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Hematological Disorders
Blood Composition And disorders of
erythrocytes
Objectives
• At the end of this presentation the students will be
able to:
• Define blood and discuss its composition.
• Define anemia and identify different types of anemia.
• Discuss polycythemia.
• Enlist causes, signs, symptoms and complications of
different types of anemia & polycythemia.
• Discuss medical diagnosis and treatments for different
types of anemia.
• Make nursing diagnosis and nursing interventions for
anemia.
Blood
• Blood is a specialized bodily fluid in humans and
other animals that delivers necessary substances
such as nutrients and oxygen to the cells and
transports metabolic waste products away from
those same cells.
Composition of Blood
• Consists of blood cells (45%) suspended & carried in
plasma (55%).
• Total blood volume is about 5-6L.
• Plasma is straw-colored liquid consisting of H20 (90%) &
dissolved solutes
– Includes ions, metabolites, hormones, antibodies and
plasma proteins.
13-7
Blood Components
• Plasma:
– The liquid part of blood. All the blood cells are suspended
in this liquid.
– Contains dissolved salts (electrolytes) and proteins
• Albumin helps keeps blood vessels from leaking and carries
hormones and drugs to different parts of the body.
• Antibodies (immunoglobulins) that defend the body against
viruses, fungi, and cancer cells
– Prevents blood vessels from collapsing and clotting by
keeping them filled and circulating
– Plays role in thermoregulation the body
Blood Components
• Red Blood Cells
– Erthrocytes: Make up 40% of the blood’s volume
– Produced in the bone marrow
– Contain hemoglobin, a protein that gives blood its red color and
enables it to carry oxygen.
• White Blood Cells
– Leukocytes: Fewer in number than RBCs (1:660)
– Primary responsibility: Defend the body against infection
• Platelets
– Thrombocytes: cell-like particles smaller than RBCs and WBCs.
– Help with clotting process by gathering at bleeding site and
clumping together to form a plug that helps seal the blood vessel.
Blood cells
Granulocytes
Anemia
Definition:
A reduction in the quantity in the oxygen
carrying pigment hemoglobin in the blood
characterized by excessive tiredness, fatigue, pallor
and poor resistance to infection is called anemia.
Types Of Anemia
• Iron deficiency Anemia
• Pernicious Anemia
• Hemolytic Anemia
• Aplastic Anemia
• Folic acid deficiency Anemia
• Sickle cell Anemia.
Iron Deficiency Anemia
• When our body does not have enough iron, it will
make fewer red blood cells or red blood cells that are
too small. This is called iron deficiency anemia.
• Iron deficiency anemia is the most common form
of anemia.
Etiology
• Blood loss due to:
o Heavy, long, or frequent menstrual periods
o Cancer in the esophagus, stomach, or colon
o Esophageal varices
o The use of aspirin, ibuprofen, or arthritis medicines
for a long time
o Peptic ulcer disease
• Malabsorption of iron in the intestines due to:
o Celiac disease
o Crohn's disease
• Poor diet such as strict vegetarian.
Signs and symptoms
• Feeling weak or tired more often than usual, or with
exercise
• Headaches
• Problems in concentrating or thinking
• Brittle nails
• Pallor
• Shortness of breath.
• Sore tongue.
• Dizziness.
• Coldness in extremities.
Diagnosis
• Physical Exam.
• Complete Blood Count.
• Reticulocyte count.
• Serum iron.
Treatment
• Dietary Changes and Iron Supplements
• Vitamin C supplements.
• Treatment To Stop Bleeding
• Blood Transfusion for Severe Iron-Deficiency Anemia.
Complications
• Severe iron deficiency anemia can cause low oxygen
levels in vital organs such as the heart, kidneys and
can lead to a heart attack and kidney failure.
Pernicious Anemia
• Pernicious anemia is a type of vitamin B12 anemia.
• The body needs vitamin B12 to make red blood cells.
this vitamin is got from eating foods such as meat,
poultry, shellfish, eggs, and dairy products.
• A special protein, called intrinsic factor, helps our
intestines to absorb vitamin B12.
