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1 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Human Diseases A Systemic Approach Sixth Edition Chapter 8 Diseases of the Blood Mary Lou Mulvihill Mark Zelman Paul Holdaway Elaine Tompary Jill Raymond

2 Chapter 8 Diseases of the Blood Slide 24Sickle Cells Slide 24Sickle Cells Slide 25Sickle Cell Anemia Slide 25Sickle Cell Anemia Slide 53Leukemia Slide 53Leukemia Slide 62Initiating Blood Therapy Slide 62Initiating Blood Therapy Slide 63Phlebotomy and Venipuncture Slide 63Phlebotomy and Venipuncture Slide 64Bloodtest Basics Slide 64Bloodtest Basics Slide 65AIDS Slide 65AIDS Slide 66AIDS (Continued) Slide 66AIDS (Continued) Multimedia Asset Directory Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved.

3 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Blood  Overview  Components of Blood – Blood cells ErythrocytesPlateletsLeukocytes – Plasma

4 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Blood  Average adult has about 5 liters of blood  Circulates through body within vessels of cardiovascular system  A mixture of plasma with cells – Formed elements – Erythrocytes – Leukocytes – Platelets

5 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Plasma  Composes 55% of blood  90–92% water  Remaining is dissolved substances  Plasma proteins Albumin—helps transport fatty substances Globulin—3 types Fibrinogen—blood clotting protein  Smaller important substances

6 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Erythrocytes (Red Blood Cells)  Enucleated—no nucleus  Hemoglobin (Hgb, Hb) gives red color  5 million per cubic millimeter of blood  Average adult has 35 trillion; more in males than females  Life span of 120 days – Spleen removes worn out cells; iron can be reused; bilirubin is disposed by liver  Bilirubin

7 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Essential Nutrients for Red Blood Cell Synthesis  Iron  Folic Acid  Vitamin B12 Nutrient deficiencies, the presence of immature red blood cells, as well as the characteristic color and volume of red blood cells, are laboratory variables that are used to distinguish among different types of anemia.

8 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Anemia  Conditions that decrease red blood cells – Hemorrhages, excessive destruction, or impaired synthesis  Symptoms are due to tissue hypoxia or lack of oxygen. – Pallor or lack of color, fatigue, dizziness, headaches, decreased exercise tolerance, rapid heartbeat, and shortness of breath  Untreated anemia may progress to death from heart failure or cardiovascular collapse or shock.

9 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Iron Deficiency Anemia   Most common cause of anemia   Etiology: Increased iron requirements, impaired iron absorption, or hemorrhage may cause iron deficiency anemia.   Without enough iron, the body fails to synthesize hemoglobin, and the ability to transport oxygen is reduced.

10 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Risks for Iron Deficiency Anemia   Iron requirements are greatest during the first two years of life.   Adolescent girls may become iron deficient due to inadequate dietary iron, increased growth requirements, and the onset of menstruation.   Sudden growth spurt in adolescent boys may significantly increase physiological demands for iron resulting in iron deficiency anemia.

11 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Risks for Iron Deficiency Anemia (continued)   Supplemental iron is needed during pregnancy as iron is provided to the developing fetus.   Decreases in iron absorption occur with malabsorption syndromes and chronic disease.   Iron absorption requires an intact gastrointestinal tract with healthy intestinal mucosal cells. Chronic disease, removal of the stomach, and bowel disorders limit availability of iron required for the synthesis of hemoglobin.

12 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Symptoms and Treatment of Iron Deficiency Anemia  Symptoms specific to iron deficiency include a craving for ice, swelling of the tongue, and dry lips.  Diagnosis of iron deficient anemia is confirmed by microscopic examination of the blood. Reduction in red blood cells is called hypochromia.  Treatment: identify and correct any causes of bleeding – Oral supplements are effective in those with an intact gastrointestinal tract. – The addition of vitamin C enhances iron absorption. – Injectable iron supplements are available for individuals with malabsorption or those who cannot tolerate oral supplementation.

