Hematological Issues and Disorders Flashcards
Leukemias
a group of malignant hematologic disease in which normal bone marrow elements are replaced by abnormal, poorly differentiated lymphocytes known as blast cells
Signs and symptoms of leukemia
anemia (dec RBC) pale listless irritable chronically tired history of repeated infections (inc/dec WBC) bleeding such as epistaxis, petechia, and hematomas (dec platelets) lymphadenopathy and hepatosplenomegaly bone and joint pain
Types of leukemia common in children
ALL: accounts for 75% of cases, a peak incidence around 4 years of age–more common in boys than girls and more common in caucasian children
AML: 20% of of all leukemia and occurs primarily in infants and older children
Laboratory and diagnostics of leukemias
CBC (thrombocytopenia is present in up to 85% of cases and anemia is usually present)
peripheral smear may demonstrate malignant cells (blasts)
bone marrow will show poorly differentiated blast cells that have been replacing the healthy bone marrow tissue
Lead poisoning
toxic levels of lead in the body that can lead to iron deficient anemia with the highest prevalence among poor, inner-city children and those living in old housing
CDC definition of lead poisoning
> 5-10 micrograms/dL
Common sources of lead
paint and paint dust (houses built before 1978)
contaminated soil
gasoline emissions
food and drinking water
mexican-american, asian, indian, or other ethnic folk remedies
Signs/symptoms of lead poisoning
vague: GI symptoms
severe: lethargy, difficult walking, neuropathies
headaches
burtonian lines
ataxia
papilledema
Burtonian lines
bluish discoloration of gingival border
General assessment for potential lead poisoning
medical and dev history (pica?)
environmental history (paint/soil exposure, outside play, family members behaviors, occupations, hobbies, exposure to imported food and pottery)
nutritional history–evaluate iron status by labs
physical exam (neurologic exam and child’s psychosocial and language dev)
Venous blood level concentrations of lead and classes
class I: = 10 class IIA: 10-14 (refer to hematologist) class IIB: level 15-19 class III: level 20-44 class IV: level 45-69 (recommend chelation therapy) class V: level >70 (hospitalize)
**observe for hemoglobinopathies, impaired renal function, vit D deficiencies
Hemophilia A
x-linked recessive
occurs in 1:7000 males
deficiency of factor VIII
Female carriers have what mendelian distribution for hemophilia A?
25% risk of having an affected son with each pregnancy
25% risk of having a carrier daughter
25% chance of heaving a healthy, non carrier daughter or son
*female carriers 1:3,500
Sickle cell anemia
vaso occlusive condition–abnormal hemoglobin leads to chronic hemolytic anemia and results in a variety of severe consequences
peak incidence of infection is between ages 1-3 yrs of age
Causes/incidence of sickle cell anemia
autosomal recessive disorder in which Hgb S develops instead of Hgb A (pt is homozygous for Hgb S, so Hgb SS)
Most prevalent in African American population
Patients who have heterozygous genotype (Hgb AS) are generally clinically asymptomatic, but are carriers
Sickle cell trait symptoms
Hgb AS
usually have no clinical symptoms
may experience painful symptoms under extreme conditions, such as exertion at high altitudes
Sickle cell anemia symptoms
Hgb SS
sudden, excruciating pain due to a vaso-occlusive crisis usually in the back, best, abdomen and long bones
low grade fever
predisposing factors may be present (infection, physical or emotional stress, blood loss)
Physical exam findings of those sickle cell anemia
chronically ill in appearance jaundice retinopathy delayed puberty hepatosplenomegaly enlarged heart with hyperdynamic precordium systolic murmur fatigue tendency toward more frequent infections (which then can aggravate crisis)
Laboratory and diagnostic findings with sickle cell anemia
hematocrit 20-30%
irreversibly sickled cells on peripheral blood smear (5-50% of total)
nucleated RBCs (immature red cells)
reticulocytosis (10-25%, immature red cells without nuclei)
target cells (abnormal RBCs)
howell-jolly bodies (asplenic conditions)
WBC increased
Platelets increased
Indirect bilirubin increased
