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Pediatrics > BRS- Heme > Flashcards

BRS- Heme Flashcards

1
Q

At what point in life is the physiologic nadir of hemoglobin concentration?
Time of HbF disappearance?

A

2-3 months= nadir

6-9 months= HbF disappearance

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2
Q

How common is anemia in kids?

A

20% US kiddos

80% worldwide kiddos

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3
Q

Reticulocyte count reflects _____.

Normal percent blood count made up of retics?

A

number of immature RBCs/ activity of bone marrow

normally 1%.

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4
Q

2 Most common types of microcytic, hypochromic anemia in kids? 3 others?

A 
#1: IDA 
#2: B thal 
(also: lead, sideroblastic, chronic dz)
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5
Q

Causes of IDA in kiddos

A
  • lack of dietary iron
  • early ingestion of cows milk
  • occult blood loss
  • menstruation
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6
Q

2 weird key symptoms of anemia

A

spoon shaped nails
diminished attention
also paleness, fatigue etc

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7
Q

Labs assc with IDA:

A
  • low ferritin (early)
  • increased transferrin
  • decreased transferrin saturation
  • increased free erythrocyte protoporphyrin
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8
Q

3 causes of macrocytic anemia

A

-B12
-folate
-thiamine
deficiencies

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9
Q

Normocytic normochromic anemia with low retic counts

A
  • malignancy
  • fanconis
  • red cell aplasias
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10
Q

Three types of red cell aplasia

A

TEC
Diamond Blackfan
Parvo B19

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11
Q

When should workup to determine cause of anemia begin?

A

When anemia is not responsive to iron therapy.

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12
Q

HbA1 makeup

A

A2B2

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13
Q

PE finding in thalassemias

A
  • increased size of bone in the face (chipmunk facies)

- increased size of bone in the skull (crew cut)

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14
Q

What populations are predisposed to a/b thal?

A

A: Asians
B: Meditteraneans

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15
Q

How many types of A thal exist?

B thal?

A

A thal- 4
B thal- 2
(4 alleles exist for A, 2 for B)

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16
Q

What are the two most severe types of a thal?

A
  • HbH disease, some Hb Barts present, which binds O2 very tightly
  • Fetal Hydrops, only Hb Barts present, not compatible with life.

(BARTS BABIES BAD!)

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17
Q

Labs assc with B Thal

A
  • increased HbF
  • low HbA1
  • target cells
  • high bili/LDH (hemolysis)
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18
Q

Complication assc with thalassemia treatment and how to prevent it?

A
  • hemochromatosis due to chronic transfusion

- prevent with deferoxamine

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19
Q

Iron level in B thal minor

A

-normal to high

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20
Q

Sideroblastic anemia:

basic pathologic cause

A

iron in the mitochondria

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21
Q

4 causes of of acquired sideroblastic anemia

A
  • lead
  • isoniazid
  • alcohol
  • chloramphenicol
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22
Q

B12:

  • cogactor for absorption + source
  • site of absorption
A
  • intrinsic factor, gastric parietal cells

- terminal ileum

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23
Q

2 weird manifestations of B12 def

A
  • beefy red tongue

- neuro findings

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24
Q

Three classes of normocytic anemia + how to distinguish them?

A
  • hemolytic (high retics)
  • aplasia (low retics, poor bone marrow effort)
  • sickle cell (high retics)
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25
Q

Three types of aplastic anemia

A
  • malignancy
  • red cell aplasia
  • drug suppression
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26
Q

Hereditary spherocytosis:

  • inheritance pattern
  • assc protein
A
  • AD

- spectrin

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27
Q

3 features assc with hereditary spherocytosis:

A
  • pigmentary gallstones
  • aplastic crises
  • splenomegaly
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28
Q

Test assc with hereditary spherocytosis:

A

-osmotic fragility studies

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29
Q

Condition aside from hereditary spherocytosis that is AD And assc with spectrin?

A

hereditary elliptocytosis

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30
Q

Enzymatic defects of RBCs:

+ which is most common?

A
  • G6PD*

- pyruvate kinase

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31
Q

Enzymatic and RBC structural defects are classified as what type of anemia?

A

hemolytic

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32
Q

PK deficiency:

  • leads to depletion of ____.
  • RBC appearance ______.
  • treatment
A
  • ATP
  • polychromic
  • transfusion/ splenectomy
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33
Q

Three drugs assc with G6PD crisis + how far from exposure do symptoms begin?

