Alterations in Hematologic Function: Anemia, Blood Disorders - PDF

Copyright © 2017, Elsevier Inc. All rights reserved. Alterations in Hematologic Function  Copyright © 2017, Elsevier Inc. All rights reserved. 2 ANEMIA Reduction in the total number of erythrocytes in the circulating blood or in the quality or quanti...

Copyright © 2017, Elsevier Inc. All rights reserved. Alterations in Hematologic Function  Copyright © 2017, Elsevier Inc. All rights reserved. 2 ANEMIA Reduction in the total number of erythrocytes in the circulating blood or in the quality or quantity of hemoglobin Impaired erythrocyte production Acute or chronic blood loss Increased erythrocyte destruction Combination of the above  Copyright © 2017, Elsevier Inc. All rights reserved. 3 MEAN HEMATOLOGIC DIFFERENTIAL COUNTS FROM BIRTH TO ADULTHOOD Hematological Newborn 3 months 7-12 Adult Diff to 6 years years of of age age Hemoglobin (g/dl) 16.8 12 13.0 13 Hematocrit (%) 55 36 38 38 Reticulocytes (%) 5.0 1.0 1.0 1.0 Fe (mcg/dL) 100-250 50 – 120 50 – 120 Male 80 –180 Female 60 –160 Transferrin Males 20 – 50% Saturation Females 15 to 50% Ferritin mcg/L 25 - 200 50-200 7-142 Male – 12 – 300 Female 10 – 150 Folate ng/ml 5 - 25 Vit B12 160-950 pg/ml MMA < 3.6 micromole/mmol  Copyright © 2017, Elsevier Inc. All rights reserved. 4 ANEMIA (CONT.) Classifications: Etiology Morphology Size Identified by terms that end in -cytic Macrocytic, microcytic, normocytic Hemoglobin content Identified by terms that end in -chromic Normochromic and hypochromic  Copyright © 2017, Elsevier Inc. All rights reserved. 5 ANEMIA (CONT.) Physiologic manifestation Reduced oxygen-carrying capacity—hypoxemia Variable symptoms based on severity and the ability of the body to compensate Classic anemia symptoms: Fatigue Weakness Dyspnea Pallor  Copyright © 2017, Elsevier Inc. All rights reserved. 6 MACROCYTIC-NORMOCHROMIC ANEMIAS Also termed megaloblastic anemias Characterized by unusually large stem cells Result of defective DNA synthesis Caused by deficiencies in vitamin B12 or folate Coenzymes for nuclear maturation and the DNA synthesis pathway Ribonucleic acid (RNA) processes occur at a normal rate  Copyright © 2017, Elsevier Inc. All rights reserved. 7 MACROCYTIC-NORMOCHROMIC ANEMIAS (CONT.) Pernicious anemia Caused by a lack of intrinsic factor from the gastric parietal cells Required for vitamin B12 absorption Results in vitamin B12 deficiency  Copyright © 2017, Elsevier Inc. All rights reserved. 8 NORMAL RBC  Copyright © 2017, Elsevier Inc. All rights reserved. 9 MACROCYTIC- NORMOCHROMIC RBC  Copyright © 2017, Elsevier Inc. All rights reserved. 10 MACROCYTIC-NORMOCHROMIC ANEMIAS (CONT.) Pernicious anemia (Cont.) Early symptoms nonspecific and vague When hemoglobin at 7-8 g/dl: Weakness Fatigue Paresthesias Difficulty walking Loss of appetite Abdominal pain Weight loss Sore tongue Neurologic manifestations  Copyright © 2017, Elsevier Inc. All rights reserved. 11 MACROCYTIC-NORMOCHROMIC ANEMIAS (CONT.) Pernicious anemia (Cont.) Treatment Parenteral or high oral doses of vitamin B12 Lifelong  Copyright © 2017, Elsevier Inc. All rights reserved. 12 MACROCYTIC-NORMOCHROMIC ANEMIAS (CONT.) Folate deficiency anemia Absorption of folate occurs in the upper small intestine Not dependent on any other factor Similar symptoms to pernicious anemia except neurologic manifestations generally not seen Treatment requires daily oral administration of folate  Copyright © 2017, Elsevier Inc. All rights reserved. 