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Mar 17 - hematologic - Hematologic System Disorders...

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Unformatted text preview: Hematologic System Disorders Disorders NURS 216 SPRING 2010 Sabra H. Smith, MS, RN Definitions Definitions Hematology – study of blood and bloodforming tissues Mononuclear phagocyte system – Mononuclear monocyte-macrophage system monocyte-macrophage Hematopoiesis – creation and maturation Hematopoiesis of blood cells (in bone marrow) of Blood dyscrasias – diseases or disorders Blood of the blood, range from mild to severe of Normal Blood Components Normal Plasma – composed of 8-9% solids Plasma proteins are important for clotting, Plasma blood volume regulation, transport of chemicals and drugs, pH and electrolyte balance, and immunity balance, Blood dyscrasias often involve malfunction Blood of the plasma proteins of Normal Blood Components Normal All All blood cells differentiate from a single stem cell (figure 9-1) stem Chemicals called ‘colony stimulating Chemicals factors’ cause differentiation factors’ Exs: Erythropoietin (EPO), G-CSF Blood Studies and Measures Blood CBC: CBC: complete blood count – RBCs, WBCs, platelets WBCs, Differential: more detailed, must smear a Differential: drop and stain, then count different types drop Shape: anisocytosis, poikolocytosis Color: normochromic, hypochromic Hemoglobin: concentration, type Hematocrit: % of RBCs in whole blood Blood Studies and Measures Blood MCV MCV – mean corpuscular volume: average size of RBCs average Reticulocyte count - # of reticulocytes in Reticulocyte blood blood Bone marrow aspirations Cytogenics Fe, EPO, B12, folic acid levels White Blood Cells Normal WBC count: 4,000-10,000/mm3 -neutrophils 55-65% -eosinophils 1-3% From the -basophils 0.3 – 0.5% differential -monocytes 3-8% -lymphocytes 20-30% colony-stimulating factors control the colony-stimulating differentiation from the stem cell differentiation WBC Disorders WBC Leukocytosis Leukocytosis – increase in # of WBCs (mostly neutrophils: neutrophilia), often in response to infection response Leukopenia/neutropenia – decreased # of Leukopenia/neutropenia WBCs/neutrophils WBCs/neutrophils -leukemias, chemotherapy, autoimmune -leukemias, conditions Agranulocytosis – usually due to Rx WBC Disorders WBC Neutropenia Neutropenia – increases risk of infection, if severe may not have inflammatory response response Infectious mononucleosis – EBVcontaminated saliva, incubates 4-8 weeks S/sx: fever, pharyngitis, lymphadenopathy possible hepatitis, splenomegaly, increased WBC (lymphocytes) Lymphomas Lymphomas Malignancies of the lymphatic system Possible etiologies: immunodeficiency, Possible toxins, viruses toxins, Initial tumor is in the secondary lymphatic Initial organs organs Hodgkins, non-Hodgkins, Burkitts Staging (I-IV) done after diagnosis Hodgkins Disease Hodgkins Common between 18 and 35, >50 years Reed-Sternberg cells S/sx: vary with location of tumors, include S/sx: lymphadenopathy, constitutional symptoms when disseminated, splenomegaly, immune defects splenomegaly, Chemotherapy, radiation Non-Hodgkin Lymphomas Non-Hodgkin Median Median age of 50 Solid tumors arising from lymph tissue B-cell or T-cells can become malignant at B-cell any stage of differentiation any Three times more prevalent than Hodgkin low-grade, intermediate, high-grade low-grade, (based on aggressiveness) (based Non-Hodgkin Lymphomas Non-Hodgkin S/sx: S/sx: lympadenopathy, may spread to GI tract, lung, liver, bone marrow, increased infections infections -depend upon type -diffuse, large B-cell types are very aggressive, fatal if untreated aggressive, Treatment depends upon type and stage Leukemias Leukemias Proliferation Proliferation of malignant hematopoietic stem cells, which replace normal bone marrow cells marrow Classifications: (1) morphologic, based on Classifications: predominant leukemic cells in marrow, (2) based on timeline, acute or chronic based Acute Leukemias Acute Acute Acute myeloblastic leukemia (AML) – affects the myeloblasts, 80% of acute leukemias in adults, fatal in 3-6 months leukemias blood smear shows decreased blood