Hematological Issues and Disorders Flashcards
This means the size of red blood cells?
Mean corpuscular volume (MCV)
< 80 fl (RBC size)
Microcytic
80 to 100 fl (RBC size)
Normocytic
100 fl (RBC size)
Macrocytic
Expression of the average hemoglobin (Hgb) concentration or proportion of each red blood cells (RBCs) occupied by Hgb as a percentage
Mean corpuscular hemoglobin concentration (MCHC)
“____” more accurate measure than mean corpuscular hemoglobin (MCH)
Color
32 to 36%
Normochromic
< 32 %
Hypochromic
> 36% (most texts deny the existence of this state, in that it is impossible for an RBC to be too red)
Hyperchromic
Weight: _____ expression of the average amount and weight of Hgb contained in a single erythrocyte; not as useful
MCH
Normal MCH is ____ to ____
26 to 34 pg
Red cell width is AKA ____ ___ ____ ___
Red Cell Distribution Width (RCDW)
Red cell size variation is?
anisocytosis
Red cell distribution width in iron deficiency anemia is?
increased
Red cell distribution width in Thalassemia is?
normal or slightly increased
Red cell distribution in Anemia of chronic disease is?
normal
Number of new, your RBCs in circulation
Reticulocyte count
The reticulocyte count is expressed as a _____ ( normal is one to two)
percent
Index of bone marrow health and response to anemia is noted in
1) ____ ____ failure
2) Hemorrhage or hemolysis
1) Bone marrow failure
Definition: Conditions caused by various disorders of the red blood cell count, quality of hemoglobin and/or volume of packed red blood cells
Anemias
Classification: Anemias are classified according to ____ _____ ____ (MCV)/and hemoglobin concentration (MCHC).
red blood cell size
Microcytic/hypochromic (Children): IDA, _______, lead poisoning
Thalassemia
Normocytic/normochromic: ACD, ____ ____ ___, early IDA
acute blood loss
Macrocytic/normochromic (Adult): ___ ___ _____, folate deficiency, pernicious anemia
Vitamin B12 deficiency
____ ____ _______
Microcytic, hypochromic anemia due to an overall deficiency of iron
Iron Deficiency Anemia
Caused by decreased iron intake, increased needs, or slow _________ blood loss
gastrointestinal (GI)
In infancy, iron deficiency is due to an inadequate intake of iron (e.g., low iron formula, solely breastfed) or micro hemorrhage from the gut from the early intake of ____ ____ (before the age of 9 months).
whole milk
In toddlers, ___ ____ is often due to increased reliance on whole milk at the expense of solid foods.
iron deficiency
In adolescence, dieting practices contribute to inadequate intake of iron, specifically in girls after _____.
menarche
Severity depends on the degree of ______
- Easy fatigue ability
- Palpitations; shortness of breath 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
anemia
Laboratory/Diagnostic Findings for anemia include:
- Hemoglobin and hematocrit low
- Low MCV (microcytic anemia)
- Low MCHC
- Low RBCs
- Increased (RDW)
- Total iron-binding capacity (TIBC) increased
- Serum ferritin < ___ ug/L
- Low serum Iron
- Reticulocyte count: low in cases of inadequate iron intake; elevated in cases of blood loss
- 30 ug/L
Management iron deficiency anemia:
- Treat with elemental iron ____ to ___mg/kg/day in one to three doses until hemoglobin (Hgb) normalizes
- To replace iron stores: ___ to ___mg/kg/day for four months
- 3 to 6
2. 2 to 3
Definition: A group of hereditary disorders that are characterized by abnormal synthesis of alpha (four genes) and beta (two genes) globin chains.
1. One or more of each gene can be missing.
2. Type is determined by which genes are missing
(alpha vs. beta-thalassemia).