13 MICROCYTIC-HYPOCHROMIC ANEMIAS Characterized by red cells that are abnormally small and contain reduced amounts of hemoglobin Related to: Disorders of iron metabolism Disorders of porphyrin and heme synthesis Disorders of globin synthesis  Copyright © 2017, Elsevier Inc. All rights reserved. 14 MICROCYTIC HYPOCHROMIC ANEMIA  Copyright © 2017, Elsevier Inc. All rights reserved. 15 MICROCYTIC-HYPOCHROMIC ANEMIAS (CONT.) Iron deficiency anemia Most common type of anemia worldwide Nutritional iron deficiency or blood loss; No intrinsic dysfunction of iron metabolism 2nd cause – metabolic or functional causes - Manifestations when serum Hgb decreased to 7 or 8 gm/dl Early: fatigue; weakness; shortness of breath; pale earlobes and palms, conjunctiva  Copyright © 2017, Elsevier Inc. All rights reserved. 16 MICROCYTIC-HYPOCHROMIC ANEMIAS (CONT.) Iron deficiency anemia (Cont.) Progression of iron deficiency causes: Brittle, thin, coarsely ridged, and spoon-shaped nails (koilonychia) A red, sore, and painful tongue Dry, sore corners of mouth (angular stomatitis) 17 Copyright © 2017, Elsevier Inc. All rights reserved.  Copyright © 2017, Elsevier Inc. All rights reserved. 18 NORMOCYTIC- NORMOCHROMIC ANEMIAS Characterized by red cells that are relatively normal in size and hemoglobin content but insufficient in number  Copyright © 2017, Elsevier Inc. All rights reserved. 19 NORMOCYTIC- NORMOCHROMIC ANEMIAS (CONT.) Aplastic Posthemorrhagic Acquired hemolytic Hereditary hemolytic Anemia of chronic inflammation  Copyright © 2017, Elsevier Inc. All rights reserved. 20 ACQUIRED DISORDERS OF ERYTHROCYTES IN CHILDREN Iron deficiency anemia Most common blood disorder of infancy and childhood Lack of iron intake or blood loss Manifestations: Irritability Decreased activity tolerance Weakness Lack of interest in play  Copyright © 2017, Elsevier Inc. All rights reserved. 21 INHERITED DISORDERS OF ERYTHROCYTES Sickle cell disease Disorders characterized by the presence of an abnormal hemoglobin (Hb S) One amino acid (valine) replaces another (glutamic acid) Deoxygenation and dehydration cause the red cells to solidify and stretch into an elongated sickle shape Inherited autosomal recessive disorder  Copyright © 2017, Elsevier Inc. All rights reserved. 22 SICKLE CELLS  Copyright © 2017, Elsevier Inc. All rights reserved. 23 INHERITED DISORDERS OF ERYTHROCYTES (CONT.) Sickle cell disease (Cont.) Can result in: Vaso-occlusive crisis (thrombotic crisis) Aplastic crisis Sequestration crisis Hyperhemolytic crisis Sickle cell trait Child inherits Hb S from one parent and Hb A from another Other forms: Sickle cell–thalassemia disease Sickle cell–Hb C disease  Copyright © 2017, Elsevier Inc. All rights reserved. 24 SICKLE CELL DISEASE (CONT.)  Copyright © 2017, Elsevier Inc. All rights reserved. 25 SICKLE CELL DISEASE (CONT.) Adapted from Hockenberry MJ et al, editors: Wong’s nursing care of infants and children, ed 10, St Louis, 2015, Mosby.  Copyright © 2017, Elsevier Inc. All rights reserved. 26 MYELOPROLIFERATIVE RBC DISORDERS Polycythemia Overproduction of red blood cells Relative polycythemia Result of dehydration Fluid loss results in relative increases of red cell counts and Hgb and Hct values  Copyright © 2017, Elsevier Inc. All rights reserved. 27 MYELOPROLIFERATIVE RBC DISORDERS (CONT.) Absolute polycythemia Primary absolute Abnormality of stem cell proliferation in the bone marrow Polycythemia vera (PV) JAK2 gene mutation Secondary absolute Increase in erythropoietin as a normal response to chronic hypoxia or an inappropriate response to erythropoietin-secreting tumors  Copyright © 2017, Elsevier Inc. All rights reserved. 