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  1. 1. POLYCYTHEMIA Introduction: Polycythemia is an increased number of red blood cells in the blood. In polycythemia, the levels of hemoglobin (Hb), hematocrit (Hct- is the ratio of the volume of red cells to the volume of whole blood. ), or the red blood cell (RBC) count may be elevated when measured in the complete blood count(CBC), as compared to normal. Hemoglobin levels greater than 16.5 g/dL (grams per deciliter) in women and greater than 18.5 g/dL in men suggest polycythemia. In terms of hematocrit, a value greater than 48 in women and 52 in men is indicative of polycythemia. Meaning: “Polycythemia is characterized by an increase in absolute quantity of red cells or total RBC volume.” In contrast, relative polycythemia (pseudoerythrocytosis) is secondary to fluid loss or decreased fluid intake resulting in hemoconcentration. Normal hematopoiesis • Production of red blood cells (erythropoiesis) occurs in the bone marrow . Myeloid stem cells give rise to erythrocytes, platelets, granulocytes, eosinophils, basophils, and monocytes. The production of each lineage is a function of cell proliferation, differentiation, and apoptosis. • These various stages of differentiation rely on multiple interrelated processes. • Protein growth factors, known as cytokines, stimulate proliferation of the multilineage cells (eg, interleukin [IL]-3, granulocyte-macrophage colony-stimulating activity [GM-CSF]). • One of the important enzymes regulating this process is called erythropoietin (Epo). The majority of Epo is produced and released by the kidneys, and a smaller portion is released by the liver. Human blood cell development: All five types originates from the same cell. The process of stem cell differentiation, and development of each blood cell is as follows Yalk salk ↓ Stem cell ↓ ↓ ↓ ↓ ↓ Lymphoblast Monoblast Myeloblast Megakaryocyte Normoblast ↓ ↓ Monocyte Reticulocyte ↓ Lymphocyte Granulocyte, Thrombocytes Erythrocyte (neutrophils Eosinophil and basophil) (platelets) (RBC) B lymphocytes Plasma cells (immunoglobulin- IgG, IgM, IgA ,IgE &IgD) T lymphocyte
  2. 2. Types: Two basic categories of polycythemia are recognized: • Primary polycythemias  Polycythemia can result from internal problems with the production of red blood cells. This is termed primary polycythemia.  It isdue to factors intrinsic to red cell precursors, including primary familial and congenital polycythemia (PFCP), idiopathic erythrocytosis, and polycythemia vera (PV). • Secondary polycythemias  If polycythemia is caused due to another underlying medical problem, it is referred to as secondary polycythemia.  These are caused by factors extrinsic to red cell precursors and include a physiologic- approproriate erythropoietin (epo) production in response to tissue hypoxia and physiologic- inappropriate erythropoietin production not in response to tissue hypoxia. Most cases of polycythemia are secondary and are caused by another medical condition. Primary polycythemias are relatively rare; for example, polycythemia vera occurs in approximately 1 to 2 adults per 100,000 in the United States. One percent to five percent of newborns can have polycythemia (neonatal polycythemia). • Relative Polycythemia  Relative polycythemia describes conditions in which red cell volume is high due to increased blood concentration of red cells as a result ofdehydration. • Stress Polycythemia Etiology: ☻ Polycythemia Primary Causes  Inherent or acquired problems with red blood cell production lead to polycythemia.  Two main conditions that belong to this category are polycythemia vera (PV or polycythemia rubra vera [PRV]) and primary familial and congenital polycythemia (PFCP).  Polycythemia vera (PV) is related to a genetic mutation in the JAK2 gene, which is thought to increase the sensitivity of bone marrow cells to Epo, resulting in increased red blood cell production. Levels of other types of blood cells (white blood cells and platelets) are also often increased in this condition.  Primary familial and congenital polycythemia (PFCP) is a condition related to a mutation in the EPOR gene and causes increased production of red blood cells in response to Epo. ☻ Polycythemia Secondary Causes  Overproduction of red blood cell results from increased sensitivity or responsiveness to Epo (often with lower than normal levels of Epo), in secondary polycythemia, more red cells are produced because of high levels of circulating Epo.  The main reasons for higher than normal Epo are chronic hypoxia (poor blood oxygen levels over the long-term), poor oxygen delivery due to abnormal red blood cell structure, and tumors releasing inappropriately high amounts of Epo.  Some of the common conditions that can result in elevated erythropoietin due to chronic hypoxia or poor oxygen supply include:  chronic obstructive pulmonary disease (COPD, emphysema, chronic bronchitis),