granulocytes and platelets – S/sx? granulocytes may have very high absolute WBC count Acute Leukemias Acute Acute Acute lymphocytic leukemia (ALL) – most common cancer in children under 15 common Abrupt onset, rapidly fatal if untreated Abnormal proliferation of lymphoblasts WBC count usually very high, others WBC usually low usually Similar s/sx to AML + lymphadenopathy, Similar bone pain, arthralgia, CNS problems bone Chronic Leukemias Chronic CML – myelocytic Insidious onset Proliferation of all granulocytes, count Proliferation usually over 30,000/mm3 usually S/sx: fatigue, wt loss, diaphoresis Chronic, accelerated, terminal blast crisis Chronic, phases phases Median survival 5 to 6 years Chronic Leukemias Chronic CLL – lymphocytic Usually in people over 60 years Proliferation of abnormal lymphocytes, can Proliferation be > 100,000/mm3 (usually B be lymphocytes-effect?) lymphocytes-effect?) Organ enlargement, ineffective immune Organ response response Survival may be >10 years Plasma Cell Dyscrasias Plasma Disorder Disorder and proliferation of plasma cells in bone marrow and/or blood in The dysfunctional plasma cells secrete an The abnormal antibody called the M component component Multiple Myeloma Multiple Most common dyscrasia A single malignant clone of a plasma cell single replicates wildly, overtaking other cells in the bone marrow and out to blood the Malignancies spread to bones and organs Bone pain and destruction, recurrent Bone infections, anemia, renal disease infections, Radiograph of the skull showing multiple punched-out lesions in a patient with multiple myeloma. Myeloma Lesions Myeloma Coagulation Coagulation Hemostasis: arrest of bleeding 3 important processes: transient important vasoconstriction, platelet activation, activation of clotting factors activation Phases: vasoconstriction, platelet plug Phases: formation, activation of clotting cascade, formation of blood clot, dissolving of clot formation Platelets Platelets AKA AKA thrombocytes: non-nucleated, granular cell fragments granular Lifespan of 10 days 150,000-400,000/mm3 Release chemicals that cause Release adhesion/aggregation and help make clot adhesion/aggregation Inactive platelet is blue; activated one is green Activated platelets surrounding a RBC Clotting Factors Clotting Most Most clotting factors are plasma proteins that circulate in an inactive form that Most are made in the liver I – fibrinogen (becomes fibrin) II – prothrombin (becomes thrombin) The Clotting Cascade The Another Another multiple-step, domino-effect reaction reaction Begins with vascular injury (extrinsic and Begins intrinsic pathways) intrinsic Net effect is the production of fibrin Each step in the cascade must work Each correctly for the reaction to proceed and produce enough fibrin!!! produce Clot Resolution Clot Size Size and lifespan of blood clot needs to be limited limited Plasma proteins help control the clot The fibrinolytic system is another series of The proteins and enzyme reactions which break up the fibrin and blood clot break Bleeding Tests Bleeding Prothrombin time (PT): normal 11-13 secs Partial thromboplastin time (PTT): normal Partial 60-90 secs 60-90 INR Longer times indicate slower response of Longer platelets and the clotting cascade = higher risk of bleeding risk Platelet Problems Platelet Thrombocytosis Thrombocytosis – excess, can cause hemorrhage or thrombosis hemorrhage Thrombocytopenia – deficiency, can occur Thrombocytopenia from any condition that inhibits bone marrow marrow ITP ITP TTP Hemophilia Hemophilia A disorder of clotting factors Factor VIII – hemophilia A Factor IX – hemophilia B Both genes located on the X chromosome Classified as mild, moderate, severe Classified based on % activity of the clotting factors based Hemophilia Hemophilia The The clotting cascade can’t progress past Factor VIII/Factor IX Factor Bleeding into joints and soft tissues, postsurgical bleeding Prolonged PTT, normal platelet counts Clotting factors must be replaced DIC DIC A complication of several conditions Microthrombi develop inside vessels, Microthrombi shortage of coagulation proteins and platelets elsewhere = hemorrhage platelets Red Blood Cells Red 120 