28 MYELOPROLIFERATIVE RBC DISORDERS (CONT.) Polycythemia vera Abnormal, uncontrolled proliferation of RBCs, WBCs, and platelets Insidious Manifestations due to increased red cell mass and hematocrit Increased blood viscosity Hypercoagulopathy  Copyright © 2017, Elsevier Inc. All rights reserved. 29 MYELOPROLIFERATIVE DISORDERS Iron overload Hereditary hemochromatosis C282Y mutation Autosomal recessive disorder of iron metabolism  Copyright © 2017, Elsevier Inc. All rights reserved. 30 ALTERATIONS OF LEUKOCYTE FUNCTION Quantitative disorders: Increases or decreases in cell numbers Bone marrow disorders or premature destruction of cells Response to infectious microorganism invasion  Copyright © 2017, Elsevier Inc. All rights reserved. 31 QUANTITATIVE ALTERATIONS OF LEUKOCYTES Leukocytosis Leukocytosis is a normal protective physiologic response to physiologic stressors, such as infectious microorganisms Leukopenia Leukopenia is not normal and not beneficial A low white count predisposes a patient to infections  Copyright © 2017, Elsevier Inc. All rights reserved. 32  Copyright © 2017, Elsevier Inc. All rights reserved. 33 GRANULOCYTOSIS (NEUTROPHILIA) Neutrophilia is evident in the first stages of an infection or inflammation If the need for neutrophils increases beyond the supply, immature neutrophils are released into the blood  Copyright © 2017, Elsevier Inc. All rights reserved. 34 GRANULOCYTOSIS (NEUTROPHILIA) (CONT.) This premature release is detected in the manual WBC differential and is termed a shift to the left When the population returns to normal, it is termed a shift to the right  Copyright © 2017, Elsevier Inc. All rights reserved. 35 NEUTROPENIA Reduction in circulating neutrophils Causes: Prolonged severe infection Decreased production Reduced survival Abnormal neutrophil distribution and sequestration  Copyright © 2017, Elsevier Inc. All rights reserved. 36 GRANULOCYTOPENIA (AGRANULOCYTOSIS) Causes: Interference with hematopoiesis Immune mechanisms Chemotherapy destruction Ionizing radiation  Copyright © 2017, Elsevier Inc. All rights reserved. 37 EOSINOPHILIA Increase in circulating eosinophils Hypersensitivity reactions trigger the release of eosinophilic chemotactic factor of anaphylaxis from mast cells Increased in allergic disorders Increased in parasitic invasions  Copyright © 2017, Elsevier Inc. All rights reserved. 38 EOSINOPENIA Decrease in circulation numbers of eosinophils Usually caused by migration of cells to inflammatory sites Other causes: Surgery Shock Trauma Burns Mental distress  Copyright © 2017, Elsevier Inc. All rights reserved. 39 BASOPHILS BASOPHILIA BASOPENIA Increase in circulating  Decrease in basophils circulating numbers of Response to basophils inflammation and hypersensitivity  Occurs in acute reactions infections, Seen in hyperthyroidism, and myeloproliferative long-term steroid disorders therapy  Copyright © 2017, Elsevier Inc. All rights reserved. 40 MONOCYTES Monocytosis Poor correlation with disease Usually occurs with neutropenia in later stages of infections Monocytes are needed to phagocytize organisms and debris Monocytopenia Very little known about this condition  Copyright © 2017, Elsevier Inc. All rights reserved. 