13 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Anemia of Chronic Disease  The second leading cause of anemia worldwide  Chronic disease such as heart disease, cancer, arthritis, and infectious disease induce inflammatory changes that suppress red blood cell synthesis in the bone marrow, and shorten survival of red blood cells already within the systemic circulation.  The chronic nature of the disease usually parallels the severity of the anemia.

14 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Vitamin B12 Deficiency Anemia Pernicious Anemia   Caused by inadequate absorption or intake of vitamin B12, or a deficiency in a protein called intrinsic factor.   Intrinsic factor is produced in the stomach and is essential for the absorption of vitamin B12 from the small intestine.   Without vitamin B12 and intrinsic factor, the membranes of immature red blood cells rupture easily within the chemical environment of the blood stream.   The result is fewer than normal red blood cells, and consequently a reduced oxygen carrying capacity.

15 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Etiology and Symptoms of Pernicious Anemia   Causes of pernicious anemia – – Inadequate diet, absorption, and utilization – – Increased requirements and increased excretion of vitamin B12 – – Strict vegetarianism – – Abnormal bacterial growth in the small intestine (impair absorption or enhance elimination of vitamin B12) – – Removal of the stomach or the bowel impairs availability of intrinsic factor and limits absorption of vitamin B12.   Symptoms: abdominal distress such as nausea, and vomiting, and burning of the tongue – – Neurological disturbances include numbness, weakness, and peculiar yellow and blue color blindness.

16 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Treatment of Pernicious Anemia   Vitamin B12 supplementation   Vitamin B12 cannot be absorbed into the bloodstream, it must be replaced by injection.   Vitamin B12 supplementation is required for life for strict vegetarians, those with chronic bowel disorders, or individuals who have had their stomach or bowel partially or fully removed.

17 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Folic Acid Deficiency Anemia – Risks and Etiology   Common in the Western world where consumption of raw fruits and vegetables is low   Inflammation of the bowel as in Crohn’s disease and other adverse conditions – certain medications impair absorption of folic acid   Body stores of folic acid are small and therefore folic acid deficiency anemia occurs within a few months.   Pregnant and lactating females, alcoholics, and individuals with kidney disease are especially susceptible to folic acid deficiency anemia owing to increased metabolic demands.

18 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Diagnosis and Treatment of Folic Acid Deficiency Anemia   Measurement of serum folic acid levels is conclusive for folic acid deficiency anemia.   Oral folic acid supplementation is effective in replacing folic acid and meeting increased requirements for those with increased metabolic demands.

19 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Hemolytic Anemia Etiology  Reduction in circulating red blood cells that is caused by pathological conditions that accelerate destruction of red blood cells.  Inherited abnormalities such as hemoglobin defects, enzyme defects, and membrane defects impair intrinsic physical properties that are needed for optimal red blood cell survival.  Infectious agents, certain medications, and immune disorders may also reduce red blood cell survival.

20 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Symptoms of Hemolytic Anemia  Significant red blood cell destruction produces symptoms similar to those of other anemias.  Hemolytic anemia produces increased serum levels of bilirubin that result from the degradation of heme in destroyed red blood cells.  Accumulation of bilirubin causes a jaundiced or yellow orange appearance in the tissues, urine, and feces.

21 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Hemoglobin  Hemoglobin is composed of four protein chains; two alpha chains and two beta chains. Each chain is attached to a heme group that contains iron. Oxygen molecules bind to the heme portion of the hemoglobin to form oxyhemoglobin. Since single hemoglobin has four heme groups it can transport four oxygen molecules. Hemoglobin also transports a small amount of carbon dioxide.

