Hematology Flashcards
Erythropoiesis: In bone marrow
- ) Decreased blood oxygen
- ) Kidneys secrete erythropoietin
- ) Bone marrow stimulated
- ) Creates new blood cells
A man with renal failure would develop anemia because of the non-secretion of erythropoietin
Anemia
Decreased Hgb &/or RBC
Causes:
Blood loss
Impaired erythrocyte production
Increased erythrocyte destruction (hemolysis)
Signs and Symptoms
Gradual vs. sudden
Magnitude
Classic signs of Anemia:
Pallor
Fatigue
Dyspnea on Exertion (DOE)
Dizziness
Compensatory Manifestations:
CV: Tachycardia, palpitations, & vasoconstriction
Respiratory: Tachypnea & increased depth of breathing
Classification of Anemia
Based on Size - MCV
Normocytic
Macrocytic (large)
Microcytic (small)
Based on Color – MCHC (Hgb content)
Normochromic
Hyperchromic- (increased Hgb)
Hypochromic – (decreased Hgb)
MCV- Mean corpuscular Volume
Normally 80-90
MCHC- Mean Corpuscular Hemoglobin Concentration
Normally 32-36%
Iron Deficiency Anemia
Microcytic- Hypochromic
Etiology
Inadequate intake
Increased demand (growth, pregnancy)
Bleeding
Signs & symptoms
Classic signs of anemia PLUS…
Angular cheilosis. (cracking lips), brittle hair & nails, smooth tongue (“glossitis”), pica (craving non-foods), koilonychia (moon-shaped)
Evaluation
CBC analysis
Serum Iron profile
Total Fe, TIBC, Ferritin
Iron Deficiency Anemia Treatment
Control chronic blood loss Bleeding/GI losses are the most common cause Increase dietary intake Iron Supplements Types ferrous sulfate iron dextran Blood transfusion if necessary
Vitamin B12 Deficiency
Macrocytic- Normochromic or “megaloblastic” anemia
Etiology
Defective gastric secretion of intrinsic factor (IF)
Interference of IF binding to B12 caused by chronic conditions of malabsorption and atrophic gastritis; gastrectomy.
Dietary deficiency (rare)
Signs and Symptoms
Classic signs of anemia
Glossitis
Neuro changes!
Vit. B12 is necessary for the synthesis of myelin
Paresthesias, loss of vibratory & position sense, ataxia, dementia
Evaluation
CBC analysis
Schilling test
Antibodies to intrinsic factor and gastric parietal cells
Folate Deficiency Anemia
Macrocytic- Normochromic
Etiology
Dietary deficiency & malnutrition
Commonly seen in alcoholism (ETOH interferes w/ folate metabolism)
Malabsorption
Symptoms
similar to B12 def. but NO neuro manifestations
Classic signs of anemia
Anemia of Chronic Disease
Normocytic - Normochromic
Decreased lifespan of erythrocyte & ↓ response to erythropoietin
In response to cell injury-
Macrophages sequester iron in the spleen
Lymphocytes release cytokines that ↓ erythropoetin response
Etiology
Chronic infections (HIV/AIDS)
Chronic inflammatory diseases (RA, SLE, CRF)
Malignancies
Clinical manifestations
Classic signs of anemia
Evaluation
CBC analysis
Screening for malignancies if chronic infection or inflammation is not present
Hemolytic Anemia
Premature, accelerated destruction of RBCs
Erythropoiesis is normal & often accelerates in an effort to compensate for the increased destruction
Elevated bilirubin as result of increased destruction
Jaundice & icterus
Elevated reticulocytes as result of hyperactive bone marrow
Acquired Immune mediated (autoimmune; transfusion reaction, hemolytic disease of the newborn); physical trauma; drugs
Hemolytic Anemia Manifestations/Treatment
Clinical manifestations
Classic signs of anemia
Jaundice
Splenomegaly
Treatment
Removing the cause
Treat underlying disorder
Blood transfusions
Splenectomy if this is site where hemolysis is occurring
Corticosteroids if immune mediated hemolysis
Sickle Cell Disease
Sickle cell disease is inherited in an autosomal recessive pattern.