  3. 3.  pulmonary hypertension,  hypoventilation syndrome,  congestive heart failure,  obstructive sleep apnea,  poor blood flow to the kidneys, and  living in high altitudes.  liver cancer (hepatocellular carcinoma),  kidney cancer (renal cell carcinoma),  adrenal adenoma or adenocarcinoma, and  uterine cancer.  There also are more benign conditions that may cause increase Epo secretion, such as kidney cysts and kidney obstruction.  Chronic carbon monoxide exposure can also lead to polycythemia. Hemoglobin naturally has a higher affinity for carbon monoxide than for oxygen. Therefore, when carbon monoxide molecules attach to hemoglobin, polycythemia (increased red cell and hemoglobin production) may occur in order to compensate for the poor oxygen delivery by the existing hemoglobin molecules. A similar scenario can also occur with carbon dioxide in long-term cigarette smoking.  Polycythemia in newborns (neonatal polycythemia) is often caused by transfer of maternal blood from the placenta or blood transfusions. Prolonged poor oxygen delivery to the fetus (intrauterine hypoxia) due to insufficiency of the placenta can also lead to neonatal polycythemia. ☻ Relative Polycythemia • In these situations (vomiting,diarrhea, excessive sweating) the number of red blood cells is normal, but because of the fluid loss affecting the blood (plasma), red blood cell counts may seem elevated. ☻ Stress Polycythemia • This is a condition that may be seen in hardworking, anxious, middle-aged men due to low plasma volume, although the red blood cell volume may be normal. • Other names for this condition are pseudopolycythemia, stress erythrocytosis, or Gaisbock's disease. Polycythemia Risk Factors Some of the risk factors for polycythemia include: • chronic hypoxia; • long-term cigarette smoking; • familial and genetic predisposition; • living in high altitudes; • long-term exposure to carbon monoxide (tunnel workers, car garage attendants, residents of highly polluted cities); and • Ashkenazi Jewish ancestry (may have increased frequency of polycythemia vera due to genetic susceptibility). Polycythemia clinical features: Symptoms of polycythemia can vary widely. • In some people with polycythemia, there may be no symptoms at all. • In secondary polycythemia, most of the symptoms are related to the underlying condition responsible for polycythemia. • Symptoms of polycythemia vera can be vague and quite general. Some of the important symptoms include:
  4. 4. • easy bruising; • easy bleeding; • blood clot formation (potentially leading to heart attacks, strokes, blood clots in the lungs [pulmonary embolism]); • bone and joint pain (hip pain or rib pain); • headache; • itching; • itching after taking a shower or bath (post-bath pruritus); • fatigue; • dizziness; and • abdominal pain. Diagnostic measures: • In the majority of instances, polycythemia may be detected incidentally in routine blood work ordered by a physician for an unrelated medical reason • A comprehensive medical history, physical examination, family history, and social and occupational history are very important. o In the physical exam, special attention may be paid to the heart and lung exam. o An enlarged spleen(splenomegaly) is one of the prominent features of polycythemia vera; therefore, a careful abdominal exam to evaluate for an enlarged spleen is important. • Compete blood count (CBC), clotting profile, and metabolic panel are basic components of laboratory tests in assessing the cause of polycythemia. • Other typical tests include chest X-rays,electrocardiogram (ECG), echocardiogram, hemoglobin analysis, and carbon monoxide measurement. • In polycythemia vera, usually other blood cells are also affected, represented by an abnormally high number of white blood cells (leukocytosis) and platelets (thrombocytosis). • Bone marrow examinations (bone marrow aspiration or biopsy) are sometimes necessary to examine blood cell production in the bone marrow. • Guidelines also recommend checking for the JAK2 gene mutation as a diagnostic criterion for polycythemia vera. • In primary polycythemia, the Epo level is typically low, whereas in Epo-secreting tumors, the level may be higher than normal. The results need to interpreted carefully as the Epo level may be appropriately high in response to chronic hypoxia, if that is the underlying cause of polycythemia. Medical Management Treatment of secondary polycythemia depends on its cause. • Supplemental oxygen can be provided for individuals with chronic hypoxia. • Other therapies can be directed toward treating the cause of polycythemia (for example, appropriate treatment of heart failure or chronic lung disease). Treatments for primary polycythemia • It play an important role in improving the outcome of the disease and will be discussed in the following sections. Self-Care at Home: In individuals with primary polycythemia, some simple measures can be taken at home to control potential symptoms and avoid possible complications.
