Developmental Alterations of Hematological Function PDF

# Developmental Alterations of Hematological Function ## Nursing 302 ### Iron Deficiency Anemia (Acquired Disorders of Erythrocytes) - Most common blood disorder of infancy and childhood - Infants & young children who drink excessive amounts of cow's milk may develop IDA because of chronic intes...

# Developmental Alterations of Hematological Function ## Nursing 302 ### Iron Deficiency Anemia (Acquired Disorders of Erythrocytes) - Most common blood disorder of infancy and childhood - Infants & young children who drink excessive amounts of cow's milk may develop IDA because of chronic intestinal blood loss - Protein that causes inflammation in GI tract, damage to the mucosa, and leads to microhemorraging ### Can result from - Dietary lack of iron - Problems with iron absorption - Blood loss - Increased requirement for iron ### Clinical Manifestations: - Irritability, decreased activity tolerance, pica (MILD) - Pallor, tachycardia, murmurs (SEVERE) - Developmental delays; changes in the child's attention span, alertness and learning ability ### Treatment? *** ### Acquired Congenital Hemolytic Anemia (Acquired Disorders of Erythrocytes) - Hemolytic disease of fetus and newborn (HDFN) - Alloimmune disease (fighting against an invader) - The first incompatible fetus causes the mothers immune system to produce antibodies that will affect the fetuses of subsequent incompatible pregnancies - ABO incompatibility or Rh incompatibility ### Manifestations: - Anemia - Hyperbilirubinemia... jaundice! - Kernicterus (brain damage from high levels of bilirubin) ### Prevention is KEY! Immunoprophylaxis using Rh immune globulin (RhoGAM) to prevent Rh-negative woman from producing antibodies. *** ### Sickle Cell Disease (Inherited Disorder of Erythrocytes) - Hereditary condition where there is a replacement of normal Hgb with abnormal Hgb S ### Sickle Cell Trait (Heterozygous, Ss) - Most common form of sickle cell disease - Child is a CARRIER of sickle cell anemia & rarely has symptoms ### Sickle Cell Anemia (Homozygous, ss) - Child is subjective to sickle cell crises - Chronic hemolytic anemia ### Pathophysiology - During episodes of sickling, RBCs become abnormally crescent shaped - Sickling depends on degree of oxygenation, pH and dehydration - Sickled cells can resume a normal shape when rehydrated and re-oxygenated - May occlude small blood vessels - RBCs become weak and are hemolyzed and removed ### Types of Sickle Cell Crises 1. Vaso-occlusive Thrombotic Crisis* 2. Splenic Sequestration/Sequestration Crisis 3. Aplastic Crisis *** ### Sickle Cell Crises ### Vaso-occlusive Thrombotic Crisis - Precipitated by dehydration, exposure to cold, acidosis, or localized hypoxemia - Caused by stasis of blood with clumping of cells in microcirculation → ischemia → infarction - Thrombosis and infarction of local tissue may occur if the crisis is not reversed - Cerebral occlusion can result in a stroke! - Symptoms: fever, pain, tissue engorgement & redness *** <h3>Splenic Sequestration/Sequestration Crisis</h3> - Life threatening: death can occur within hours - Caused by pooling of blood in the spleen - Signs include profound anemia, hypovolemia, and shock - CM of hypovolemia (low BP, high HR!) - Complications of Splenomegaly... include? (hint: life-threatening) *** ### Aplastic Crisis - Diminished production and increased destruction of RBCs - Triggered by viral infection or depletion of folic acid - Virus causes a temporary shutdown of RBC production in the bone marrow but hemolysis continues → leads to a sudden drop in hemoglobin level - Signs: profound anemia, pallor, and fatigue *** ### Hemophilias (Inherited Coagulation and Platelet Disorders) ### Coagulation Review After injury, \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ are activated by many products of tissue destruction and inflammation, including collagen, thrombin, and \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_-activating factor. A blood clot is a meshwork of protein (fibrin) strands that stabilizes the platelet plug and traps other cells, such as erythrocytes, phagocytes, and microorganisms. In hemophilia, there is a problem with the intrinsic & extrinsic pathway and/or the fibrinolytic system - It is a hereditary bleeding disorder or spontaneous mutation of the factor gene - Treatment is determined on what factor is deficient by replacement therapy; not a cure but symptoms can be controlled. - Hemophilia A and Hemophilia B are most common. *** ### Hemophilia Clinical Manifestations - Prolonged bleeding - Excessive bruising - Subcutaneous and intramuscular hemorrhages - Oral bleeding from dental work - Hemarthrosis (bleeding into a joint space) - Signs of swelling, warmth, redness, pain and loss of movement of limbs - Bleeding in the neck, mouth, or thorax - Anemia - Hemorrhage from any trauma (cuts, injections, circumcision) - Hematomas in the spinal cord → paralysis *** ### Hemophilia A & B ### PATHO X-linked recessive trait - Males are mostly affected - Females may be carriers & may exhibit manifestations - Female carrier (4 possible outcomes with each pregnancy): - A girl who is not a carrier - A girl who is a carrier - A boy without hemophilia - A boy with hemophilia ### X-linked recessive | Unaffected | Carrier | Affected | |---|---|---| | Father | Mother | Father | | XY | XX | XY | | Unaffected son | Carrier daughter | Affected daughter | Unaffected son | Carrier daughter | Son | | XY | XX | XX | XY | XX | XY| | Unaffected | Carrier | Affected | | Son | Daughter | Son | | XY | XX | XY | | Unaffected | Carrier | Affected | | Son | Daughter | Son | | XY | XX | XY | *Note: a few carriers may be mildly affected due to skewed X-inactivation.* *** ### Hemophilia A & B ### Hemophilia A - Known as “classic hemophilia” - Deficiency of factor VIII (antihemophilic factor) - AHF is produced in the liver & is necessary for the formation of thromboplastin - Accounts for 80-85% of cases of hemophilia ### Hemophilia B - Also known as "Christmas disease" - Caused by deficiency of factor IX (Christmas factor)