Hematologic Dysfunction Flashcards
Iron deficiency anemia
Inadequate supply of iron causes smaller RBCs, depleted RBC mass, decreased hemoglobin, decreased oxygen carrying capacity
Prominent with rapid growth
Iron deficiency anemia signs
Pallor, fatigue, PICA, headaches, dizziness/lightheadedness, irritability, slowed thought process, decreased attention span, apathy, depression
Iron deficiency anemia treatment
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
Sickle cell disease
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
Sickle cell disease signs
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)
Sickle cell disease complications
Vaso-occlusive crisis, splenic sequestration, aplastic crisis, stroke, acute chest syndrome, overwhelming infections
Sickle cell disease care
Prophylactic antibiotic (penicillin), pain management, hydration, electrolyte replacement, oxygen, transfusions No cold application - can cause vasoconstriction
Aplastic anemia
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
Fanconi syndrome
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
Blackfan-Diamond syndrome
Hypoplastic anemia, rare condition characterized by destruction of RBCs and a slight decrease in WBCs and platelets
Causes of aplastic anemia
Radiation, chemotherapy, sulfonamides, methcillin, chloramphenicol, thiazides, household chemicals, infections, leukemia, lymphoma, SCD, autoimmune or allergy states
Aplastic anemia signs
Lack of RBCs = pallor, lethargy, tachycardia, SOB
Lack of WBCs = recurrent infections
Lack of platelets = abnormal bleeding, petechiae, bruising
Aplastic anemia treatment
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
Thalassemia
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
Thalassemia signs
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
Signs of iron overload
Abdominal pain, bloody diarrhea, emesis, decreased LOC, shock, metabolic acidosis
Thalassemia treatment
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
Hemophilia
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
Hemophilia A (classic hemophilia)
Factor VIII deficiency - necessary for formation of thromboplastin
Hemophilia B (Christmas disease)
Factor IX deficiency - impaired ability to form fibrin clot
Hemophilia signs
Excessive bleeding after cutting umbilical cord
Easy bruising, prolonged bleeding, hematuria, epistaxis, hemarthrosis (hemorrhage in joints)
Hemarthrosis treatment
Immobilize, ice, replacement factor
Hemophilia complications
Bone changes, intracranial bleeding, GI hemorrhage, hematoma, airway obstruction
Hemophilia treatment
Desmopressin - helps release factor VIII Safety - bleeding precautions Soft toothbrush Monitor for CNS manifestations Avoid aspirin, blood thinners