  5. 5. • It is important to stay well hydrated in order to avoid concentrating the blood even further by dehydration. • In general, there are no restrictions in physical activity. • If a person has an enlarged spleen, contact sports may be avoided to prevent splenic injury and rupture. • It is best to avoid iron supplementation as this can promote more red blood cell production. Medical Treatment • The mainstay of therapy for polycythemia vera remains phlebotomy (blood letting). o The goal of phlebotomy is to keep the hematocrit around 45% in men and 42% in women. o Initially, it may be necessary to do phlebotomy every 2 to 3 days and remove 250 to 500 milliliters of blood each session. o Once the goal is reached, maintenance phlebotomy can be performed less frequently. • A commonly recommended medication for the treatment of polycythemia is called hydroxyurea (Hydrea). o This is especially advised in people at risk of clot formation. o Hydroxyurea is also recommended in patients who are unable to tolerate phlebotomy. • Aspirin has also been used in treating polycythemia to lower the risk of clotting (thrombotic) events. o Its use is generally avoided in those people with any bleeding history. o Aspirin is usually used in conjunction with phlebotomy. Follow-up • Frequent monitoring is recommended during early treatment with phlebotomy until an acceptable hematocrit is adequately maintained. • Thereafter, blood letting can be done as needed to maintain appropriate hematocrit based on each individual's response to this therapy. Complication • People with primary polycythemia need to be aware of some of the potentially serious complications that may occur. • The formation of blood clots (heart attacks, strokes, blood clots in the lungs [pulmonary embolism] or legs [deep vein thrombosis]) and uncontrolled hemorrhaging (nose bleeds, gastrointestinal bleeding) typically require prompt medical attention by the treating physician or emergency department. • Transformation to blood cancers (for example, leukemia, myelofibrosis). Prevention Many causes of secondary polycythemia are not preventable. However, some potential preventive measures are: • smoking cessation; • avoid prolonged carbon monoxide exposure; and • appropriate management of diseases such as chronic lung disease, heart disease or obstructive sleep apnea. Primary polycythemia due to mutation of genes is generally not preventable.
