pain, decreased ROM, muscle atrophy, crepitus Excessive bleeding after trauma Risk for intracranial hemorrhage

Answer 32

Hemophilia

Question 33

What are the therapist's roles for hemophilia?

Answer 33

A shift from rehabilitation to prevention - Protective strengthening of the musculature surrounding affected joints, muscle reeducation, gait training, and client education. Physical Activity and Exercise - A regular exercise program, including appropriate sports activities, resistance training, cardiovascular/aerobic training, and therapeutic strengthening and stretching exercises for affected extremities, is an important part of the comprehensive care of the individual with hemophilia. - Help individuals identify, seek out, and enjoy physical activity, exercise, and sport participation that provides benefits that outweigh the risks - Client education early on for family, client, school personnel, and coaches for prevention, conditioning, and wellness. Maintaining Joint Range of Motion - The therapist and client must be alert to recognize any signs of early (first 24-48 hours) bleeding episodes. - RICE (rest, ice, compression, and elevation) principle to promote comfort and healing are two goals for treating an acute joint (hemarthrosis) or muscle bleed (intramuscular hemorrhage). - The joint range of motion can be measured during this acute episode in the pain-free range but should not be strength tested. - Static or dynamic night splints may be used to apply a low-load stretch to a muscle shortened because of an underlying condition such as synovitis or articular contracture.

Question 34

What is a decrease in platelet count? Etiological factors include: - Inadequate platelet production from the bone marrow, - Increased platelet destruction outside the bone marrow It manifests as excessive bruising; bleeding; oozing around wounds.

Answer 34

Thrombocytopenia

Question 35

What are the therapist's roles for Thrombocytopenia?

Answer 35

Monitor for - Severe bruising/external hematomas - Signs of infection Educate patient - Instruct the client to watch for signs of thrombocytopenia and when noted to immediately apply ice and pressure to any external bleeding site. - Avoid aspirin and aspirin-containing compounds without a physician’s approval because of the risk of increased bleeding Safety! - Fall prevention; ADL safety, etc. Exercise prescription is highly individualized - intensity, duration, and frequency appropriate for the individual’s condition, age, and previous activity level.

Question 36

What is the process of uncontrolled activation of both coagulation and fibrinolysis? - diffuse or widespread coagulation occurring within blood vessels all over the body. Clotting and hemorrhage occur simultaneously.

Answer 36

Disseminated Intravascular Coagulation

Question 37

What is broad activation of the clotting system, using clotting factors faster than they are produced and released, and forming thrombi throughout the vasculature? - Uses up all clotting factors and platelets which causes a problem if a bleed occurs

Answer 37

Disseminated Intravascular Coagulation

Question 38

What are the therapist's roles for Disseminated Intravascular Coagulation?

Answer 38

Clients with DIC are treated by the therapist in oncology or intensive care units - DIC is either the consequence of malignancy or the result of multisystem organ failure after trauma affecting multiple systems (e.g., severe trauma or burns). Clients are in critical condition and require bedside care. Care must be taken to avoid dislodging clots and causing a new onset of bleeding. - Monitor the results of serial blood studies, particularly hematocrit, hemoglobin, and coagulation times before any intervention. - To prevent injury, bed rest during bleeding episodes is required. When monitoring vital signs, watch for hypotension and tachycardia. Regularly assess for signs and symptoms of bleeding, such as bleeding gums, bruising, petechiae, nosebleeds, reports of melena or hematuria, headaches, or changes in mental status. Assess the skin for necrosis and hematomas.

Question 39

What is hereditary? RBCs change from their usual biconcave disk shape to a crescent or sickle shape. - Chronic hemolytic anemia - Vascular occlusion

Answer 39

Sickle cell disease (SCD)

Question 40

What is the most common symptom of sickle cell disease? - Blockage from vessel occlusion Two life-threatening complications: 1. Chest syndrome 2. Stroke

Answer 40

Sickle Cell

Question 41

What are therapist's roles in sickle cell disease?

Answer 41

Recognize signs of complications - Acute chest syndrome, stroke, and neurodevelopmental impairment - Providing client education about risks and risk prevention, including the importance of physical activity and/or mobility, prevention of pulmonary complications - Any signs of weakness, abdominal pain, fatigue, dyspnea, tachycardia accompanied by pallor, and hypotension require emergency medical attention. Neurodevelopment - SCD is a blood disorder; however, the CNS is frequently affected by the disease. - Approximately one-fourth to one-third of children with SCD have some form of CNS effects from the disease, which typically manifest as deficits in specific cognitive domains and academic difficulties. Exercise - Multiple factors contribute to exercise intolerance in individuals with sickle cell anemia, but little information exists regarding the safety of maximal cardiopulmonary exercise testing or the mechanisms of exercise limitation in these clients. - Low peak VO2, low anaerobic threshold, gas exchange abnormalities, and high ventilatory reserve - During acute, avoidance of overexertion, dehydration, smoking, and exposure to cold or the use of cryotherapy for painful, swollen joints. Pain Management - Joint effusions, swelling, and septic arthritis - Physical and emotional distress - Frequent hospitalizations and consequent job absences often result in stressful financial constraints. - The combined use of medications, psychologic support, relaxation techniques, biofeedback, and imagery is a useful intervention to lessen the effects of painful episodes

Question 42

What is a group of inherited disorders of abnormalities in hemoglobin synthesis? Often linked with anemia. It manifests defective synthesis of Hb, Structurally impaired RBCs, Hemolysis or destruction of the erythrocytes.

Answer 42

Thalassemia

Question 43

What manifests as anemia?

Answer 43

Thalassemia

Question 44

What are the therapist's roles in Thalassemia?

Answer 44

Same considerations as anemia. Intervertebral disc degeneration from iron overload. - Back and lower extremity pain, paresthesia, abnormal proprioception, bowel/bladder incontinence - ADL compensations. Adaptive equipment/techniques - Gait compensations. Balance training. Assistive device? Spinal asymmetry and scoliosis are also common