3. Severity depends on the number of genes affected.
Thalassemia
Causes/Incidence: Thalassemia
- The ______ most common cause of microcytic anemia
- The autosomal recessive genetic disorder
second
Symptoms Varies from asymptomatic to severe signs/symptoms of anemia 1. Pale or bronze color to the skin 2. Tachycardia 3. Tachypnea 4. Hepatosplenomegaly 5. Frontal bossing
Thalassemia
Physical Exam Findings
- Prenatal diagnosis
- Newborn screening
- Infancy
a. FTT/irritability
b. _________
c. Pallor/severe anemia - Older child
a. Bony changes
b. Splenomegaly
c. Iron overload due to multiple transfusions
- b. Splenomegaly
Laboratory/Diagnostic Findings/ _____ anemia
- CBC
a. decreased HGB
b. decreased MCV (microcytic anemia)
c. Hypochromic RBCs - Increased Reticulocyte count
- Hemoglobin electrophoresis
- Beta globin gene mapping
- Ferritin
- Total bilirubin
Thalassemia
Management of Thalassemia
Referral to hematologist
_____________ is anemia in which abnormal hemoglobin (due to DNA point mutation) leads to chronic hemolytic anemia and results in a variety of severe clinical consequences. The peak incidence of infection is between ages one and three years of age.
Sickle cell anemia
Causes/Incidence: Sickle Cell Anemia
1. Autosomal recessive disorder in which Hgb ___ develops instead of Hgb A. The patient is homozygous for Hgb S (Hgb SS)
- S
- Most prevalent in persons of African or African-American ancestry; Hgb S gene is carried in 8% of African-Americans: Incidence is ___ in 400.
- one
- Patients who have heterozygous genotype (Hgb AS) are generally clinically asymptomatic (unless they become _____) but are carriers.
hypoxic
_____________
a. Patients usually have no clinical symptoms
b. May experience acute painful symptoms under
extreme conditions such as exertion at high altitudes
Sickle cell trait (Hgb AS)
_____________
a. Sudden, excruciating pain due to a vaso-occlusive
crisis usually in the back, chest, abdomen and long
bones
b. Low-grade fever
c. Predisposing factors may be present (e.g.,
infection, physical or emotional stress, blood loss).
Sickle cell anemia (Hgb SS)
___ ___ ____
a. These patients are clinically normal with no abnormal exam findings.
Sickle cell trait
___ ____ _____
a. Chronically ill in appearance
b. Jaundice
c. Retinopathy
d. Delayed puberty
e. Hepatosplenomegaly (spleen usually not palpable in
the adult)
f. Enlarged heart with hyperdynamic precordium
g. Systolic murmur
h. Fatigue
i. The tendency toward more frequent infections (which also aggravates crisis) Laboratory/Diagnostic Findings
Sickle cell anemia
Laboratory/ Diagnostic Findings for Sickle cell anemia:
- Hematocrit 20 to 30%
- Irreversibly sickled cells on a peripheral blood smear (five to 50% of total)
- Nucleated red blood cells (immature red cells)
- Reticulocytosis (10 to 25%; immature red cells without nuclei)
- Target cells (abnormal RBCs)
- Howell-Jolly bodies (asplenic conditions)
- White blood cells (WBC) characteristically elevated to 12,000 to 15,000
- Indirect bilirubin elevated
- Platelets may be elevated (> _______).
- Hemoglobin electrophoresis makes the diagnosis.
- Children with sickle cell trait may have episodes of gross hematuria and inability to concentrate the l~rine due to renal tubular defect.
- > 400,000
Management Sickle Cell anemia:
- Collaboration with hematologist
- Maintained chronically on folic acid supplementation
- The cornerstone of therapy supports during the crisis.
a. Keep the client adequately hydrated
b. Ensure adequate oxygenation
c. Analgesics for pain control
d. Antibiotics for associated infection
e. Transfusions and/or exchange transfusions for intractable crisis and as a preventative measure for clients undergoing anesthesia - Hydroxyurea ___ mg/kg/day to stimulate fetal hemoglobin (Hgb F), which does not sickle
- Immunize with Pneumovax (PS-23) and confirm hepatitis B immunity.
- Provide or refer to genetic counseling
- 35 mg/kg
_____ A (X-linked Recessive)
Hemophilia
Hemophilia A (X-linked Recessive)
Occurs in 1: 7,000 _____
Deficiency of factor VIII
Female carriers (Mendelian distribution) have a:
a. ___ _____% percent risk of having an affected son
with each pregnancy
b. Twenty-five percent risk of having a carrier daughter
c. Twenty-five percent chance of having a healthy, non-carrier daughter or son Frequency of carrier females is about 1: 3,500
Severe form occurs in about 48% of cases.