Offspring inherit one recessive allele (S) and one normal allele (heterozygous expression) they become unaffected carriers and are said to have sickle cell trait (clinically silent).
Sickle cell disease is caused by a mutation in the hemoglobin beta (HBB) gene, leading to a single change in amino acid sequence.
The abnormal gene product (hemoglobin S) is not stable.
Heterozygote Advantage for SCD
When a person inherits only one allele for Hemoglobin S, they do not express disease.
Instead, heterozygous expression of Hgb S is correlated with lower rates of mortality among carriers who are of African and Mediterranean descent,
Because the HbS allele decreases the risk of infection by malarial parasites endemic in those areas. (“heterozygote advantage”)
Phenotypic Features for SCD
Blood vessel occlusion**
Acute pain
Infarctions cause chronic damage to liver, spleen, heart, kidneys, eyes, bones
Pulmonary infarction acute chest syndrome (PNA)
Cerebral infarction stroke
Marrow infarcts painful bone crisis
Splenic injury functional aspenia
Management & Treatment for SCD
Avoid factors that trigger sickling and vessel occlusion Stress Cold Infection Dehydration Hypoxia Acidosis Pain Management Hydration
Hydroxyurea is the most commonly prescribed therapy for sickle cell disease & has been shown to reduce the number of painful episodes, acute chest syndrome, and transfusions as well as to improve overall survival
Bone marrow or stem cell transplant if a suitable donor is found
Thalassemias: Alpha
Defective gene for alpha-chain synthesis May have 1–4 defective genes Severity depends on how many genes deleted Affects both fetal and adult Hgb Most common among Asians Also Africans & African Am
Thalassemias: Beta
Defective gene for beta-chain synthesis May have 1–2 defective genes Affects only adult Hb Most common among Mediterranean population Also Africans & African Am
Aplastic Anemia
Caused by bone marrow failure
Reduction of hematopoietic cells (RBCs, WBCs, platelets)
Pancytopenia (other name)
Etiology Acquired: Primary- idiopathic Secondary- Chemicals; chemotherapy; ionizing radiation; complication of AIDS Hereditary: rare
Clinical Manifestations
Classic signs of anemia
If WBCs low- infection
If platelets low- bleeding. Petechiae & echymoses
Polycythemia
Abnormally high RBC mass with a Hct > 50%
Relative
Loss of plasma volume
Absolute
Increase in total red cell mass
Classified as primary or secondary
Primary Polycythemia
“Polycythemia Vera”
Neoplastic disorder characterized by an abnormal proliferation of pluripotent cells of bone marrow
causes an increase in total red cell mass, WBC, and platelets.
Clinical manifestations
r/t ↑blood volume & viscosity
Decreased cerebral blood flow: headache, inability concentrating, hearing & visual difficulties
Venous stasis: plethoric (dusky red appearance)
Thromboembolism
Pruritis - r/t increased mast cells in the skin
Hemorrhage r/t platelet abnormalities
Treatment
Periodic phlebotomy
Chemotherapy
Secondary Polycythemia
A physiologic response to hypoxia caused by an appropriate secretion of erythropoietin
High risk groups: Living at high altitudes Smokers Persons with CHF Persons with COPD Persons w/ neoplasms that develop the ability to secrete erythropoietin
Clinical manifestations- Similar to polycythemia vera
Treatment
Relieving hypoxia
Platelets (Thrombocytes)
Megakaryocytes formed in bone marrow
Break apart to form platelets
Thrombopoietin
Made in liver, kidney, smooth muscle, bone marrow
Platelets live 8–9 days in circulation
Many are stored in spleen
Released when needed
150,000 – 400,000 platelets/μL