Hematological Conditions

Hematology

• Branch of science that studies the form, structure, and function of blood and blood-forming tissues

  • Plasma

  • Formed elements

    • RBCs, WBCs, and platelet

Hematologic conditions

• Alter the oxygen-carrying capacity of the blood and the constituents, structure, consistency, and the flow of the blood

  • Hypo-coagulopathy or hyper-coagulopathy

  • Increased work of the heart and breathing

  • Impaired tissue perfusion

  • Increased risk of thrombus

What does a blood test panel or complete blood count (CBC) measure?

• RBCs

• WBCs

• Hemoglobin (Hb)

• Hematocrit

• Platelets

Hemoglobin (Hb)

Oxygen-carrying protein

Hematocrit

Amount/proportion of RBCs

How does aging affect the hematological system?

• 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

  • BZ12/Iron deficiencies

What are reasons for blood transfusions?

• Surgery

• Trauma

• Childbirth

• Liver disorders

• Hematological disorders (hemophilia, anemia)

• Chemotherapy

• Kidney failure

What stimulates erythropoiesis and reduces the need for blood transfusions?

Recombinant (artificial) human erythropoietin

Erythropoiesis

Bone marrow production of RBCs

What is the most common transfusion-related reaction?

Febrile Nonhemolytic reaction

Febrile nonhemolytic reaction

Increase in temperature during or within 4 hours after transfusion

Transfusion-Associated Circulatory Overload (TACO)

• Volume overload from a rapidly infused transfusion

What are signs and symptoms of TACO?

Shortness of breath, red face, tachycardia, hypertension, headaches, and seizures typically within 6 months of transfusion

Who is most likely to have TACO?

Older clients with comorbidities, like heart or kidney disease

Transfusion-Related Acute Lung Injury (TRALI)

Neutrophil-specific antibodies in donor plasma are directed against recipient leukocytes in the pulmonary vasculature

What is the most common cause of transfusion-related death?

Transfusion-related acute lung injury (TRALI)

What are the signs and symptoms of TRALI?

Fever, hypotension, hypoxia, and pulmonary edema

Acute/delayed hemolytic transfusion reaction

When a patient is given the wrong blood or blood is mislabeled

What are the signs and symptoms of an acute /delayed hemolytic transfusion reaction?

• Fever and flank pain

• Drop in hemoglobin, increased bilirubin level → jaudice

Allergic Reactions

Mild ones (like pruritus and urticaria) are common (1%-3% of transfusions); anaphylaxis reaction is rare

What are the signs and symptoms of an allergic reaction

Hives, rash, wheezing, mucosal edema

Septic reactions

Bacterial contamination of blood products

What are the signs and symptoms of a septic reaction?

Fever, hypotension, back/chest/abdominal pain, shortness of breath

Rehabilitation professionals’ roles for patients with blood transfusions

• Patients are usually not exercising or being treated by the physical therapist during a blood transfusions

  • Exercise/therapy is not advised during the first 30-60 min of the transfusion

  • Monitor for adverse reactions

    • Most occur during the first 15 minutes (e.g., fever, chills, urticaria, etc)

    • Signs of orthostatic hypotension

Important considerations for rehabilitation professionals’ roles for blood transfusions?

• Why is the patient receiving a blood production?

• What is the underlying medical condition and the goals of therapy for that day/week?

• What is 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?

Physiological process of erythropoiesis?

• Hypoxia detected by kidneys

• Stimulates the 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

Hereditary Hemochromatosis

• Autosomal recessive hereditary disorder characterized by excessive iron absorption by the small intestine

  • Present at birth but remains asymptomatic until development of iron overloading; onset of symptoms between ages 40-60 years old

Pathogenesis of Hemochromatosis

• Gene mutations lead to a decreased production of hepcidin, resulting in increased absorption of iron by intestinal enterocytes and release of iron by macrophages

• Excess iron is slowly deposited in cells, particularly in the liver, heart, pancreas, and other endocrine glands (e.g., pituitary)

Clinical manifestations of Hemochromatosis

Weakness, chronic fatigue, myalgias, joint paint (particularly the 2nd and 3rd MCPs), abdominal pain, hepatomegaly, elevated hemoglobin, and elevated liver enzymes

Rehabilitation professionals’ roles for Hemochromatosis

• Arthropathy occurs in 40-60% of individuals, can be the first manifestation of the disease

  • Twinges of pain on flexing small joints of the hand (especially 2nd and 4rd MCP joints)

• ROM, strength, and proper alignment to promote function, prevent falls, and prevent the loss of independence in ADLs

• Evaluating the need for compensatory techniques, assistive devices, orthotics, and splints toward these goals

Anemia

Decrease in the number of RBCS

Pathogenesis of Anemia

• In acute-onset anemia with severe loss of intravascular volume, peripheral vasoconstriction and central vasodilation occur to preserve blood flow to the vital organs

• If the anemia persists, small-vessel vasodilation -> increased blood flow to ensure better tissue oxygenation

  • Decreased systemic vascular resistance, increased cardiac output, and tachycardia, resulting in a higher rate of delivery of oxygen-bearing erythrocytes to the tissues.