22 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Sickle Cell Anemia Anemia Due to Defective Hemoglobin Synthesis  Sickle cell anemia is a genetically transmitted disorder marked by severe hemolytic anemia, episodes of painful crisis, and increased susceptibility to infections.  Approximately ten percent of African Americans have the sickle cell trait.  Red blood cells contain an abnormal form of hemoglobin or hemoglobin S.  As the red blood cell deoxygenates, hemoglobin S forms cross-links with other hemoglobin S molecules and long crystals develop. Crystals continue to form as oxygen is released and the red cells assume a sickled shape.

23 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Sickle Cell Anemia Complications  Sickled red blood cells are inflexible and rigid and cause mechanical obstruction of small arterioles and capillaries, leading to pain and ischemia.  Sickled cells are also more fragile than normal cells leading to hemolysis.  Tissue death secondary to ischemia causes painful crisis that progresses to organ failure with repeated occlusive episodes.

24 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Sickle Cell Anemia – Treatment  Sickle cell anemia cannot be cured.  Treatment is aimed at preventing sickle cell crisis, controlling the anemia, and relieving painful symptoms.  Painful crises are adequately managed with narcotic analgesics.  Blood transfusions and fluid replacement expand blood volume and oxygen exchange needed for reperfusion of occluded vessels.

25 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Click here to view an animation showing sickle cells. Return to Directory

26 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Click here to view a video on the topic of sickle cell anemia Return to Directory

27 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Thalassemia  A group of inherited blood disorders in which there is deficient synthesis of one or more alpha or beta chains required for proper formation and optimal performance of the hemoglobin molecule. There are several different categories of Thalassemia that produce mild to severe symptoms.  The most severe forms of thalassemia produce severe life threatening anemia, bone marrow hyperactivity, and enlargement of the spleen, growth retardation, and bone deformities. Blood transfusions are required to sustain life and life expectancy is reduced.

28 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Table 8-1: Categories of Thalassemia

29 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Table 8-1 (continued): Categories of Thalassemia

30 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Platelets  Thrombocyte  Smallest of all blood elements  Not whole cells  200,000–300,000 per cubic millimeter  Critical in clotting or hemostasis  Agglutinate into small clusters  Leads to formation of thrombin, which converts fibrinogen to fibrin

31 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Platelet Disorders – Thrombocytopenia  Small number of platelets or thrombocytopenia  Result from conditions that either impair the production, increase destruction, or cause sequestration of platelets  Prolonged bleeding results from minor and major trauma.  Spontaneous hemorrhages are often visible on the skin as small, flat, red spots called petechiae, or larger purplish patches called ecchymosis. Spontaneous hemorrhages may also occur in the mucous membranes of the mouth and internal organs.

32 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Thrombocytopenia – Etiology and Treatment  Suppression of the bone marrow by certain medications or cancer may diminish platelet production.  Autoimmune disorders may increase platelet destruction or impair platelet function.  Massive blood transfusions dilute circulating platelets and decrease platelet viability.  Thrombocytopenia can usually be corrected by treating the underlying cause.  Preventative measures such as bed rest, to avoid accidental trauma, are highly recommended until platelet counts increase to acceptable levels.  Platelet transfusions are reserved for severe thrombocytopenia or in cases of severe bleeding.

33 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Primary Thrombycythemia  Primary thrombocythemia is a marked increase in circulating platelets due to unknown causes.  Primary thrombocythemia occurs most frequently in adult men and women during the sixth or seventh decade of life.  Symptoms are related to abnormal platelet function and thrombosis. Thrombosis causes ischemia to the central nervous system, the peripheral extremities, and vital organs of the body.  Symptoms include dizziness, visual problems, headaches, difficulty breathing, and extreme pain in the extremities. Bleeding may result in some cases due to abnormal platelet function.

34 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Disorders of Platelet Adhesion  von Willebrand's disease  A mutation in the von Willebrand factor gene is a severe disorder characterized by a tendency to bleed from mucous membranes despite adequate levels of circulating platelets. A deficiency in the von Willebrand clotting factor as well as defective platelet aggregation and adherence leads to excessive and prolonged bleeding and anemia.  Chronic medical treatment is generally not required for most individuals with platelet function disorders. The avoidance of aspirin that inhibits platelet function is recommended, as well as preventive measures prior to invasive medical, surgical, or dental procedures. The most definitive treatment for severe bleeding is platelet transfusion.

