Hematologic Dysfunction

Question 1

Iron deficiency anemia

Answer 1

Inadequate supply of iron causes smaller RBCs, depleted RBC mass, decreased hemoglobin, decreased oxygen carrying capacity
Prominent with rapid growth

Question 2

Iron deficiency anemia signs

Answer 2

Pallor, fatigue, PICA, headaches, dizziness/lightheadedness, irritability, slowed thought process, decreased attention span, apathy, depression

Question 3

Iron deficiency anemia treatment

Answer 3

Promote iron-fortified formula and cereals, lean meat, fish, legumes, dried fruits, potatoes, dark leafy green vegetables, beans, whole-grain bread
Give with small amount of vitamin C to enhance absorption - brush teeth after administering
Avoid vitamin A/E - causes N/V, diarrhea, constipation, more toxic when overdosed

Question 4

Sickle cell disease

Answer 4

Chronic, genetic hemolytic disease
Under conditions of increased oxygen tension and lowered pH, RBCs sickle - causes clumping of cells, thrombosis, arterial obstruction, increased blood viscosity, hemolysis, tissue ischemia/necrosis

Question 5

Sickle cell disease signs

Answer 5

Enlarged spleen (congestion caused by sickled cells), enlarged and tender liver from blood stasis, hematuria, inability to concentrate urine, enuresis, nephrotic syndrome, bone weakness, dactylitis (inflammation of finger and toe joints)

Question 6

Sickle cell disease complications

Answer 6

Vaso-occlusive crisis, splenic sequestration, aplastic crisis, stroke, acute chest syndrome, overwhelming infections

Question 7

Sickle cell disease care

Answer 7

Prophylactic antibiotic (penicillin), pain management, hydration, electrolyte replacement, oxygen, transfusions
No cold application - can cause vasoconstriction

Question 8

Aplastic anemia

Answer 8

Characterized by pancytopenia (anemia, granulocytopenia, thrombocytopenia) and bone marrow hypoplasia
Decreased functional capacity of hypoplastic bone marrow results in pancytopenia - can cause bleeding or infection

Question 9

Fanconi syndrome

Answer 9

Autosomal recessive trait associated with cytopenia and multiple congenital anomalies including short stature, malformed kidneys and heart, microcephaly, microphthalmus, cafe-au-lait spots, absent radii and thumbs
Aplastic anemia

Question 10

Blackfan-Diamond syndrome

Answer 10

Hypoplastic anemia, rare condition characterized by destruction of RBCs and a slight decrease in WBCs and platelets

Question 11

Causes of aplastic anemia

Answer 11

Radiation, chemotherapy, sulfonamides, methcillin, chloramphenicol, thiazides, household chemicals, infections, leukemia, lymphoma, SCD, autoimmune or allergy states

Question 12

Aplastic anemia signs

Answer 12

Lack of RBCs = pallor, lethargy, tachycardia, SOB
Lack of WBCs = recurrent infections
Lack of platelets = abnormal bleeding, petechiae, bruising

Question 13

Aplastic anemia treatment

Answer 13

Prevent infection 
Monitor for bleeding
Administer blood products 
Androgen therapy
Anti-lymphocyte globulin (ALG) or anti-thymocyte globulin (ATG therapy) - suppresses immune system
Bone marrow transplant
Eliminate toxic agents

Question 14

Thalassemia

Answer 14

Autosomal recessive disorder
Defect in the production of specific globulin chains in hemoglobin results in compensatory increase in hemoglobin production among other hemoglobin chains which then become unbalanced, disintegrate, and destroy RBCs

Question 15

Thalassemia signs

Answer 15

Early - anemia, unexplained fever, poor feeding/weight gain, enlarged spleen
Late - chronic hypoxia, damage to liver, spleen, heart, pancreas, and lymph glands from hemochromatosis (too much iron) - jaundice, thick cranial bones, growth retardation, skeletal complications, gallbladder disease, skin changes, delayed sexual development

Question 16

Signs of iron overload

Answer 16

Abdominal pain, bloody diarrhea, emesis, decreased LOC, shock, metabolic acidosis

Question 17

Thalassemia treatment

Answer 17

Desferal, deferasirox - eliminates excess iron, can be ototoxic, oculartoxic, nephrotoxic - may turn urine red
Splenectomy - prophylactic antibiotics, avoid people with infections
Avoid certain activities - at risk for fractures

Question 18

Hemophilia

Answer 18

X-linked recessive disorder, bleeding disorder characterized by deficiency in a blood clotting factor
Hemophilia A and B
Mild - prolonged bleeding only when injured
Moderate - prolonged bleeding with trauma and surgery, may have spontaneous bleeding
Severe - bleeding can occur spontaneously

Question 19

Hemophilia A (classic hemophilia)

Answer 19

Factor VIII deficiency - necessary for formation of thromboplastin

Question 20

Hemophilia B (Christmas disease)

Answer 20

Factor IX deficiency - impaired ability to form fibrin clot

Question 21

Hemophilia signs

Answer 21

Excessive bleeding after cutting umbilical cord

Easy bruising, prolonged bleeding, hematuria, epistaxis, hemarthrosis (hemorrhage in joints)

Question 22

Hemarthrosis treatment

Answer 22

Immobilize, ice, replacement factor

Question 23

Hemophilia complications

Answer 23

Bone changes, intracranial bleeding, GI hemorrhage, hematoma, airway obstruction

Question 24

Hemophilia treatment

Answer 24

Desmopressin - helps release factor VIII
Safety - bleeding precautions 
Soft toothbrush
Monitor for CNS manifestations 
Avoid aspirin, blood thinners

Question 25

Immune thrombocytopenic purpura

Answer 25

Acquired hemorrhagic disorder in which the number of circulating platelets is reduced - <20,000
May be caused by autoimmune system - RI, measles, chicken pox, fifth disease
Can be mistaken for child abuse - need to rule out

Question 26

Immune thrombocytopenic purpura signs

Answer 26

Easy bruising, petechiae, bleeding from mucous membranes, blood in stool/urine, hematoma, low platelets

Question 27

Immune thrombocytopenic purpura treatment

Answer 27

Minimize bleeding

Steroids, IVIG, anti-D antibody

Question 28

Anti-D antibody pros

Answer 28

Alleviates need for bone marrow aspiration to rule out leukemia for prednisone administration
Less expensive than IVIG