Production of RBCs is controlled by the _______________.

Erythroproietin

Blood loss, decreased production of erythrocytes, and peripheral destruction of erythrocytes are etiological factors of what condition?

Anemia

Rehabilitation professionals’ roles for anemia

• Exercise

  • Discuss exercise plan

  • Pacing/training to distribute the intensity of workload overtime

  • Progress slowly for anyone with decreased exercise tolerance and monitor vital signs closely

• Precautions

  • 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

• Monitor vital signs

  • Many individuals who have anemia are asymptomatic

  • Tachycardia, fatigue, generalized weakness, loss of stamina, exertional dyspnea, low diastolic blood pressure

Leukemia

Malignant neoplasm of the blood-forming cells that repalce the normal bone marrow with a malignant clone (genetically identical cell) of lymphocytic or myelogenous cells

What are the etiological factors of leukemia?

• Associated with radiation, chemotherapy, chemical (herbicides, pesticides, cigarettes) exposure

Pathogenesis of Leukemia

Chromosomal abnormality → abnormal bone marrow and blood cell productions

Clinical manifestations of Leukemia

• 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)

Rehabilitation professionals’ roles for patient with Leukemia

• 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

What is the most common inherited bleeding disorder?

von Willebrand disease

Etiological factors and pathogenesis of von Willebrand disease

• Dysfunction of the plasma protein (VWF)

  • Mediates the initial adhesion of platelets (to other platelets and endothelial collagen) at sites of bleeding disorder

• Testing/diagnosis is difficult because VWF fluctuates due to stress/exercise/inflammation

What are the clinical manifestations of von Willebrand disease?

Easy bruising and bleeding

Rehabilitation professionals’ roles for von Willebrand disease

• Prevention/monitoring

  • Wound healing

  • Secondary infection

• Fall prevention

• Education

Hemophilia

Bleeding disorder inherited as a n x-linked autosomal recessive trait

What are etiological factors of hemophilia?

Mild, moderate, and severe decreases in clotting factors

Pathogenesis of hemophilia

~10 proteins that affect clotting

Clinical manifestations of hemophilia

• 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

Rehabilitation professionals’ roles for hemophilia

• Shift from rehabilitation to prevention

  • Protective strengthening of the musculature surrounding affected joints, muscle reeducation, gain training, and client education

• Physical activity and exercise

  • Regular exercise program

• Maintaining Joint ROM

  • Be alert to recognize signs of early (24-48 hrs) bleeding episodes

  • RICE → Rest, ice, compression, and elevation

    • Promote comfort and healing for an acute joint (hemarthrosis), and muscle bleed (intramuscular hemorrhage)

  • Joint ROM can be measured in the pain-free range, but no strength testing

  • Static/dynamic night splints

Thrombocytopenia

Decrease in platelet count

Etiological factors and pathogenesis of Thrombocytopenia

• Inadequate platelet production from the bone marrow

• Increase platelet destruction outside the bone marrow

• Splenic sequestration

• Common complication with cancer and associated treatments

Splenic Sequestration

Entrapment of blood and enlargement in the spleen

Rehabilitation professionals’ roles for Thrombocytopenia

• Monitor for:

  • Severe bruising/external hematoma

  • Signs of infections

• Educate patient

  • Instruct patient to watch for signs and to apply ice and pressure to bleeding site

  • Avoid aspirin and aspirin containing compounds without a physician’s approval due to increased risk of bleeding

• Safety (fall prevention; ADL safety)

• Exercise prescription is highly individualized

  • Intensity, duration, and frequency appropriate for individual’s condition, age, and previous activity level

Etiological factors of Disseminated Intravascular Coagulation

• Process of uncontrolled activation of both coagulation and fibrinolysis

• Diffuse or widespread coagulation occurring within blood vessels all over the body

• Associated with sepsis, cancer, massive trauma

Clotting and hemorrhage occur simultaneously for which condition?

Disseminated Intravascular Coagulation

Pathogenesis for Disseminated Intravascular Coagulation

• Broad activation of the clotting system, using clotting factors faster than they are produced and released, and forming thrombi through the vasculature

• Uses up all clotting factors and platelets which causes a problem if a bleed occurs

What are the clinical manifestations of Disseminated Intravascular Coagulation?

