Pediatric WBC/Neutropenia Flashcards
What is the order of WBC #? Remember the pneumonic
Neutrophils > lymphocytes > monocytes > eosinophils > basophils
Never let monkeys eat bananas
What is the formula for ANC
(%neutrophils + %bands) x WBC / 100
What is the definition of neutropenia?
Mild vs moderate vs severe
What is assoc w/ severe neutropenia
Neutropenia is ANC <1500
Mild 1000-1500, moderate 500-1000, and severe <500
Severe neutropenia is assoc w/ inc risk of severe infection
What is Kostmann syndrome?
Does it inc the risk of malignancy and if so what type?
Kostmann syndrome is autorecessive severe congenital neutropenia leading to life threatening pyogenic infections in infants due to impaired myeloid differentiation leading to maturation arrest of neutrophilic precursors
Inc risk of AML
What is cyclic neutropenia?
Does it inc risk of malignancy and if so what type?
Cyclic neutropenia is sporadic or autodom disorder characterized by cyclic fever, oral ulcers, gingivitis, periodontal diseae, and recurrent bacterial infections due to defects in stem cells leading to defective maturation
Does not inc risk of malignancies
What is Shwachman Diamond syndrome?
Does it inc risk of any malignancy and if so what type?
Schwachman diamond syndrome is autorecessive disorder characterized by triad of neutropenia, pancreatic insufficiency, and skeletal abnml due to defective neutrophil mobility, migration, and chemotaxis
SD syndrome inc risk of MDS and leukemia
What is Fanconi Anemia?
When does it present in life? What cells does it affect?
Fanconi anemia is autorecessive bone marrow failure characterized by GU and skeletal abnml and chrom fragility
Presents in first decade of life and affects all cell lineages
What is Leukocyte Adhesion Deficiency?
LAD is rare autorecessive disorder characterized by delayed separation of umbilical cord for >3 weeks and recurrent severe bacterial/fungal infections w/o pus due to dec neutrophil adhesion
What is Chronic Granulomatous Disease?
Whats the prognosis? How common/rare is it?
Chronic granulomatous disease is characterized by recurrent purulent infection by catalase + orgs in infants leading to chronic inflammatory granulomas due to defects in oxidative metabolism leading to absence of superoxide
Good prognosis; affects 1 in 250,000
What is infection assoc neutropenia?
Explain B19 parvovirus
Infection assoc neutropenia- viral infections lead to neutropenia w/in 3 days and lasts about 1 week
B19 parvovirus causes 5ths disease characterized by slapped check appearance that causes neutropenia 1-2 weeks post infection
What are 2 additional causes of pediatric neutropenia?
Cytotoxic agents and b12 deficiency
What is leukocytosis
What are 3 additional assoc with it?
Leukocytosis is reactive process to infection that subsides w/ resolution of infection
Chronic inflammation- autoimmune disorders
Oncogenic processes- leukemia, lymphoma, neuroblastoma
Seizures
What is the most common general cancer type affect adolescents?
Lymphomas
What is the most common cancer affecting 15-19 yo?
Hodgkins lymphoma
What is a pathognomoic morph feature of HL?
Reed sternberg cells
What type of symptoms is HL assoc w/ and what are they?
HL assoc w/ B symptoms
Unexplained fever over 39, night sweats, and wt loss >10% of body mass in past 6 months
What is general rule for diagnosis of HL w/ regards to when you should get a LN biopsy?
Any pt w/ unexplained persistent LAD that is not related to inflammatory or infectious processes should undergo a chest radiograph to rule out mediastinal mass prior to having a LN biopsy
NHL accounts for what % of lymphomas affecting children and adolescents?
60%
A small % of NHL is assoc w/ acquired/inherited immunodeficiencies, what are they and explain
SCID
X linked Wiscott syndrome characterized by recurrent sino-pulm and ear infections, severe atopic dermatitis, and bleeding secondary to thrombocytopenia
Burkitts Lymphoma
Where does sporadic form arise?
Where does endemic form arise and w/ what else involved?
Sporadic BL- arises as abdominal mass
Endemic BL- arises as head/neck mass w/ CNS and BM involvement
NHL Prognosis
Localized
Advanced
What does prognosis depend on
Localized NHL- >90% survivial
Advanced NHL- 60-95% survival
Prognosis of NHL depends on patho subtype, tumor burden at time of diagnosis based off LDH levels, sites of spread, and whether or not there is CNS disease along with it
What are the 2 most common types of cancers affecting children?