35 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Blood Coagulation Disorders  Hemophilia is a sex linked, inherited coagulation disorder caused by a deficiency of clotting factors. Because hemophilia is an X-linked disorder, almost all symptomatic individuals are males. Daughters of affected males have a 50:50 chance of being carriers, whereas sons of carrier shave a 50:50 chance of having hemophilia.

36 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Severity of Hemophilia  The severity of hemophilia depends on how the gene affects the activity of the clotting factors. – The number of bleeds and whether the bleed occurs spontaneously or with trauma – Severely affected individuals may have two or three bleeding episodes per month, or may spontaneously bleed without noticeable trauma, or may bleed profusely without immediate treatment. – Moderately affected individuals bleed approximately five or six times per year but may have prolonged periods free of bleeding, and usually bleed only with trauma. – Mildly affected individuals bleed rarely, unless provoked by significant trauma or surgery.

37 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Treatment of Hemophilia  Regular transfusion to replace deficient clotting factors  Mildly affected individuals may occasionally need transfusions.  In all cases, situations that might provoke bleeding should be avoided, and preventive medications can be administered prior to dental procedures and surgery.

38 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Purpura Simplex  Easy bruising, usually affects women and appears to have a hereditary predisposition  Bruises develop without an apparent cause and the vasculature appears fragile.  The number of platelets, as well as the platelet activity, is normal, and the condition is generally not serious. Bed rest and avoidance of aspirin- containing products that can suppress platelet function is recommended.

39 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Vascular Bleeding Disorders  Purpura Simplex  Hereditary Hemorrhagic Telangiectasia

40 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Hereditary Hemorrhagic Telangiectasia  A disorder characterized by malformations of the vasculature. It is a hereditary disorder that affects both men and women. Red to violet telangiectatic lesions appear on the face, lips, oral and nasal mucosa, as well as on the tips of the fingers and toes.  Telangiectatic lesions are due to abnormal dilation of existing small vessels. These vessels rupture easily and form artificial shunts or fistulas to critical organs of the body. Mild disease is characterized by recurrent nosebleeds.

41 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Hereditary Hemorrhagic Telangiectasia – Complications and Treatment  With severe disease, extensive fistulas develop to the lungs, resulting in shortness of breath and fatigue, due to deficient oxygenation.  Infected emboli also develop in telangiectatic vessels, resulting in strokes or ischemia to the brain.  Telangiectases in the nose and gastrointestinal tract may be treated with laser ablation.  Large fistulas usually require surgical resection. Multiple blood transfusions and continuous iron therapy may be needed if blood loss is excessive.

42 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Bleeding Disorders Platelets and Clotting Factors  Bleeding disorders result from platelet dysfunction or deficiency, vitamin K deficiency, and clotting factor deficiencies.  Platelets or thrombocytes are blood elements produced in the bone marrow that are essential for blood clotting in response to immediate injury, and for the mobilization of clotting factors.  Clotting factors are formed in the liver and are released in response to tissue injury and platelet fragments to form insoluble fibrin clots.  Vitamin K is required for the synthesis of the prothrombin and fibrinogen, clotting factors. Platelets, clotting factors, vitamin K, and calcium are essential for hemostasis, or the arrest of bleeding.

43 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. White Blood Cells  Leukocytes or white blood cells include neutrophils, eosinophils, basophils, monocytes, and lymphocytes.  Synthesized in the bone marrow  Primary function of leukocytes is to defend tissues against infections and foreign substances.  Quantitative abnormalities, inherited acquired defects, and neoplastic alterations result in disease and disability.