  6. 6. THROMBOCYTOPENIA Introduction: Bleeding and Clotting Disorders can be divided as Disseminated Intravascular Coagulation (DIC) ,Hemophilia, Henoch-Schönlein Purpura , Hereditary Hemorrhagic Telangiectasia, Thrombocytopenia (ITP, TTP), Thrombophilia, Von Willebrand's Disease. The blood usually contains about 140,000 to 440,000 platelets per microliter. Bleeding can occur with relatively minor trauma when the platelet count falls below about 50,000 platelets per microliter of blood. The most serious risk of bleeding, however, generally does not occur until the platelet count falls below 10,000 to 20,000 platelets per microliter. At these very low levels, bleeding may occur without any injury. Causes of Thrombocytopenia Bone marrow does not produce enough platelets • Leukemia • Lymphoma • Aplastic anemia • Heavy alcohol consumption • Megaloblastic anemias, including vitamin B12 and folic acid deficiency anemias • Some bone marrow disorders Platelets become entrapped in an enlarged spleen • Cirrhosis with congestive splenomegaly • Myelofibrosis • Gaucher's disease Platelets become diluted • Massive blood replacement or exchange transfusion with stored blood containing too few platelets • Cardiopulmonary bypass surgery Use or destruction of platelets increases • Idiopathic thrombocytopenic purpura • HIV infection • Drugs such as heparin, quinidine, quinine, sulfa-containing antibiotics, some oral drugs for diabetes, gold salts, and rifampin • Conditions involving disseminated intravascular coagulation within blood vessels, such as can occur with obstetric complications, cancer, blood poisoning (septicemia) from gram-negative bacteria, and traumatic brain damage • Thrombotic thrombocytopenic purpura • Hemolytic-uremic syndrome • Paroxysmal nocturnal hemoglobinuria Types of thrombocytopenia: Idiopathic thrombocytopenic purpura (ITP): • ITP is a disease in which antibodies form and destroy the body's platelets. • Why the antibodies form is not known. • Although the bone marrow increases platelet production to compensate for the destruction, the supply cannot keep up with the demand. Thrombotic thrombocytopenic purpura (TTP):
  7. 7. • TTP is a rare disease in which small blood clots form suddenly throughout the body. • The blood clots mean that an abnormally high number of platelets are being used, which leads to a sharp decrease in the number of platelets in the bloodstream. Hemolytic-uremic syndrome (HUS): • HUS is a disorder related to TTP in which the number of platelets suddenly decreases, red blood cells are destroyed, and the kidneys stop functioning. • HUS is rare but can occur with certain bacterial infections (particularly intestinal infections withEscherichia coli O157:H7 or some strains ofShigella dysenteriae) and with the use of some drugs (including quinine ,cyclosporine , and mitomycin C). • The syndrome is most common in infants, young children, and women who are pregnant or have just given birth, although it can occur in older children, adults, and women who are not pregnant.
  8. 8. Symptoms and Complications • Bleeding in the skin may be the first sign of a low platelet count. o Many tiny red dots (petechiae) often appear in the skin on the lower legs, and minor injuries may cause small scattered bruises. o The gums may bleed, and blood may appear in the stool or urine. Menstrual periods may be unusually heavy. o Bleeding may be hard to stop. • Bleeding worsens as the number of platelets decreases.  People who have very few platelets may lose large amounts of blood into the digestive tract or may develop life-threatening bleeding in the brain even though they have not been injured.
  9. 9. • The rate at which symptoms develop can vary depending on the cause of thrombocytopenia.  For example, in TTP and HUS, symptoms develop suddenly. In ITP, symptoms may develop suddenly or gradually and subtly. • Symptoms in TTP and HUS are quite distinct from symptoms of most other forms of thrombocytopenia. • In TTP, o The small blood clots that develop (using up platelets) cause a wide range of symptoms and complications, some of which can be life threatening. o Symptoms that result from clots in the brain may include headache, confusion, seizures, and coma. Symptoms that result from clots elsewhere in the body include abnormal heart rhythms, blood in the urine that accompanies kidney damage, and abdominal pain. • In HUS  The predominant symptoms and complications of HUS are related to blood clots that develop in the kidneys, causing damage that is usually severe and may progress to kidney failure Diagnosis • History of abnormal bruising and bleeding • Check the number of platelets routinely in people who have disorders that cause thrombocytopenia. • Sometimes they discover thrombocytopenia when blood tests are performed for other reasons in people who have no bruising or bleeding. • Determining the cause of thrombocytopenia is critical to treating the condition. • Certain symptoms may help determine the cause.  For example, people usually have a fever when thrombocytopenia results from an infection.  In contrast, they usually do not have a fever when the cause is ITP, TTP, or HUS.  An enlarged spleen, which a doctor may be able to feel during a physical examination, suggests that the spleen is trapping platelets and that thrombocytopenia results from a disorder that causes the spleen to enlarge.  HUS is diagnosed when poor kidney function is identified by blood tests that show high levels of urea nitrogen and creatinine. • A sample of blood may be examined under a microscope, or the platelet count may be measured with an electronic counter to determine the severity of thrombocytopenia • Bone marrow aspiration and biopsy may be needed to provide information about platelet production. Treatment: • People who have a very low platelet count are often treated in a hospital or advised to stay in bed to avoid accidental injury. • When bleeding is severe, platelets may be transfused. • Addressing the underlying cause can often treat the thrombocytopenia. o Thrombocytopenia caused by a drug usually is corrected by discontinuing the drug. o The effects produced by antibodies that destroy platelets in ITP can be blocked temporarily with a corticosteroid (for example, prednisone) or intravenous immune globulin, allowing the number of platelets to increase. Danazol may have similar effects as prednisone.