41 LYMPHOCYTES LYMPHOCYTOSIS LYMPHOCYTOPENIA Increase in the Decrease in the number or number of circulating proportion of lymphocytes in the blood lymphocytes in the blood Immune deficiencies Acute viral infections Drug destruction Epstein-Barr virus Viral destruction Radiation  Copyright © 2017, Elsevier Inc. All rights reserved. 42 LEUKEMIAS Malignant disorder of the blood and blood- forming organs Excessive accumulation of leukemic cells Pancytopenia if cells crowd bone marrow Acute leukemia Presence of undifferentiated or immature cells, usually blast cells Chronic leukemia Predominant cell is mature but does not function normally  Copyright © 2017, Elsevier Inc. All rights reserved. 43 ACUTE LEUKEMIAS Acute lymphocytic leukemia (ALL) Too many lymphoblasts Mostly occurs in children Acute myelogenous leukemia (AML) Too many myeloblasts Most common adult leukemia Manifested by bone marrow depression signs/symptoms, fever, anorexia, and neurologic symptoms Treated with chemotherapy  Copyright © 2017, Elsevier Inc. All rights reserved. 44 CHRONIC LEUKEMIAS Chronic myelogenous leukemia (CML) Too many blood cells made in bone marrow Chronic lymphocytic leukemia (CLL) Too many immature lymphocytes found in blood and bone marrow Most common adult leukemia in western world Slow progression Lymphadenopathy  Copyright © 2017, Elsevier Inc. All rights reserved. 45 LYMPHADENOPATHY Enlarged lymph nodes that become palpable and tender Local lymphadenopathy Drainage of an inflammatory lesion located near the enlarged node General lymphadenopathy Occurs in the presence of malignant or nonmalignant disease  Copyright © 2017, Elsevier Inc. All rights reserved. 46 LYMPHADENOPATHY (CONT.) Courtesy Dr. A.R. Kagan, Los Angeles. From del Regato JA et al: Cancer: diagnosis, treatment, and prognosis, ed 6, St Louis, 1985, Mosby.  Copyright © 2017, Elsevier Inc. All rights reserved. 47 MALIGNANT LYMPHOMAS Diverse group of neoplasms that develop from the proliferation of malignant lymphocytes in the lymphoid system REAL classification Two major categories: Hodgkin lymphoma Non-Hodgkin lymphoma  Copyright © 2017, Elsevier Inc. All rights reserved. 48 HODGKIN LYMPHOMA Characterized by the presence of Reed- Sternberg cells in the lymph nodes Reed-Sternberg cells are necessary for diagnosis, but they are not specific to Hodgkin lymphoma Classical Hodgkin lymphoma Nodular lymphocyte predominant Hodgkin lymphoma  Copyright © 2017, Elsevier Inc. All rights reserved. 49 HODGKIN LYMPHOMA (CONT.) Physical findings Adenopathy, mediastinal mass, splenomegaly, and abdominal mass Symptoms Fever, weight loss, night sweats, pruritus, fatigue Adult Hodgkin lymphoma can usually be cured with early diagnosis and treatment Chemotherapy Radiation Surgery  Copyright © 2017, Elsevier Inc. All rights reserved. 50 HODGKIN LYMPHOMA (CONT.) From Damjanov I, Linder J, editors, Anderson’s pathology, ed 10, St Louis, 1996, Mosby.  Copyright © 2017, Elsevier Inc. All rights reserved. 51 HODGKIN LYMPHOMA (CONT.) From del Regato JA et al: Cancer: diagnosis, treatment, and prognosis, ed 6, St Louis, 1985, Mosby.  Copyright © 2017, Elsevier Inc. All rights reserved. 52 HODGKIN LYMPHOMA (CONT.)  Copyright © 2017, Elsevier Inc. All rights reserved. 53 NON-HODGKIN LYMPHOMA Generic term for a diverse group of lymphomas New classification based on cell type (T, B, NK) Differentiated based on etiology, unique features, and response to therapies  Copyright © 2017, Elsevier Inc. All rights reserved. 54 DISORDERS OF PLATELETS Thrombocytopenia Platelet count