44 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Leukocytes  White blood cells  Provide protection against pathogens – Bacteria – Viruses – Foreign material  Spherical shape with nucleus  8,000 per cubic millimeter of blood

45 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Leukocyte Classification  LeukocytesFunction  Granulocytes – BasophilsRelease histamine and heparin to damaged tissue – EosinophilsDestroy parasites and increase during allergic reaction – Neutrophils Important for phagocytosis  Agranulocytes – MonocytesImportant for phagocytosis – LymphocytesProvide protection through immunity

46 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Neutropenia  A reduction of circulating neutrophils increases the risk for bacterial and fungal infections. Because neutrophils are responsible for most clinical findings during an acute infection, the classic signs of infection may be diminished or absent in a severely neutropenic individual.

47 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Neutropenia – Etiology  Complication of medication used for cancer chemotherapy or medications used for immune suppression – These medications suppress cellular proliferation within the bone marrow.  Infectious complications depend on the severity of neutropenia; they are usually profound and severe in cancer patients.  Immune destruction of neutrophils occurs with rheumatoid arthritis, or as a primary condition with unknown causes.  Neutropenia may either be mild or severe and infectious complications are variable. Chronic and severe cases require medical treatment with medications that increase neutrophil proliferation or medications that suppress immune function.

48 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Leukemia  Cancer of the white blood cells results in production of a large number of abnormal leukocytes.  Overproduction of malignant white cells suppresses the production of red blood cells and platelets.  Organs where blood is stored, such as the liver and the spleen, become greatly enlarged with infiltration of malignant white blood cells.

49 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Table 8-2: Comparison of leukemia types.

50 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Leukemia – Etiology  Etiology is unknown; may be due to a virus or exposure to radiation  A high incidence of leukemia has been reported in areas around the world exposed to fallout from nuclear energy. Heredity may also play a part in its etiology.

51 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Leukemia – Signs and Symptoms  Fever, swollen lymph nodes (lymphadenopathy), joint pain, abnormal bleeding and weight loss  Anemia, with its manifestations of weakness, shortness of breath, and heart palpitations accompanies leukemia.  Blood clotting is reduced with a reduction of platelets causing a tendency to bruise and hemorrhage.  White blood cells are produced faster than they mature and are ineffective in fighting infections.

52 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Types of Leukemia  Myelogenous: cancer originates in the bone marrow – Primitive white cells in this tissue are called myelocytes. In myelogenous leukemia, neutrophil production is greatly increased, and both red blood cell and platelet production is suppressed.  Lymphocytic leukemia – Malignancy of the lymphatic cells, found both in the bone marrow and lymph nodes. The lymphocytes, in this case, are the only blood cells that are increased; however, they become disproportionately high in number and are immature and ineffective.

53 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Types of Leukemia (continued)  Both types of leukemia may be chronic or acute. – Acute lymphocytic leukemia is the more common form in children – Has an abrupt onset and progresses rapidly. – Immature lymphocytes with diminished activity accumulate in the systemic circulation and symptoms appear rapidly.  Chronic forms are more common in adults. – Produce cells that undergo some maturation and are at least partially functional – Because the cells do function, the disease is slow to develop and is often discovered by accident during routine blood tests.

54 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Leukemia – Treatment  Progress is being made in controlling and finding a cure for leukemia.  Treatment goals include – Eliminating leukemic cells by inhibiting their growth, maintaining remission, and prevention of complications from the disease and its treatments – Inhibiting the growth of malignant cells and maintaining healthy cells with chemotherapy medications  Side effects of chemotherapy – Due in part to growth suppression of healthy cells – The ability of the patient to tolerate adverse medication effects determines the intensity of the chemotherapy. Remission is possible in 50 to 90% of patients.

55 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Click on the screenshot to view a video on the topic of leukemia. Return to Directory

56 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Abnormalities of Monocytes  Myelomonocytic leukemia  Myelomonocytic leukemia is a variation of acute myelogenous leukemia most commonly seen in adults.  Common symptoms are fever, weight loss, lymphadenopathy, enlarged spleen, anemia, and thrombocytopenia. This disorder is rapidly fatal if left untreated. With treatment, current survival is approximately 40%.

