Topic 5 Child with Hematologic or Immunologic Disorders

• What is anemia?


  Decrease in the number of RBC's or a concentration of hemoglobin below normal levels.
• What are the three types of anemia?

  • Decreased RBC production
  • Blood loss
  • RBC destruction
• What can iron and folate deficiency cause?

  Decreased RBC production.
• What can nose bleeds, ITP and DIC cause?

  Red blood cell loss.
• What happens to RBCs with sickle cell anemia?

  Red blood cells are destroyed.
• What is pure red cell aplasia (PRCA)?

  A condition where the bone marrow stops producing red blood cells.
• What is immune thrombocytopenic purpura (ITP)?

  The immune system is destroying platelets which causes people to have too little platelets.
• What is disseminated intravascular coagulation (DIC)?

  Blood clotting disorder that causes blood to clot excessively.
• What is autoimmune hemolytic anemia?

  Immune system attacks red blood cells.
• What are signs of low RBC production?

  • Tachycardia
  • Tachypnea
  • Pallor
  • Fatigue
  • Muscle weakness
  • Pica
• What are signs of blood loss?

  • Tachycardia
  • Tachypnea
  • Pallor
  • Fatigue
  • Muscle weakness
  • Cool skin
  • Weak peripheral pulses
  • Low blood pressure
• What are signs of RBC destruction?

  • Tachycardia
  • Pallor
  • Fatigue
  • Headache
  • Dark urine
  • Hepatomegaly
  • Splenomegaly
  • Frontal bossing (protruding forehead)
• What are some clinical manifestations of anemia?

  Pale, weakness, fatigue, confusion, tachycardia, black and tarry stools, history of frequent infections, any bleeding, and family history of hereditary diseases.
• What nutritional findings are there for those with anemia (low RBCs)?

  Known lactose intolerance, inadequate intake of iron, diet history reveals poor iron sources, Pica.
• What labs are done for anemia?

  • CBCD (CBC with differential)
  • Platelets
  • RBC & RBC indices (MCV, MCHC, Reticulocyte count)
  • ANC (Absolute neutrophil count)
• What treatments are there for underlying causes of anemia?

  Packed red blood cells, platelet transfusions, nutritional intervention for deficiencies, rule out major medical diagnoses.
• What is supportive care for anemia?

  IV fluids, oxygen therapy, bed rest.
• What is iron deficiency anemia?

  Inadequate supply of dietary iron.
• What age is most common for iron deficiency anemia?

  Toddlers between 12 - 36 months and during the growth spurt in adolescence.
• How can iron deficiency anemia be prevented?

  Iron-rich diet.
• What part of poor diet can cause iron deficiency anemia?

  Excessive cow's milk leads to decreased iron absorption.
• What fruit can help with iron absorption?

  Citrus fruits.
• What should you know about liquid iron supplements?

  Brush teeth after to minimize staining and administer through a straw to prevent teeth staining.
• When should iron supplements be given?

  Between meals with citrus fruits or juice.
• What causes MCV to be small?

  Iron deficiency anemia.
• What is normal range for MCV?

  80 - 95 fL.
• What is normal range for hematocrit?

  42 - 52% for males, 37 -47 % for females.
• What is sickle cell anemia?

  Sickle cell anemia is a genetic disorder characterized by abnormal hemoglobin, leading to misshapen red blood cells.
• When do symptoms usually appear for sickle cell patients?

  After the 1st year.
• What age is sickle cell anemia generally asymptomatic?


  Newborns.
• What kind of disorder is sickle cell anemia?

  Autosomal recessive.
• If both parents have the sickle cell trait, the child has a 25% chance of having sickle cell disease.
• Where in the world is sickle cell anemia a survival advantage?

  Areas where malaria is common.
• Where do sickling episodes usually occur in young children?

  Joints.
• What is dactylitis?


  Inflammation of a digit that cause them to swell and become painful.
• What are general clinical manifestations of sickle cell anemia?

  Growth retardation, susceptibility to sepsis, chronic anemia, delayed sexual development.
• What is done for patients who have a sickle cell crisis occurring?

  Hydration, rest, analgesia, blood replacement, antibiotics.
• What kind of procedure can help replace diseased bone marrow with sickle cell patients?

  Bone marrow transplant.
• What is the leading cause of death in young children with sickle cell disease?

  Infection.
• What is a vaso-occlusive crisis?

  Painful episode in sickle cell disease due to ischemia.