• This protein is released by cells in the stomach.
When the stomach does not make enough intrinsic
factor, the intestine cannot properly absorb vitamin
B12.
Etiology
Common causes of pernicious anemia include:
• Weakened stomach lining gastritis)
• An autoimmune gastritis in which the body's
immune system attacks intrinsic factor protein or the
cells that make it.
• It may be congenital ,hence the affected babies
cannot absorb vit-b12 properly.
Signs and Symptoms
o Fatigue
o Loss of appetite
o Pale skin
o Problems in concentrating
o Shortness of breath, mostly during exercise
o Swollen, red tongue or bleeding gums
Diagnosis
• Physical exam.
• Bone marrow examination.
• CBC
• Hgb level
• Reticulocyte count
• Schilling test
• Vitamin B12 level
Hemolytic anemia
• Hemolytic anemia is a condition in which there are
not enough red blood cells in the blood, due to the
premature (Before 120 days) destruction of red
blood cells.
Etiology
• Hereditary.
• Immune disorders
• Infections
• Reactions to medicines or blood transfusions
• Hypersplenism
Signs and Symptoms
• Chills
• Dark urine
• Enlarged spleen
• Fatigue
• Fever
• Pale skin color (pallor)
• Rapid heart rate
• Shortness of breath
• Yellow skin color (jaundice)
Diagnosis
• Reticulocyte count.
• Red blood cell count (RBC).
• Hemoglobin level , and hematocrit (HCT).
• Serum LDH(105-333 IU/L).
Treatment
• Blood Transfusions
• Corticosteroid medicines, such as prednisone, can
stop immune system from, or limit its ability to,
make antibodies (proteins) against red blood cells.
• Plasmapheresis.
• Surgery: Some people who have hemolytic anemia
may need surgery to remove their spleens.
• Blood and Marrow Stem Cell Transplant.
Complications
• Severe hemolytic anemia can cause cardiovascular
collapse (failure of the heart and blood pressure,
leading to death).
• Severe anemia can worsen heart disease, lung
disease, or cerebrovascular disease.
Folic acid deficiency anemia
• Folic acid deficiency anemia happens when your
body does not get enough folic acid. Folic acid is one
of the B vitamins, and it helps your body make new
cells, including new red blood cells
Etiology
• Poor folic acid diet.
• Being pregnant or have some medical problems,
such as sickle cell disease.
• Malabsorption of folic acid in the gut.
• Certain medicines, such as some used
for cancer, rheumatoid arthritis, and seizures.
Signs and Symptoms
• Feel weak and tird
• Be forgetful.
• Feel grouchy.
• Lose of appetite and weight lose.
• Problems in concentrating.
Diagnosis
• Physical examination.
• Complete blood count.
• Hgb level.
• Folic acid level.
• Vitamin B12 level.
Treatment
• To treat the anemia, take folic acid pills each day to
bring folic acid level back up.
• After folic acid level is normal, eat foods rich in folic
acid so we don't get anemia again. These foods
include fortified breads and cereals, citrus fruits, and
dark green, leafy vegetables.
Aplastic Anemia
• Aplastic anemia is a blood disorder in which the
body's bone marrow doesn't make enough new
blood cells. Bone marrow is a sponge-like tissue
inside the bones. It makes stem cells that develop
into red blood cells, white blood cells, and platelets.
Etiology
• Many diseases, conditions, and factors can cause
aplastic anemia, including:
• Toxins, such as pesticides, arsenic, and benzene.
• Radiation and chemotherapy (treatments for cancer).
• Medicines, such as chloramphenicol.
• Autoimmune disorders, such as lupus and
rheumatoid arthritis.
• Hereditary.
