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Heme Onc pharm > Pediatric WBC/Neutropenia > Flashcards

Pediatric WBC/Neutropenia Flashcards

1
Q

What is the order of WBC #? Remember the pneumonic

A

Neutrophils > lymphocytes > monocytes > eosinophils > basophils

Never let monkeys eat bananas

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2
Q

What is the formula for ANC

A

(%neutrophils + %bands) x WBC / 100

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3
Q

What is the definition of neutropenia?
Mild vs moderate vs severe
What is assoc w/ severe neutropenia

A

Neutropenia is ANC <1500
Mild 1000-1500, moderate 500-1000, and severe <500
Severe neutropenia is assoc w/ inc risk of severe infection

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4
Q

What is Kostmann syndrome?

Does it inc the risk of malignancy and if so what type?

A

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

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5
Q

What is cyclic neutropenia?

Does it inc risk of malignancy and if so what type?

A

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

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6
Q

What is Shwachman Diamond syndrome?

Does it inc risk of any malignancy and if so what type?

A

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

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7
Q

What is Fanconi Anemia?

When does it present in life? What cells does it affect?

A

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

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8
Q

What is Leukocyte Adhesion Deficiency?

A

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

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9
Q

What is Chronic Granulomatous Disease?

Whats the prognosis? How common/rare is it?

A

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

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10
Q

What is infection assoc neutropenia?

Explain B19 parvovirus

A

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

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11
Q

What are 2 additional causes of pediatric neutropenia?

A

Cytotoxic agents and b12 deficiency

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12
Q

What is leukocytosis

What are 3 additional assoc with it?

A

Leukocytosis is reactive process to infection that subsides w/ resolution of infection
Chronic inflammation- autoimmune disorders
Oncogenic processes- leukemia, lymphoma, neuroblastoma
Seizures

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13
Q

What is the most common general cancer type affect adolescents?

A

Lymphomas

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14
Q

What is the most common cancer affecting 15-19 yo?

A

Hodgkins lymphoma

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15
Q

What is a pathognomoic morph feature of HL?

A

Reed sternberg cells

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16
Q

What type of symptoms is HL assoc w/ and what are they?

A

HL assoc w/ B symptoms

Unexplained fever over 39, night sweats, and wt loss >10% of body mass in past 6 months

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17
Q

What is general rule for diagnosis of HL w/ regards to when you should get a LN biopsy?

A

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

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18
Q

NHL accounts for what % of lymphomas affecting children and adolescents?

A

60%

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19
Q

A small % of NHL is assoc w/ acquired/inherited immunodeficiencies, what are they and explain

A

SCID
X linked Wiscott syndrome characterized by recurrent sino-pulm and ear infections, severe atopic dermatitis, and bleeding secondary to thrombocytopenia

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20
Q

Burkitts Lymphoma
Where does sporadic form arise?
Where does endemic form arise and w/ what else involved?

A

Sporadic BL- arises as abdominal mass

Endemic BL- arises as head/neck mass w/ CNS and BM involvement

21
Q

NHL Prognosis
Localized
Advanced
What does prognosis depend on

A

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

22
Q

What are the 2 most common types of cancers affecting children?

A

Leukemias and brain tumor

23
Q

Explain how leukemia arises?

A

Leukemias arise due to malignant transformation and clonal expansion of hematopoietic cell at early stage of differentiation that is unable to undergo maturation

24
Q

What are the 2 general classes of leukemias? Think general WBC neoplasm classes

A

Lymphoid and myeloid

25
Q

What % of childhood leukemias are acute or chronic and what are the 2 major examples

A

More than 97% of childhood leukemias are acute; most commonly ALL or AML

26
Q

What common genetic disorder inc the risk of childhood leukemia?

A

Trisomy 21 inc risk of childhood leukemia

27
Q

With anemia what happens to [2,3-DPG]? What does that do to the O2 dissociation curve and O2 binding affinity?

A

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

28
Q

What is MCV?

How can it be used to classify anemias?

A

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)

29
Q

Whats MCHC?

How it is used to classify anemias?

A

MCHC
Mean corpuscular Hb concentration- grams of Hb per 100 mL of RBC

MCHC low (hypochromatic), MCHC nml (normo), MCHC high (hyperchromatic)

30
Q

What is MCH?

A

MCH presents average amount of Hb per RBC

31
Q

Whats RDW?

A

RDW

RBC distribution width- measure of anisocytosis

32
Q

What does a peripheral smear allow us to evaluate w/ regards to RBC?

A

We can use peripheral smears to evaluate RBC morph change

33
Q

Dec RBC production in marrow is due to what 2 causes?

A

Dec RBC production in marrow is due to ineffective hematopoiesis/MDS or marrow/erythropoeisis failure

34
Q

Intravascular hemolysis results in what?

A

Anemia, hemoglobinemia, hemoglobinuria, hemosiderinuria, jaundice, dec haptoglobin, hemosiderosis due to free iron, and free Hb is oxidized to methamoglobin and excreted causing dark urine

35
Q

Extravascular hemolysis causes what?

A

Anemia splenomegaly and jaundice

36
Q

Blood loss anemia is classified in what two categories?

A

Acute and chronic blood loss anemia

37
Q

What does a low reticulocyte count indicated during anemia?

A

Low reticulocyte count indicates inadequate marrow response to anemia due to ineffective hematopoiesis or due to marrow failure

38
Q

What does a high reticulocyte count indicate during anemia? What causes the anemia?

A

High reticulocyte count represents a nml marrow response to anemia meaning the anemia is do to blood loss, hemolysis, or due to splenic sequestration

39
Q

What is the physiologic nader

A

At about 2 months old fetal Hb and Hct will dec after initially being elevated very high before rising to a nml value

40
Q

What are the 5 neonatal causes of anemia?

A

Isoimmunization, congenital hemolytic anemia, B19 parvovirus, shwachmann diamond syndrome, and fanconi anemia

41
Q

Explain neonatal anemia due to isoimmunization

A

Isoimmunization occurs due to ABO incompatability and results in positive direct coombs test, elevated unconjugated bilirubin, elevated reticulocyte, and normocytic anemia

42
Q

Explain neonatal anemia due to congenital hemolytic anemia

A

G6PD Deficiency- Heinz bodies and bite cells

Pyruvate Kinase Deficiency- spur cells

43
Q

Explain B19 parvovirus w/ regards to neonatal anemia

A

Normocytic w/ low reticulocyte count

44
Q

Explain diamond syndrome w/ regards to neonatal anemia

A

diamond syndrome causes congenital pure RBC aplasia leading to macrocytic and low reticulocyte

45
Q

Explain fanconi anemia w/ regards to neonatal anemia?

A

Macrocytic, reticulocytopenia, and pancytopenia

46
Q

What are the 3 causes of infancy anemia

A

Iron deficiency
Defective Hb structure or synthesis
Lead Poisoning

47
Q

Explain iron deficiency w/ regards to infancy anemia

A

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

48
Q

Explain defective Hb structure/synthesis w/ regards to infancy anemia?

A

Microcytic, dec RDW, dec Mentzer index in thallassemias, basophilic stippling, hemolysis, and reticulocytosis

49
Q

Explain lead poisoning w/ regards to infancy anemia?

A

Microcytic w/ basophilic stippling

Risks- young age, house built before 1970, contaminated soil, and pica

Heme Onc pharm (5 decks)

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