Typical findings
a. Phenotypically normal at birth
b. Bleeding tendency: Ranges from spontaneous
bleeding to bleeding after trauma
males
a. Twenty-five
Lead poisoning is a chronic disease as the result of toxic accumulation of lead in the body that leads to iron deficiency anemia (IDA). The Centers for Disease Control and Prevention (CDC) definition of lead poisoning is a level > ____ug/dL; it can occur via ingestion or inhalation. The highest prevalence is among poor, inner-city children, and those living in old housing.
> 10 ug/dL
Common Sources lead poisoning is:
- Paint and ____ dust (houses prior to 1978: mandated laws require lead-free paint!) 2. Contaminated soil
- Gasoline emissions
- Food and drinking water
- Mexican-American, Asian, Indian, or other ethnic folk remedies
paint
Signs/Symptoms of lead poisoning include:
1. Vague: GI symptoms 2. Severe: \_\_\_\_\_, difficult walking, neuropathies 3. Headaches 4. Burtonian lines: Bluish discoloration of gingival border 5. Ataxia 6. Papilledema
Lethargy
General Assessment for lead poisoning:
1. Medical and developmental history (mouthing and pica history)
2. Environmental history
a. Paint and soil exposure
b. Residence: Year (< ___) and condition
c. Outside play
d. Control of dust and dirt
e. Family members behaviors, occupations
f. Family members hobbies (fishing, ceramic work, stain
glass, and hunting)
g. Exposure to imported food, folk remedies, pottery,
metal vessels
3. Nutritional history
a. Diet history
b. Evaluate the child’s iron status by using the
appropriate laboratory tests
c. Ask about the history of food stamps or participation
in the Special Supplemental Nutrition Program for
Women, Infants, and Children (WlC)
4. Physical exam
a. Pay particular attention to the neurologic examination and the child’s psychosocial and language development
b 1978
Laboratory/Diagnostics (Venous Blood Level Concentrations) Lead poisoning:
Class I: Level < ______
10 ug/dL
Laboratory/Diagnostics (Venous Blood Level Concentrations) Lead poisoning:
Class IIA: Level ____ (referral to hematologist)
10 to 14 ug/dL
Laboratory/Diagnostics (Venous Blood Level Concentrations) Lead poisoning
Class llB: Level _____
15 to 19 ug/dL
Laboratory/Diagnostics (Venous Blood Level Concentrations) Lead poisoning
Class III: Level ______
20 to 44 ug/dL
Laboratory/Diagnostics (Venous Blood Level Concentrations) Lead poisoning
Class IV: Level ______ (recommend chelation therapy)
45 to 69 ug/dL
Laboratory/Diagnostics (Venous Blood Level Concentrations) Lead poisoning
Class V: Level > ____ (hospitalize for chelation, hydration, and close observation)
70ug/dL
Management/ Lead poisoning:
1. Observe for hemoglobinopathies, impaired ___ ____, or vitamin D deficiencies.
renal function
_________
Definition: A group of malignant hematological diseases in which normal bone marrow elements are replaced by abnormal, poorly differentiated lymphocytes known as blast cells.
Leukemias
____ _____ ____accounts for about
75% of cases, a peak incidence around four years of
age; it occurs more frequently in boys than girls and is
more common among Caucasian children.
Acute lymphocytic leukemia (ALL)
____ _____ ____ accounts for
about 20% of all leukemia and occurs primarily in
infants and older children.
Acute myelogenous leukemia (AML)
Signs/Symptoms Leukemia: I. Anemia 2. \_\_\_\_ 3. Listless 4. Irritable 5. Chronically tired 6. History of repeated infections 7. Bleeding such as epistaxis, petechia, and hematomas 8. Lymphadenopathy and hepatosplenomegaly 9. Bone and \_\_\_\_ \_\_\_\_
- Pale
9. joint pain
Laboratory/Diagnostics Leukemia:
- ____ ___ ____ with differential WBC, platelet, and reticulocyte counts (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 the poorly differentiated blast cells that have been replacing the healthy bone marrow tissue.
- Complete blood count (CBC)
Management Leukemia:
- Referral to an _____
- Family support
- oncologist