Signs and Symptoms
• Fatigue (tiredness)
• Shortness of breath
• Swelling or pain in the abdomen or swelling in the
legs caused by blood clots
• Blood in the urine
• Headaches
• Irregular heartbeat
• Heart murmur
Diagnosis
• Physical Exam
• Complete Blood Count
• Reticulocyte Count
• Bone Marrow Tests
• Biopsy of bone marrow
Treatment
• Blood Transfusions
• Blood and Marrow Stem Cell Transplants
• Medicines to:
o Stimulate bone marrow (erythropoietin)
o Suppress your immune system (antithymocyte
globulin (ATG), cyclosporine, and
methylprednisolone)
o Prevent and treat infections (antibiotic and antiviral
medicines)
Sickle Cell Anemia
• Sickle cell anemia is a serious disorder in which the
body makes sickle-shaped red blood cells. “Sickle-
shaped” means that the red blood cells are shaped
like a crescent.
• Sickle cells contain abnormal hemoglobin called
sickle hemoglobin. Sickle hemoglobin causes the
cells to develop a sickle, or crescent, shape.
• This occur because amino acids glutamine is in the
beta chain of hgb is replaced by amino acid valine.
Etiology
• Sickle cell anemia is an inherited disease. People who
have the disease inherit two genes for sickle
hemoglobin—one from each parent.
Signs and Symptoms
• Shortness of breath
• Dizziness
• Headaches
• Coldness in the hands and feet
• Paler than normal skin or mucous membranes
• Fatigue
• Pain throughout the body.
Diagnosis
• Complete blood count (CBC)
• Sickle cell test
• Other tests may include:
o Blood oxygen
o CT scan or MRI
o Peripheral smear
o Serum hemoglobin.
Treatment
• The goal of treatment is to manage and control
symptoms, and to limit the number of crises.
• Folic acid supplements should be taken. Folic acid is
needed to make red blood cells.
• Treatment for a sickle cell anemia includes:
• Blood transfusions (may also be given regularly to
prevent stroke)
• Pain medicines
• Plenty of fluids
Cont….
• Other treatments for sickle cell anemia may include:
• Hydroxyurea (Hydrea), a medicine that may help
reduce the number of pain episodes (including chest
pain and difficulty breathing) in some people
• Antibiotics to prevent bacterial infections, which are
common in children with sickle cell disease
Nursing diagnosis
• Activity intolerance related to weakness, fatigue, and
shortness of breath.
• Imbalanced nutrition less than body requirements
related to anorexia.
• Risk for impaired skin integrity related to
decreased mobility, bed rest and tissue hypoxia.
• High risk of infection related to an inadequate
secondary defenses or a decrease in granulocytes
(inflammatory response depressed)).
• Constipation or diarrhea related to decreased dietary
inputs secondary to changes in the digestive process.
Nursing interventions
• Monitor vital signs, color of skin, mucous
membranes.
• Administer medication such as vitamins and mineral
supplements.
• Suggest the patient for balanced diet, avoiding hard
and spicy foods.
• Provide oxygen as needed.
• Assess the capability of the patient to
ingest nutrients or any other barriers such
as being lactose intolerant or has a pancreatic
disease.
Cont….
• Encourage intake of folic acid supplements.
• Assist the patient in the activities of daily living.
• Note changes in balance/ gait disturbance, muscle
weakness to indicate neurological changes
associated with vitamin B12 deficiency, affecting
patient safety or risk of injury.
• Elevate the head of the bed to enhances lung
expansion to maximize oxygenation for cellular
uptake.
• Explain necessity for bone marrow aspiration and
biopsy for definitive diagnosis.
Polycythemia Vera
Polycythemia Vera
Polycythemia Vera or P.V. is a disease where the
bone marrow creates too much red blood cells.
Polycythaemia means “many cells in the
blood”.
It is a rare disease that occurs once in every
200,000 people.
Etiology
– Chronic pulmonary or cardiac disease
– High oxygen affinity hemoglobinopathy
– Residence at high altitude
Conti…
– Tumors producing erythropoietin or anabolic
steroids
– Disorders associated with decreased plasma
volume (e.g., diarrhea, emesis, renal diseases).
Pathophysiology
Polycythaemia Vera is a bone marrow disorder
characterized by erythrocytosis, usually with a
simultaneous leukolytosis and thrombocytosis,
hypervolemia, increased blood viscosity from the
increased RBC mass and platelet dysfunction occur.