Leukemias and brain tumor
Explain how leukemia arises?
Leukemias arise due to malignant transformation and clonal expansion of hematopoietic cell at early stage of differentiation that is unable to undergo maturation
What are the 2 general classes of leukemias? Think general WBC neoplasm classes
Lymphoid and myeloid
What % of childhood leukemias are acute or chronic and what are the 2 major examples
More than 97% of childhood leukemias are acute; most commonly ALL or AML
What common genetic disorder inc the risk of childhood leukemia?
Trisomy 21 inc risk of childhood leukemia
With anemia what happens to [2,3-DPG]? What does that do to the O2 dissociation curve and O2 binding affinity?
With anemia 2,3-DPG concentration inc in RBC causing a right shift in O2 dissoc curve which causes Hb to have dec binding affinity to O2
What is MCV?
How can it be used to classify anemias?
MCV
Mean corpuscle vol- represents the mean value of the vol of individual RBC in a sample
MCV low (microcytic), MCV nnml (normocytic), MCV high (macrocytic)
Whats MCHC?
How it is used to classify anemias?
MCHC
Mean corpuscular Hb concentration- grams of Hb per 100 mL of RBC
MCHC low (hypochromatic), MCHC nml (normo), MCHC high (hyperchromatic)
What is MCH?
MCH presents average amount of Hb per RBC
Whats RDW?
RDW
RBC distribution width- measure of anisocytosis
What does a peripheral smear allow us to evaluate w/ regards to RBC?
We can use peripheral smears to evaluate RBC morph change
Dec RBC production in marrow is due to what 2 causes?
Dec RBC production in marrow is due to ineffective hematopoiesis/MDS or marrow/erythropoeisis failure
Intravascular hemolysis results in what?
Anemia, hemoglobinemia, hemoglobinuria, hemosiderinuria, jaundice, dec haptoglobin, hemosiderosis due to free iron, and free Hb is oxidized to methamoglobin and excreted causing dark urine
Extravascular hemolysis causes what?
Anemia splenomegaly and jaundice
Blood loss anemia is classified in what two categories?
Acute and chronic blood loss anemia
What does a low reticulocyte count indicated during anemia?
Low reticulocyte count indicates inadequate marrow response to anemia due to ineffective hematopoiesis or due to marrow failure
What does a high reticulocyte count indicate during anemia? What causes the anemia?
High reticulocyte count represents a nml marrow response to anemia meaning the anemia is do to blood loss, hemolysis, or due to splenic sequestration
What is the physiologic nader
At about 2 months old fetal Hb and Hct will dec after initially being elevated very high before rising to a nml value
What are the 5 neonatal causes of anemia?
Isoimmunization, congenital hemolytic anemia, B19 parvovirus, shwachmann diamond syndrome, and fanconi anemia
Explain neonatal anemia due to isoimmunization
Isoimmunization occurs due to ABO incompatability and results in positive direct coombs test, elevated unconjugated bilirubin, elevated reticulocyte, and normocytic anemia
Explain neonatal anemia due to congenital hemolytic anemia
G6PD Deficiency- Heinz bodies and bite cells
Pyruvate Kinase Deficiency- spur cells
Explain B19 parvovirus w/ regards to neonatal anemia
Normocytic w/ low reticulocyte count
Explain diamond syndrome w/ regards to neonatal anemia
diamond syndrome causes congenital pure RBC aplasia leading to macrocytic and low reticulocyte
Explain fanconi anemia w/ regards to neonatal anemia?
Macrocytic, reticulocytopenia, and pancytopenia
What are the 3 causes of infancy anemia
Iron deficiency
Defective Hb structure or synthesis
Lead Poisoning
Explain iron deficiency w/ regards to infancy anemia
Iron anemia causes microcytic hypochromatic anemia w/ low iron/ferratin and high transferrin
Due to low dietary iron (milk rich diet) or chronic occult blood loss
Explain defective Hb structure/synthesis w/ regards to infancy anemia?
Microcytic, dec RDW, dec Mentzer index in thallassemias, basophilic stippling, hemolysis, and reticulocytosis
Explain lead poisoning w/ regards to infancy anemia?
Microcytic w/ basophilic stippling
Risks- young age, house built before 1970, contaminated soil, and pica