57 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Abnormalities of Eosinophils and Basophils Idiopathic Hypereosinophilic Syndrome  The onset of this disease occurs between the ages of 20 and 50 years and there is a strong male predominance.  Persistent increases in blood eosinophils and associated involvement of the heart and nervous system is responsible for the most important clinical symptoms. – Congestive heart failure, valvular dysfunction, conduction defects, and myocarditis – Congestive heart failure is a frequent cause of death. – Neurologic findings may include altered behavior and cognitive function, spasticity, and ataxia.  Prognosis in the idiopathic hypereosinophilic syndrome historically has been poor, with median survival of approximately 1 year. However, chemotherapy has recently been reported to produce 70% survival at 10 years.

58 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Eosinophilia – Myalgia Syndrome  A chronic multisystem disease with a spectrum of clinical symptoms – Self-limited myalgias or muscle pain and fatigue – Progressive and potentially fatal illness – Skin changes, nervous system abnormalities, and pulmonary hypertension  Elevation of circulating levels of eosinophils is a universal feature of this disorder, and the illness has been related to ingestion of the dietary supplement L-tryptophan.

59 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Diagnostic Tests   Blood tests – Total blood counts (red blood cells and white blood cells and platelets), hemoglobin, hematocrit, serum chemistry, and enzyme and hormone levels within the body. – Differential blood analysis provides qualitative information such as size, shape, and ratio of one cell type to another.  Bone marrow – To diagnose malignant blood disorders, and increases or decreases in blood counts without any apparent cause – Provides information on the function of the bone marrow and the qualitative characteristics of stem cells that give rise to all blood cells

60 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Blood Typing  A laboratory test to determine if the donated blood is compatible with the recipient’s blood  There are many different subgroups of blood markers, but the two most important ones are the ABO system and Rh factor.

61 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. ABO System  There are two possible RBC markers, A and B. A person with an A marker is said to have type A blood.  Type A blood produces anti-B antibodies. The presence of a B marker gives type B blood and anti-A antibodies.  Absence of either an A or a B marker results in type O blood, which contains both anti-A and anti-B antibodies.  If both markers are present, the blood is type AB and does not result in any antibodies.

62 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Universal Donor/Recipient  Because type O blood does not have either marker A or B, it will not react with anti-A or anti-B antibodies.  For this reason a person with type O blood is referred to as a universal donor. In an emergency, type O blood may be given to a person with any of the other blood types.  Similarly, type AB blood is the universal recipient  A person with type AB blood has no antibodies against the other blood types and therefore, in an emergency, can receive any type of blood.

63 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Rh Factor  Rh factor is not as difficult to understand as the ABO system.  A person with the Rh factor on his or her red blood cells is said to be Rh-positive (Rh+).  Since this person has the factor, he or she will not make anti-Rh antibodies.  A person without the Rh factor is Rh-negative (Rh- ) and will produce anti-Rh antibodies.  Therefore, an Rh+ person may receive both an Rh+ and an Rh- transfusion, but an Rh- person can receive only Rh- blood.

64 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Click on the screenshot to view a video on the topic of initiating blood therapy. Return to Directory

65 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Click on the screenshot to view a video on the topic of phlebotomy and venipuncture. Return to Directory

66 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Click on the screenshot to view a video on the topic of blood test basics. Return to Directory

67 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Click on the screenshot to view an animation on AIDS. Return to Directory

68 Mulvihill, Zelman, Holdaway, Tompary, and Raymond Human Diseases: A Systemic Approach, 6e Copyright ©2006 by Prentice-Hall, Inc. Upper Saddle River, New Jersey 07458 All rights reserved. Click on the screenshot to view a video on the topic of AIDS. Return to Directory


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