Symptoms usually are absent in early stage,
circulatory manifestations of polycythaemia Vera
occur b/c of the hypertention caused by
hypervolaemia and hyperviscosity.
They are often the first symptoms and include the
following:
Headache
Vertigo
Dizziness
Tinnitus and visual disturbances.
Signs and symptoms
Conti…
Angina
CHF
Diagnostic tests
Increased hemoglobin & hematocrit
Normoblasts may be present
Mild to moderate leukocytosis
Mild neutrophilia and/or basophilia
Thrombocytosis
Treatment
Phlebotomy (the surgical opening or puncture of a
vein in order to remove blood or infuse fluids or
drugs.
Alkalating agents (busulfan, hydroxyurea,
melphalan).
Radioactive phosphorus or interferon to inhibit bone
marrow activity.
Nursing diagnosis
i. Acute pain related to headache.
ii. Deficient knowledge (disease and treatment)
related to lack of exposure to information.
iii. Disturbed sensory perception (visual) related to
hypervolemia.
iv. Impaired gas exchange related to dyspnea.
Nursing interventions
 Monitor the respiratory status (pulmonary
embolism)
 Assess vital signs for signs of hemorrhage
 Assess the cardiovascular status to detect
arrhythmias or MI
 Reduce the physical activity/ provide bed rest
 Monitor the laboratory values to determine
effectiveness of the therapy.
 Balanced diet as small frequent meals.
Conti…
 Measures to maintain body temperature.
 Monitor for infection, bleeding , and bruising caused
by decreased platelet level.
 Encourage fluids and administer IV fluids to replace
fluid lost by fever and bleeding.
 Avoid aspirin and related drugs.
References
• Lee MT, Piomelli S, Granger S, et al. Stroke
prevention trial in sickle cell anemia (STOP):
extended follow-up and final results. Blood.
2006;108:847-852.
• Brawley OW, Cornelius LJ, Edwards LR, Gamble VN,
Green BL, Inturrisi C, et al. National Institutes of
Health consensus development conference
statement: hydroxyurea treatment for sickle cell
disease.Ann Intern Med. 2008;148:932-938.
• Geller AK, O'Connor MK. The sickle cell crisis: a
dilemma in pain relief. Mayo Clin Proc. 2008;83:320-
323.

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1 Hematological Disorders-I.pptx

  • 1. Hematological Disorders Blood Composition And disorders of erythrocytes
  • 2. Objectives • At the end of this presentation the students will be able to: • Define blood and discuss its composition. • Define anemia and identify different types of anemia. • Discuss polycythemia. • Enlist causes, signs, symptoms and complications of different types of anemia & polycythemia. • Discuss medical diagnosis and treatments for different types of anemia. • Make nursing diagnosis and nursing interventions for anemia.
  • 3. Blood • Blood is a specialized bodily fluid in humans and other animals that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from those same cells.
  • 4. Composition of Blood • Consists of blood cells (45%) suspended & carried in plasma (55%). • Total blood volume is about 5-6L. • Plasma is straw-colored liquid consisting of H20 (90%) & dissolved solutes – Includes ions, metabolites, hormones, antibodies and plasma proteins. 13-7
  • 5. Blood Components • Plasma: – The liquid part of blood. All the blood cells are suspended in this liquid. – Contains dissolved salts (electrolytes) and proteins • Albumin helps keeps blood vessels from leaking and carries hormones and drugs to different parts of the body. • Antibodies (immunoglobulins) that defend the body against viruses, fungi, and cancer cells – Prevents blood vessels from collapsing and clotting by keeping them filled and circulating – Plays role in thermoregulation the body
  • 6. Blood Components • Red Blood Cells – Erthrocytes: Make up 40% of the blood’s volume – Produced in the bone marrow – Contain hemoglobin, a protein that gives blood its red color and enables it to carry oxygen. • White Blood Cells – Leukocytes: Fewer in number than RBCs (1:660) – Primary responsibility: Defend the body against infection • Platelets – Thrombocytes: cell-like particles smaller than RBCs and WBCs. – Help with clotting process by gathering at bleeding site and clumping together to form a plug that helps seal the blood vessel.