day lifespan RBC count: 4-6 million/mm3 MCV: 81-96 um3/RBC Hb: 12-16 g/dL (women),12-18 g/dL (men) Hct: 38-46% (women), 42-53% (men) Reticulocyte count: 1-2% Hemoglobin Hemoglobin The protein in RBCs that transports O2 O2 Each Hb molecule made of 4 protein Each chains and 4 ‘heme’ groups chains O2 molecules attach to heme groups Heme groups each contain a molecule of Heme iron iron Hemoglobin Hemoglobin RBC Life Cycle RBC Balance Balance between old and new cells must be maintained be EPO produced by kidneys when they EPO sense low O2 or low Hb sense RBC RBC maturation requires adequate nutrients: B12, copper, iron, folic acid, nutrients: copper, protein protein Old RBCs rupture and are phagocytosed RBC Recycling RBC Hb Hb is broken down to heme and globin – protein re-enters amino acid supply, iron recycled recycled Some heme breaks down to free bilirubin, Some conjugated by the liver and excreted as bile, urine, feces bile, RBC Problems RBC Anemias Anemias – decrease in #, size or Hb concentration concentration Anemias classified by morphology of Anemias RBCs or etiology RBCs Sickle cell disease Polycythemias – increase in # Anemias Anemias Manifestations Manifestations Differences Differences vary, but all are due to decreased O2 delivery to tissues! decreased between acute and chronic anemia anemia Pallor, weakness, tachycardia, chest pain Morphologic Anemias Morphologic Size: micro- or macrocytic Color: normo- or hypochromic Normocytic, normochromic: Normocytic, Macrocytic, Microcytic, normochromic: hypochromic Etiologies of Anemia Etiologies Increased Increased RBC loss – bleeding, destruction (hemolysis) due to intrinsic or extrinsic problems extrinsic Intrinsic: defective Hb (sickle cell disease), Intrinsic: impaired globin synthesis, membrane defects, enzyme deficiencies (G6PD) defects, Extrinsic: isoimmune or autoimmune Extrinsic: responses, malaria, hypersplenism responses, Etiologies of Anemia Etiologies Decreased or defective RBC production Disruption of bone marrow function Disruption (aplastic) (aplastic) Chronic disease of kidneys or liver Chronic Lack of essential ‘building blocks’ for Lack RBCs (iron deficiency, megaloblastic) RBCs Aplastic Anemia Aplastic Disorder of stem cells in bone marrow Life-threatening b/c causes pancytopenia Life-threatening pancytopenia Congenital, idiopathic (immunemediated?), or secondary Morphologic classification: S/sx: S/sx: general anemia problems, bleeding, increased infections increased Iron Deficiency Anemia Iron Major Major cause of anemia world-wide, especially in menstruating women especially Morphologic classification: May May be due to decreased iron intake, impaired absorption, or iron loss impaired S/sx: general anemia problems, brittle hair S/sx: and nails, tongue atrophy/inflammation, angular stomatitis angular Megaloblastic Anemia Megaloblastic Morphologic Usually classification: due to B12 or folic acid deficiency deficiency deficiency causes problems with DNA synthesis, failure of nuclei to mature and divide divide Folate def. s/sx: anemia + severe glossitis, Folate diarrhea, loss of appetite diarrhea, Sickle Cell Disease Sickle An An inherited genetic mutation changes the structure of Hb – Hb S structure During periods of deoxygenation, the Hb During molecule changes shape – ‘sickles’ molecule Tends to happen in capillaries, leads to Tends vascular occlusion -> sickle cell crisis vascular Repeated crises weaken RBCs, decrease Repeated their lifespan their Sickle Cell Disease Sickle Most Most common form of congenital hemolytic anemia hemolytic 1/600 African Americans S/sx due to vascular occlusions/infarcts in S/sx organs and CNS: impaired growth, atrophy of the spleen, recurrent crises (worsened by infection or dehydration), cardiac effects of anemia cardiac Changes of Sickle Cell Disease Polycythemia Polycythemia Increased Increased RBC count causes increased blood volume and viscosity blood Individuals prone to thrombosis, many Individuals non-specific symptoms of high blood volume volume Primary and secondary causes Tmt: phlebotomy, some Rx, appropriate Tmt: hydration if due to dehydration hydration ...
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