• Thrombosis, bleeding, or both

• Oozing from wounds or catheters

• Nose bleeds

• Serious hemorrhage

  • Delirium

  • Hypoxia

Rehabilitation professionals’ roles of Disseminated Intravascular Coagulation

• 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 several times before any intervention

  • To prevent injury, bed rest during bleeding episodes is requires

• When monitoring vital signs, watch for hypotension and tachycardia

• Regularly assess for signs/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

Etiological factors of Sickle cell disease

Hereditary; may be formed to protect against malaria

Pathogenesis of Sickle cell disease

• RBCs changes from their usual biconcave disc shape to a crescent or sickle shape

  • Chronic hemolytic anemia

  • Vascular occlusion

Clinical manifestations of Sickle cell disease

• Pain is the most common symptom

  • Blockage from vessel occlusion

• Two life-threatening complications

  • Chest syndrome

  • Stroke

Rehabilitation professionals’ roles for Sickle cell disease

• Recognize signs of complications

  • Acute chest syndrome, stroke, neurodevelopmental impairment

  • Client education about risks and risk prevention

  • Any signs of weakness, abdominal pain, fatigue, tachycardia, etc

• Neurodevelopment

  • CNS is frequently affected

    • Manifest as deficits in specific cognitive domains and academic difficulties

  • Exercise

    • Multiple factors contribute to exercise intolerance. Limited info on the safety of maximal cardiopulmonary exercise testing or the exercise limitation in clients

  • Pain management

Etiological factors of Thalassemia

• Group of inherited disorders of abnormalities in hemoglobin synthesis

  • Often linked to anemia

• Most prevalent in people from Africa, Middle East, Southeast Asia

Pathogenesis of Thalassemia

• Defective synthesis of Hb (ineffective erythropoiesis

• Structurally impaired RBCs

• Hemolysis or destruction of the erythrocytes

Clinical manifestations of Thalassemia

• Anemia and iron overload → endocrine, cardiac, and liver disease

  • Decreased bone mineral density

• Frequent and regular transfusions beginning in infancy

  • Risks associated with transfusions

Rehabilitation professionals’ roles for Thalassemia

• Same as anemia!

• Exercise

  • Discuss exercise plan

  • Pacing/training to distribute the intensity of workload over time

  • Progress slowly for anyone with decreased exercise tolerance and monitor vital signs closely

• Precautions

  • 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 anemia are asymptomatic

  • Tachycardia fatigue, generalized weakness, loss of stamina, exertional dyspnea, low diastolic blood pressure

• Intervertebral disc degeneration from overload -> ADL compensations (adaptive equipment/techniques), gait compensations (balance training)

Leukocytosis

• Increase in the # of leukocytes in the blood in response to:

  • Infection and leukemia

  • Hemorrhage (GI bleed, skull fracture, etc)

  • Normal protective response to physiologic stressors (strenuous exercise, emotional changes, temperature changes, anesthesia, surgery, etc)

Clinical manifestations of Leukocytosis?

Fever, headache, shortness of breath, symptoms of localized or systemic infeciton, and symptoms of inflammation or trauma to tissue

Leukopenia

Decrease in any or all of WBC (most common: neutrophils)

Clinical manifestations of Leukopenia

• Asymptomatic (and detected by routine tests)

• S/s consistent with infection, such as sore throat, cough, fever

Basophilia

• Increased # of production of basophils to other leukocytes in the blood or tissue

• Primarily associated with myeloproliferative disorders, particularly chronic myeloid leukemia

Clinical manifestations of Basophilia

• Allergic and chronic inflammatory reactions or infections (such as tuberculosis), as well as in autoimmune disorders and drug exposures

• Increased release of histamines, heparin, cytokines, and leukotrienes

Eosinophilia

Increase # of eosinophils in tissue or blood

Eosinophils

Prominent feature of parasitic infections, allergy, asthma, and pulmonary and vascular disorders

Clinical manifestations of Eosinophilia

Mild atopic asthma, allergic reactions to drugs (ibuprofen, aspirin, etc), parasitic reactions, malignancies, connective tissue disorders and hyper-eosinophilic syndromes

Neutrophilia

Elevation in the # of neutrophils in the blood

Clinical manifestations of Neutrophilia

Inflammation of tissue necrosis, acute infection

Neutropenia

• Reduction in circulating neutrophils

• Increased risk for infection

What causes Neutropenia?

Drugs (chemotherapy, immunosuppressive agents) or infectious agents (HIV, hepatitis)

Monocytosis

Increase in monocytes; often seen in chronic infections, such as tuberculosis, syphilis, and subacute endocarditis and other inflammatory processes

Monocyte

Circulating blood cells that give rise to macrophages and dendritic cells

Describe the genetic predisposition in acquiring hereditary forms of blood and bone marrow disorders, like Sickle cell?

Involves inheriting two copies of the mutated gene from both parents, with carriers of one being asymptomatic but able to pass the gene on to their offspring.