  • 8. Anemia Definition: A reduction in the quantity in the oxygen carrying pigment hemoglobin in the blood characterized by excessive tiredness, fatigue, pallor and poor resistance to infection is called anemia.
  • 9. Types Of Anemia • Iron deficiency Anemia • Pernicious Anemia • Hemolytic Anemia • Aplastic Anemia • Folic acid deficiency Anemia • Sickle cell Anemia.
  • 10. Iron Deficiency Anemia • When our body does not have enough iron, it will make fewer red blood cells or red blood cells that are too small. This is called iron deficiency anemia. • Iron deficiency anemia is the most common form of anemia.
  • 11. Etiology • Blood loss due to: o Heavy, long, or frequent menstrual periods o Cancer in the esophagus, stomach, or colon o Esophageal varices o The use of aspirin, ibuprofen, or arthritis medicines for a long time o Peptic ulcer disease • Malabsorption of iron in the intestines due to: o Celiac disease o Crohn's disease • Poor diet such as strict vegetarian.
  • 12. Signs and symptoms • Feeling weak or tired more often than usual, or with exercise • Headaches • Problems in concentrating or thinking • Brittle nails • Pallor • Shortness of breath. • Sore tongue. • Dizziness. • Coldness in extremities.
  • 13. Diagnosis • Physical Exam. • Complete Blood Count. • Reticulocyte count. • Serum iron.
  • 14. Treatment • Dietary Changes and Iron Supplements • Vitamin C supplements. • Treatment To Stop Bleeding • Blood Transfusion for Severe Iron-Deficiency Anemia.
  • 15. Complications • Severe iron deficiency anemia can cause low oxygen levels in vital organs such as the heart, kidneys and can lead to a heart attack and kidney failure.
  • 16. Pernicious Anemia • Pernicious anemia is a type of vitamin B12 anemia. • The body needs vitamin B12 to make red blood cells. this vitamin is got from eating foods such as meat, poultry, shellfish, eggs, and dairy products. • A special protein, called intrinsic factor, helps our intestines to absorb vitamin B12. • This protein is released by cells in the stomach. When the stomach does not make enough intrinsic factor, the intestine cannot properly absorb vitamin B12.
  • 17. Etiology Common causes of pernicious anemia include: • Weakened stomach lining gastritis) • An autoimmune gastritis in which the body's immune system attacks intrinsic factor protein or the cells that make it. • It may be congenital ,hence the affected babies cannot absorb vit-b12 properly.
  • 18. Signs and Symptoms o Fatigue o Loss of appetite o Pale skin o Problems in concentrating o Shortness of breath, mostly during exercise o Swollen, red tongue or bleeding gums
  • 19. Diagnosis • Physical exam. • Bone marrow examination. • CBC • Hgb level • Reticulocyte count • Schilling test • Vitamin B12 level
  • 20. Hemolytic anemia • Hemolytic anemia is a condition in which there are not enough red blood cells in the blood, due to the premature (Before 120 days) destruction of red blood cells.
  • 21. Etiology • Hereditary. • Immune disorders • Infections • Reactions to medicines or blood transfusions • Hypersplenism
  • 22. Signs and Symptoms • Chills • Dark urine • Enlarged spleen • Fatigue • Fever • Pale skin color (pallor) • Rapid heart rate • Shortness of breath • Yellow skin color (jaundice)
  • 23. Diagnosis • Reticulocyte count. • Red blood cell count (RBC). • Hemoglobin level , and hematocrit (HCT). • Serum LDH(105-333 IU/L).
  • 24. Treatment • Blood Transfusions • Corticosteroid medicines, such as prednisone, can stop immune system from, or limit its ability to, make antibodies (proteins) against red blood cells. • Plasmapheresis. • Surgery: Some people who have hemolytic anemia may need surgery to remove their spleens. • Blood and Marrow Stem Cell Transplant.
  • 25. Complications • Severe hemolytic anemia can cause cardiovascular collapse (failure of the heart and blood pressure, leading to death). • Severe anemia can worsen heart disease, lung disease, or cerebrovascular disease.
