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
What causes delayed separation of the umbilical cord (> 3weeks) w/recurrent bacterial and fungal infections w/out pus forming? Inheritance?
Leukocyte adhesion deficiency AR
40
How does the oxygen dissociation curve move in response to anemia?
Shifts to the right
43
Microcornea seen w/what disease?
Fanconi anemia
45
what shows a triad of neutropenia, exocrine pancreas insufficiency, and skeletal abnormalities?
Shwachman-Diamond syndrome
47
What are the causes of eosinophilia? Apparently this laundry is a TQ
NAACP does DRUGS ``` Neoplasia Asthma Allergy Chronic adrenal Insufficiency Parasites Drugs ```
49
+ direct Coombs, Inc indirect bilirubin, normocytic, INC reticulocyte is what? Examples?
Isoimmunization ABO or Rh incompatibility
51
How does Fe deficiency anemia present (CFs)?
Fatigue Conjuctival pallor Pica Spoon nails (koilonychia)
52
What is the most common cause of acquired neutropenia in infancy and childhood? Exhibit what? Present when?
Primary autoimmune neutropenia Uri or skin infection 2mo - 3yrs
53
Menzter index > 13 means what? < 13?
Fe deficiency Thalassemia
55
Heinz bodies, bite cells seen in what?
G6PD def
56
What causes recurrent purulent infections w/fungal or bacterial catalase(+) organisms starting in infancy? Defect in what? diagnose how?
Chronic granulomatous disease Oxidative metabolism, can't make superoxide Nitroblue tetrazolium test
57
What is included in CBC w/diff?
CBC | Total WBC
58
Neutropenia defines as what? Mild? Moderate? Severe?
ANC < 1500 Bw 1000 and 1500 Bw 500 and 1000 < 500
59
Increased # of reticulocytes in face of anemia is a normal BM response, it indicates what?
Hemolysis Sequestration loss from bleeding
63
What causes macrocytic anemia w/low reticulocyte count resulting from increased apoptosis in erythroid precursors?
Diamond-Blackfan syndrome | causes aplastic anemia
64
What may show basophilic stippling?
Lead poisoning | Sideroblastic anemia
66
Describe the following lab values in ACD: Fe ferritin TIBC
Down Up Down
67
Common symptoms of anemia in kids? Semi-useful laundry
``` Pallor Sleepiness Irritability DEC exercise tolerance Flow murmur ```
68
Hyperpigmentation seen w/what disease?
Fanconi anemia
69
Mentzer index = ?
MCV / RBC
70
Ulcers on LE seen in what?
Sickle cell | Thalassemia
71
Life span of rbc? Wbc?
120 days 21
73
Glossitis seen w/what disease?
B12 def | Fe def
78
A low reticulocyte count in face of anemia means what?
Relative bone marrow failure | Ineffective erythropoiesis
80
Chloramphenicol causes what?
Aplastic anemia
81
What congenital infection of a neonate causes normocytic anemia w/low reticulocytes?
Parvovirus B19
93
What is the main red and bolded feature of extravascular hemolysis?
Splenomegaly
95
What can kids develop when they are anemic?
Flow murmur (inc CO)
96
In anemia, what does the level of EPO do? RBC production in marrow?
Increase Increase