Hematological Issues and Disorders

Question 1

Leukemias

Answer 1

a group of malignant hematologic disease in which normal bone marrow elements are replaced by abnormal, poorly differentiated lymphocytes known as blast cells

Question 2

Signs and symptoms of leukemia

Answer 2

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

Question 3

Types of leukemia common in children

Answer 3

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

Question 4

Laboratory and diagnostics of leukemias

Answer 4

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

Question 5

Lead poisoning

Answer 5

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

Question 6

CDC definition of lead poisoning

Answer 6

> 5-10 micrograms/dL

Question 7

Common sources of lead

Answer 7

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

Question 8

Signs/symptoms of lead poisoning

Answer 8

vague: GI symptoms
severe: lethargy, difficult walking, neuropathies
headaches
burtonian lines
ataxia
papilledema

Question 9

Burtonian lines

Answer 9

bluish discoloration of gingival border

Question 10

General assessment for potential lead poisoning

Answer 10

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)

Question 11

Venous blood level concentrations of lead and classes

Answer 11

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

Question 12

Hemophilia A

Answer 12

x-linked recessive
occurs in 1:7000 males
deficiency of factor VIII

Question 13

Female carriers have what mendelian distribution for hemophilia A?

Answer 13

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

Question 14

Sickle cell anemia

Answer 14

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

Question 15

Causes/incidence of sickle cell anemia

Answer 15

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

Question 16

Sickle cell trait symptoms

Answer 16

Hgb AS

usually have no clinical symptoms
may experience painful symptoms under extreme conditions, such as exertion at high altitudes

Question 17

Sickle cell anemia symptoms

Answer 17

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)

Question 18

Physical exam findings of those sickle cell anemia

Answer 18

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)

Question 19

Laboratory and diagnostic findings with sickle cell anemia

Answer 19

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

Question 20

Management of sickle cell

Answer 20

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

Question 21

How to provide support during sickle cell crisis

Answer 21

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

Question 22

Thalassemia

Answer 22

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

Question 23

Causes of thalassemia

Answer 23

second most common cause of microcytic anemia

autosomal recessive genetic disorder

Question 24

Symptoms of thalassemia

Answer 24

varies from asymptomatic to severe sx/s of anemia

pale or bronze color skin
tachycardia
tachypnea
hepatosplenomegaly
frontal bossing

Question 25

Physical exam findings

Answer 25

prenatal dx and newborn screening

infancy: FTT, irritability, splenomegaly, pallor or severe anemia
older child: bony changes, splenomegaly, iron overload d/t multiple transfusions

Question 26

Lab/diagnostic findings of thalassemia

Answer 26

dec hemoglobin
dec MCV (microcytic anemia)
hypochromic RBCs
increased reticulocyte count 
hemoglobin electrophoresis 
beta globin gene mapping
ferritin
total bilirubin

Question 27

Iron deficiency anemia

Answer 27

microcytic, hypochromic anemia due to an overall deficiency of iron

caused by dec iron intake, inc needs, or slow GI blood loss

Question 28

What causes iron deficiency anemia in infancy?

Answer 28

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)

Question 29

What causes iron deficiency anemia in toddlers?

Answer 29

increased reliance on whole milk at the expense of solid foods

Question 30

What cause iron deficiency anemia in adolescents?

Answer 30

dieting practices contribute, especially after menarche in females

Question 31

Symptoms of iron deficiency anemia

Answer 31

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

Question 32

Lab findings of iron deficiency anemia

Answer 32

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

Question 33

How to manage iron deficiency anemia

Answer 33

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)

Question 34

Microcytic/hypochromic anemia (children)

Answer 34

IDA, thalassemia, lead poisoning, G6PD deficiency

Question 35

Normocytic/normochromic anemia

Answer 35

ACD, acute blood loss, early IDA

Question 36

Macrocytic/normochromic anemia (adults)

Answer 36

vit b12 deficiency, folate deficiency, pernicious anemia

Question 37

Mean corpuscular volume (MCV)

Answer 37

average volume and size of individual erythrocytes

microcytic: too small
normocytic: normal (80-100)
macrocytic: too big

Question 38

Mean corpuscular hemoglobin concentration (MCHC)

Answer 38

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

Question 39

Mean corpuscular hemoglobin (MCH)

Answer 39

expression of the average amount and weight of hgb contained in a single erythrocyte–not as useful as MCHC

normal 26-34

Question 40

Red cell distribution width (RCDW)

Answer 40

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

Question 41

Reticulocyte count

Answer 41

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