  10. 10. • Drugs that suppress the immune system, including cyclophosphamide and sometimes azathioprine , may reduce the formation of antibodies. Most adults (but not children) with ITP eventually require surgical removal of the spleen (splenectomy) to increase the number of platelets. • People with TTP are often treated with plasma transfusions along with plasmapheresis • Complications that require long-term treatment can result from some causes of thrombocytopenia. o For example, the number of platelets usually increases as people recover from the hemolytic- uremic syndromeHUS; however, lifelong dialysis or kidney transplantation may be needed if the kidney failure persists. Conclusion: Thrombocytopenia which is a deficiency of platelets (thrombocytes) occurs when the bone marrow makes too few platelets or when too many platelets are destroyed.Bleeding in the skin and bruising occur.Blood tests are used to make the diagnosis and determine the cause.Sometimes platelet transfusions are needed. NURSING MANAGEMENT _- POLYCYTHEMIA & THROMBOCYTOPENIA Nursing Diagnoses: Probable nursing Diagnoses: 1. Activity intolerance 2. Fatigue 3. Imbalanced nutrition less than body requirements 4. Impaired gas exchange 5. Ineffective thermoregulation 6. Ineffective tissue perfusion: peripheral Possible nursing diagnoses: 1. Acute pain 2. Risk for activity intolerance 3. Risk for imbalanced body temperature 4. Risk for imbalanced fluid volume 5. Risk for impaired skin integrity 6. Risk for infection Nursing Intervention: • Monitor the respiratory status (pulmonary embolism) • Assess vital signs for signs of haemorrhage • Assess the cardiovascular status to detect arrhythmias or MI • Reduce the physical activity/ provide bed rest • Quite play • Monitor the laboratory values to determine effectiveness of the therapy • Balanced diet as small frequent meals • O2 administration • Measures to maintain body temperature • Assess stool urine and emesis for occult blood loss caused by decreased platelet level • 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
  11. 11. • Perform plasma transfusion • Avoid giving IM injection to reduce risk of haemorrhage • Avoid taking rectal temperature to reduce the risk of tissue trauma • Avoid contact sports and excessive exercise Bibliography: 1. Wong D.L etal . Essentials Of Paediatric Nursing. 6th edition. Missouri: Mosby;2001 2. Marlow D.R. Redding B. Textbook of Paediatric nursing. 1st edition.Singapore: Harwourt Brace & company; 1998 3. Parthasarathy IAP textbook of Paediatrics. 2nd edition. jaypee: NewDelhi; 2002 4. Judith A.S Straight A’s in Paediatric Nursing. 2nd edition.Wolters Kluwer : Newyork;2008 5. http://emedicine.medscape.com/article/957343-overview (Retreved on 5.7.20010) 6. http://www.google.co.in/#hl=en&source=hp&q=polycythemia+causes&aq=2&aqi=g10&aql=&oq=pol ycythemia&gs_rfai=&fp=4ebf31f893dc20e5 (Retreved on 5.7.20010) 7. http://www.medicinenet.com/thrombocytopenia_low_platelet_count/article.htm (Retreved on 5.7.20010) 8. http://www.medicinenet.com/polycythemia_high_red_blood_cell_count/page2.htm (Retreved on 5.7.20010) 9. http://www.childrenshospital.org/az/Site1466/mainpageS1466P0.html (Retreved on 5.7.20010)
  12. 12. POLYCYTHEMIA & THROMBOCYTOPENIA Submitted to : Submitted by: Ms. G . Laviga Ms. Shesly P. Jose Lecturer II Year MSc (N) NUINS NUINS Submitted on: 06.07.2010