- children with sickle cell trait may have episodes of gross hematuria and inability to concentrate the urine d/t renal tubular defect
**hemoglobin electrophoresis makes diagnosis
Management of sickle cell
collaboration with hematologist
maintained chronically on folic acid supplementation
support during crisis
hydroxyurea to stimulate fetal hemoglobin, which does not sickle
immunize with pneumovax and confirm hep B immunity
How to provide support during sickle cell crisis
adequately hydrated adequate oxygenation analgesics for pain control antibiotics for associated infection transfusions and/or exchange transfusion for intractable crisis and as a preventative measure for clients undergoing anesthesia
Thalassemia
a group of hereditary disorders (dx at birth) that are characterized by abnormal synthesis of alpha (4) and beta (2) globin chains
one or more of each gene can be missing
type is determine by which genes are missing
severity depends on number of genes affected
Causes of thalassemia
second most common cause of microcytic anemia
autosomal recessive genetic disorder
Symptoms of thalassemia
varies from asymptomatic to severe sx/s of anemia
pale or bronze color skin tachycardia tachypnea hepatosplenomegaly frontal bossing
Physical exam findings
prenatal dx and newborn screening
infancy: FTT, irritability, splenomegaly, pallor or severe anemia
older child: bony changes, splenomegaly, iron overload d/t multiple transfusions
Lab/diagnostic findings of thalassemia
dec hemoglobin dec MCV (microcytic anemia) hypochromic RBCs increased reticulocyte count hemoglobin electrophoresis beta globin gene mapping ferritin total bilirubin
Iron deficiency anemia
microcytic, hypochromic anemia due to an overall deficiency of iron
caused by dec iron intake, inc needs, or slow GI blood loss
What causes iron deficiency anemia in infancy?
inadequate intake of iron (sole breastfed or low iron formula) or micro hemorrhage from the gut from early intake of whole milk (before 9 months old)
What causes iron deficiency anemia in toddlers?
increased reliance on whole milk at the expense of solid foods
What cause iron deficiency anemia in adolescents?
dieting practices contribute, especially after menarche in females
Symptoms of iron deficiency anemia
severity depends on the degree of anemia
easy fatigue ability palpitations, SOB on exertion lethargy headaches pica delayed motor development pale, dry skin and mucous membranes tachycardia tachypnea postural hypotension in severe anemia brittle hair flat, brittle or spoon shaped nails
Lab findings of iron deficiency anemia
hemoglobin and hematocrit low
low mcv
low mchc
low rbcs
increased RDW b/c nothing in it, big and flat
total iron binding capacity increased because taking all the iron you can give
serum ferritin
How to manage iron deficiency anemia
correct underlying cause
treat with elemental iron 3-6 mg/kg/day in 1-3 does until hgb normalizes
to replace iron stores: 2-3 mg/kg/day for 4 months (RBC lives for 120 days)
Microcytic/hypochromic anemia (children)
IDA, thalassemia, lead poisoning, G6PD deficiency
Normocytic/normochromic anemia
ACD, acute blood loss, early IDA
Macrocytic/normochromic anemia (adults)
vit b12 deficiency, folate deficiency, pernicious anemia
Mean corpuscular volume (MCV)
average volume and size of individual erythrocytes
microcytic: too small
normocytic: normal (80-100)
macrocytic: too big
Mean corpuscular hemoglobin concentration (MCHC)
expression of the average hemoglobin concentration or proportion of each RBCs occupied by hgb as a percentage–color
normochromic: normal concentration of hgb (32-36%)
hypochromic: less concentration of hgb
hyperchromic: more concentration of hgb
Mean corpuscular hemoglobin (MCH)
expression of the average amount and weight of hgb contained in a single erythrocyte–not as useful as MCHC
normal 26-34
Red cell distribution width (RCDW)
red cell size variation (anisocytosis)
differentiates between iron deficiency anemia, thalassemia, and anemia of chronic disease
IDA: increased
Thalassemia: normal or slightly increased
ACD: normal
Reticulocyte count
normal is 1-2%
number of new, young RBCs in circulation (baby RBCs bc body is saying need more RBC, so put baby RBCs to work)
- if reticulocyte count is high, then it shows body is making more