  • 26. Folic acid deficiency anemia • Folic acid deficiency anemia happens when your body does not get enough folic acid. Folic acid is one of the B vitamins, and it helps your body make new cells, including new red blood cells
  • 27. Etiology • Poor folic acid diet. • Being pregnant or have some medical problems, such as sickle cell disease. • Malabsorption of folic acid in the gut. • Certain medicines, such as some used for cancer, rheumatoid arthritis, and seizures.
  • 28. Signs and Symptoms • Feel weak and tird • Be forgetful. • Feel grouchy. • Lose of appetite and weight lose. • Problems in concentrating.
  • 29. Diagnosis • Physical examination. • Complete blood count. • Hgb level. • Folic acid level. • Vitamin B12 level.
  • 30. Treatment • To treat the anemia, take folic acid pills each day to bring folic acid level back up. • After folic acid level is normal, eat foods rich in folic acid so we don't get anemia again. These foods include fortified breads and cereals, citrus fruits, and dark green, leafy vegetables.
  • 31. Aplastic Anemia • Aplastic anemia is a blood disorder in which the body's bone marrow doesn't make enough new blood cells. Bone marrow is a sponge-like tissue inside the bones. It makes stem cells that develop into red blood cells, white blood cells, and platelets.
  • 32. Etiology • Many diseases, conditions, and factors can cause aplastic anemia, including: • Toxins, such as pesticides, arsenic, and benzene. • Radiation and chemotherapy (treatments for cancer). • Medicines, such as chloramphenicol. • Autoimmune disorders, such as lupus and rheumatoid arthritis. • Hereditary.
  • 33. Signs and Symptoms • Fatigue (tiredness) • Shortness of breath • Swelling or pain in the abdomen or swelling in the legs caused by blood clots • Blood in the urine • Headaches • Irregular heartbeat • Heart murmur
  • 34. Diagnosis • Physical Exam • Complete Blood Count • Reticulocyte Count • Bone Marrow Tests • Biopsy of bone marrow
  • 35. Treatment • Blood Transfusions • Blood and Marrow Stem Cell Transplants • Medicines to: o Stimulate bone marrow (erythropoietin) o Suppress your immune system (antithymocyte globulin (ATG), cyclosporine, and methylprednisolone) o Prevent and treat infections (antibiotic and antiviral medicines)
  • 36. Sickle Cell Anemia • Sickle cell anemia is a serious disorder in which the body makes sickle-shaped red blood cells. “Sickle- shaped” means that the red blood cells are shaped like a crescent. • Sickle cells contain abnormal hemoglobin called sickle hemoglobin. Sickle hemoglobin causes the cells to develop a sickle, or crescent, shape. • This occur because amino acids glutamine is in the beta chain of hgb is replaced by amino acid valine.
  • 37.
  • 38. Etiology • Sickle cell anemia is an inherited disease. People who have the disease inherit two genes for sickle hemoglobin—one from each parent.
  • 39. Signs and Symptoms • Shortness of breath • Dizziness • Headaches • Coldness in the hands and feet • Paler than normal skin or mucous membranes • Fatigue • Pain throughout the body.
  • 40. Diagnosis • Complete blood count (CBC) • Sickle cell test • Other tests may include: o Blood oxygen o CT scan or MRI o Peripheral smear o Serum hemoglobin.
  • 41. Treatment • The goal of treatment is to manage and control symptoms, and to limit the number of crises. • Folic acid supplements should be taken. Folic acid is needed to make red blood cells. • Treatment for a sickle cell anemia includes: • Blood transfusions (may also be given regularly to prevent stroke) • Pain medicines • Plenty of fluids
  • 42. Cont…. • Other treatments for sickle cell anemia may include: • Hydroxyurea (Hydrea), a medicine that may help reduce the number of pain episodes (including chest pain and difficulty breathing) in some people • Antibiotics to prevent bacterial infections, which are common in children with sickle cell disease
  • 43. Nursing diagnosis • Activity intolerance related to weakness, fatigue, and shortness of breath. • Imbalanced nutrition less than body requirements related to anorexia. • Risk for impaired skin integrity related to decreased mobility, bed rest and tissue hypoxia. • High risk of infection related to an inadequate secondary defenses or a decrease in granulocytes (inflammatory response depressed)). • Constipation or diarrhea related to decreased dietary inputs secondary to changes in the digestive process.
