Pediatric anemia and wbc - Newman Flashcards

1
Q

What common virus causes neutropenia?

What other viruses?

A

Parvovirus B19

HIV, EBV, CMV

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3
Q

Clinical manifestation of Kostmann syndrome?

Inheritance?

Severity?

What mutation?
At risk for what?

A

Life threatening pyogenic infections in infancy

AR

< 200 neutrophils since birth, anemia, mild mono/eosinophils

ELA-2 mutated
Inc risk for AML in kids

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5
Q

Tortuosity of conjuctival and retinal vessels seen w/what disease?

What other eye symptom common?

A

Sickle cell

Microaneurysms of retinal vessels

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6
Q

Cytotoxic agents for treatment of malignancies can cause drug-induced neutropenia, what will the major lab finding be?

A

Low ANC

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7
Q

Parietal cells found where?

Make what?

A

Fundus and body of stomach

Intrinsic Factor

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8
Q

Give the order of most to least wbc #s

A
Neuts
Lymphocytes
Monos
Eosin
Basos

Neutrophils like making everything better

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10
Q

ANC = ?

A

(% neuts + %bands) x (wbc) all / by 100

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15
Q

What is included in a CBC?

A
Rbc
Hg, Hct
MCV, MCH, MCHC
Platelets
RDW
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16
Q

What are the 3 red and bolded features of Intravascular hemolysis?

A

Hemoglobinuria
Hemoglobinemia
Hemosiderinuria

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17
Q

Lead poisoning inhibits what?

A

Ferrochelatase
ALA dehydratase
rRNA degradation

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18
Q

Inheritance of Shwachman-Diamond syndrome?

Severity?

Diagnose how?

A

AR

Chronic severe or intermittent, anemia or thrombocytopenia may be present

Low serum Trypsinogen, Inc fecal fat excretion, fucked up skeleton/rib
SBDS mutation

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19
Q

Cataracts seen w/what disease?

A

G6PD deficiency

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20
Q

What is the cause of microcytic anemia and reticulocytopenia, thrombocytopenia, or leukopenia due to DNA sequencing gene mutation?

A

Fanconi anemia

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21
Q

What causes bone marrow failure, GU and skeletal abnormalities, and increased chromosome fragility?

A

Fanconi anemia

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24
Q

CF of cyclic neutropenia?

Defect?

Inheritance?

Severity?

Diagnose?

A

Cyclic fevers, oral ulcers, gingivitis, periodontal disease, recurrent bacterial infections

Stem cell reg defect

AD or sporadic

<200 for 3-7 days every 3 was

CBC, ELA-2 mutation in 80-90%

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25
Q

What disorder has an increased risk for MDS or leukemia?

A

Shwachman-Diamond syndrome

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27
Q

What can cause Fe def anemia?

A
Inadequate diet
Chronic occult blood loss
Too much milk
IBD
Meckel's D
Parasites
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28
Q

Cranial hemorrhages in infants are often associated with what?

What can happen?

A

Birth trauma via vacuum or forceps

Subgaleal bleed

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29
Q

Word for different shapes?

A

Poikilocytosis

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31
Q

If the wbc > 100,000, what should you suspect?

A

Oncologic process and marrow infiltration

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32
Q

Inheritance of sickle cell?

What special cells seen?

A

AR

Target cells
Howell-Jolly bodies

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33
Q

Inheritance of chronic granulomatous disease?

prognosis?

A

X-linked recessive, rarely AR

Good w/aggressive management of infection

34
Q

What is the name for severe congenital neutropenia?

A

Kostmann syndrome

36
Q

Word for different sizes?

A

Anisocytosis

38
Q

What causes delayed separation of the umbilical cord (> 3weeks) w/recurrent bacterial and fungal infections w/out pus forming?

Inheritance?

A

Leukocyte adhesion deficiency

AR

40
Q

How does the oxygen dissociation curve move in response to anemia?

A

Shifts to the right

43
Q

Microcornea seen w/what disease?

A

Fanconi anemia

45
Q

what shows a triad of neutropenia, exocrine pancreas insufficiency, and skeletal abnormalities?

A

Shwachman-Diamond syndrome

47
Q

What are the causes of eosinophilia?

Apparently this laundry is a TQ

A

NAACP does DRUGS

Neoplasia
Asthma
Allergy
Chronic adrenal Insufficiency
Parasites
Drugs
49
Q

+ direct Coombs, Inc indirect bilirubin, normocytic, INC reticulocyte is what?

Examples?

A

Isoimmunization

ABO or Rh incompatibility

51
Q

How does Fe deficiency anemia present (CFs)?

A

Fatigue
Conjuctival pallor
Pica
Spoon nails (koilonychia)

52
Q

What is the most common cause of acquired neutropenia in infancy and childhood?

Exhibit what?

Present when?

A

Primary autoimmune neutropenia

Uri or skin infection

2mo - 3yrs

53
Q

Menzter index > 13 means what?

< 13?

A

Fe deficiency

Thalassemia

55
Q

Heinz bodies, bite cells seen in what?

A

G6PD def

56
Q

What causes recurrent purulent infections w/fungal or bacterial catalase(+) organisms starting in infancy?

Defect in what?

diagnose how?

A

Chronic granulomatous disease

Oxidative metabolism, can’t make superoxide

Nitroblue tetrazolium test

57
Q

What is included in CBC w/diff?

A

CBC

Total WBC

58
Q

Neutropenia defines as what?

Mild?

Moderate?

Severe?

A

ANC < 1500

Bw 1000 and 1500

Bw 500 and 1000

< 500

59
Q

Increased # of reticulocytes in face of anemia is a normal BM response, it indicates what?

A

Hemolysis
Sequestration
loss from bleeding

63
Q

What causes macrocytic anemia w/low reticulocyte count resulting from increased apoptosis in erythroid precursors?

A

Diamond-Blackfan syndrome

causes aplastic anemia

64
Q

What may show basophilic stippling?

A

Lead poisoning

Sideroblastic anemia

66
Q

Describe the following lab values in ACD:

Fe
ferritin
TIBC

A

Down
Up
Down

67
Q

Common symptoms of anemia in kids?

Semi-useful laundry

A
Pallor
Sleepiness
Irritability
DEC exercise tolerance
Flow murmur
68
Q

Hyperpigmentation seen w/what disease?

A

Fanconi anemia

69
Q

Mentzer index = ?

A

MCV / RBC

70
Q

Ulcers on LE seen in what?

A

Sickle cell

Thalassemia

71
Q

Life span of rbc?

Wbc?

A

120 days

21

73
Q

Glossitis seen w/what disease?

A

B12 def

Fe def

78
Q

A low reticulocyte count in face of anemia means what?

A

Relative bone marrow failure

Ineffective erythropoiesis

80
Q

Chloramphenicol causes what?

A

Aplastic anemia

81
Q

What congenital infection of a neonate causes normocytic anemia w/low reticulocytes?

A

Parvovirus B19

93
Q

What is the main red and bolded feature of extravascular hemolysis?

A

Splenomegaly

95
Q

What can kids develop when they are anemic?

A

Flow murmur (inc CO)

96
Q

In anemia, what does the level of EPO do?

RBC production in marrow?

A

Increase

Increase