  • 44. Nursing interventions • Monitor vital signs, color of skin, mucous membranes. • Administer medication such as vitamins and mineral supplements. • Suggest the patient for balanced diet, avoiding hard and spicy foods. • Provide oxygen as needed. • Assess the capability of the patient to ingest nutrients or any other barriers such as being lactose intolerant or has a pancreatic disease.
  • 45. Cont…. • Encourage intake of folic acid supplements. • Assist the patient in the activities of daily living. • Note changes in balance/ gait disturbance, muscle weakness to indicate neurological changes associated with vitamin B12 deficiency, affecting patient safety or risk of injury. • Elevate the head of the bed to enhances lung expansion to maximize oxygenation for cellular uptake. • Explain necessity for bone marrow aspiration and biopsy for definitive diagnosis.
  • 47. Polycythemia Vera Polycythemia Vera or P.V. is a disease where the bone marrow creates too much red blood cells. Polycythaemia means “many cells in the blood”. It is a rare disease that occurs once in every 200,000 people.
  • 48. Etiology – Chronic pulmonary or cardiac disease – High oxygen affinity hemoglobinopathy – Residence at high altitude
  • 49. Conti… – Tumors producing erythropoietin or anabolic steroids – Disorders associated with decreased plasma volume (e.g., diarrhea, emesis, renal diseases).
  • 50. Pathophysiology Polycythaemia Vera is a bone marrow disorder characterized by erythrocytosis, usually with a simultaneous leukolytosis and thrombocytosis, hypervolemia, increased blood viscosity from the increased RBC mass and platelet dysfunction occur.
  • 51. Symptoms usually are absent in early stage, circulatory manifestations of polycythaemia Vera occur b/c of the hypertention caused by hypervolaemia and hyperviscosity. They are often the first symptoms and include the following: Headache Vertigo Dizziness Tinnitus and visual disturbances. Signs and symptoms
  • 53. Diagnostic tests Increased hemoglobin & hematocrit Normoblasts may be present Mild to moderate leukocytosis Mild neutrophilia and/or basophilia Thrombocytosis
  • 54. Treatment Phlebotomy (the surgical opening or puncture of a vein in order to remove blood or infuse fluids or drugs. Alkalating agents (busulfan, hydroxyurea, melphalan). Radioactive phosphorus or interferon to inhibit bone marrow activity.
  • 55. Nursing diagnosis i. Acute pain related to headache. ii. Deficient knowledge (disease and treatment) related to lack of exposure to information. iii. Disturbed sensory perception (visual) related to hypervolemia. iv. Impaired gas exchange related to dyspnea.
  • 56. Nursing interventions  Monitor the respiratory status (pulmonary embolism)  Assess vital signs for signs of hemorrhage  Assess the cardiovascular status to detect arrhythmias or MI  Reduce the physical activity/ provide bed rest  Monitor the laboratory values to determine effectiveness of the therapy.  Balanced diet as small frequent meals.
  • 57. Conti…  Measures to maintain body temperature.  Monitor for infection, bleeding , and bruising caused by decreased platelet level.  Encourage fluids and administer IV fluids to replace fluid lost by fever and bleeding.  Avoid aspirin and related drugs.
  • 58. References • Lee MT, Piomelli S, Granger S, et al. Stroke prevention trial in sickle cell anemia (STOP): extended follow-up and final results. Blood. 2006;108:847-852. • Brawley OW, Cornelius LJ, Edwards LR, Gamble VN, Green BL, Inturrisi C, et al. National Institutes of Health consensus development conference statement: hydroxyurea treatment for sickle cell disease.Ann Intern Med. 2008;148:932-938. • Geller AK, O'Connor MK. The sickle cell crisis: a dilemma in pain relief. Mayo Clin Proc. 2008;83:320- 323.