A

nitrofurantoin
sulfa drugs
antimalarials
fava beans

24-48 hours

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34
Q

G6PD smear appearance

A
  • bite cells

- Heinz bodies

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35
Q

AIHA:

  • frequent cause of acute AIHA
  • assc test
  • treatment
A
  • respiratory infection, virus, drugs
  • positive direct coombs
  • steroids = rapid complete recovery
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36
Q

Secondary AIHA/ chronic causes:

A
  • lymphoma
  • SLE
  • Immunodeficiency
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37
Q

Two most common causes of alloimmune hemolytic anemia

A
  • Rh Hemolytic Disease (mom type -)

- ABO hemolytic Disease (mom type O)

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38
Q

Test that distinguishes Rh Hemolytic Disease from ABO -

A

direct coombs

  • strongly positive in Rh
  • weakly positive in ABO
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39
Q

Microangiopathic Hemolytic Anemia:

  • type of damage
  • four assc conditions
A
  • mechanical damage

- HUS, artificial heart valves, hemangioma, DIC

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40
Q

Two types of cells seen on Micro HA smear:

A
  • burr cells

- target cells

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41
Q

Gene mutation assc with sickle cell

A

VGB6

valine –> glutamic acid B6 chain

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42
Q

When does sickle cell become symptomatic?

A

~6-9 months when HbF declines

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43
Q

Leading cause of death in sickle cell

A

-infection by encapsulated bacteria due to decreased splenic function

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44
Q

Bacteria dangerous to SS patients

A
  • strep pneumo
  • H flu
  • Neisseria
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45
Q

5 weird sickle cell complications

A
  • dactylitis
  • salmonella osteomyelitis
  • stroke
  • priapism
  • acute chest syndrome
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46
Q

SCA Sequestration crisis:

  • labs
  • symptoms
A
  • low Hb
  • high retics
  • abdominal pain and distention
  • shock
  • usually kiddos under
47
Q

Blood smear findings assc with SCA

A
  • Howell jolly bodies
  • sickled cells
  • target cells
48
Q

5 Preventative measures in SCA

A
  • hydroxyurea to increase HbF
  • penicillin px
  • folic acid
  • immunization (esp flu, pneumo)
  • serial transcranial DUS
49
Q

Bone complication aside from osteo in sickle cell anemia

A

avascular necrosis of the femoral head

50
Q

Three nonmalignant RED cell aplasias (not pancytopenia

A
  • transient erythroblastopenia of childhood (TEC)
  • congenital hypolpastic anemia (Diamond Blackfan)
  • parvovirus B19
51
Q

CHA? Diamond Blackfan:

  • inheritance pattern
  • lab value that is increased
  • physical exam findings (3)
  • two other involved organ systems
A
  • AD/AR
  • high HbF
  • short, triphalangeal thumbs, craniofacial changes
  • renal, cardiac changes
52
Q

Possible cause of transient erythroblastopenia of childhood + prognosis

A

-viral infection –> spontaneously resolves, requires no treatment

53
Q

Findings assc with parvovirus B19 aside from RBC aplasia? Another name?

A
  • slapped cheeks
  • lacy rash
  • fifths disease
54
Q

Congenital cause of pancytopenia +inheritance pattern

A

-fanconi anemia, AR

55
Q

Skeletal +renal abnormalities assc with Fanconis

A
  • absence/ hypoplasia of thumb + radius
  • type 2/ proximal RTA (inability to reabsorb bicarb)

**also see skin pigmentation

56
Q

Drugs causing acquired aplastic anemia

A
  • anticonvulsants
  • chloramphenicol
  • sulfas
57
Q

Infections causing acquired aplastic anemia

A
  • HIV
  • EBV
  • CMV
58
Q

Two types of polycythemia:

A
  • primary (poly vera, malignant)

- secondary (increased EPO)

59
Q

Cause of appropriate polycythemia:

A
  • hypoxemia

- pulm disease

60
Q

EPO/ inappropriate poly can be cause by malignancy of which organs?

A
  • cerebellum/ kidney
  • ovary
  • adrenal
  • liver

(C/KOAL)

61
Q

Appearance assc with polycythemia + 2 complications:

A
  • ruddy complexion
  • thrombosis
  • bleeding
62
Q

Cause of relative polycythemia

A

-dehydration most commonly

63
Q

Three factors assc with hemostasis

A
  • vessels
  • platelets
  • clotting factors
64
Q

Hemophilia A and vWF are both considered _____.
What are their inheritance patterns?
Which is most common?

A

Factor VIII disorders
A: X linked; vWF: AD
vWF = MC bleeding disorder

65
Q

Contrast lab findings in Hemophilia A/ vWF Disease

A
  • both have prolonged PTT, normal PT
  • only vWF has prolonged bleeding time
  • both have normal platelet count
66
Q

Which two bleeding disorders are assc with hemarthroses?

A
  • Factor VIII/ Hemophilia A

- Vitamin K deficiency

67
Q

Order of intrinsic pathway clotting

A

-12,11,9,8,10

68
Q

Order of extrinsic pathway clotting

A

7,10

69
Q

Order of common pathway clotting

A

10,2 (thrombin),1 (fibrin)

70
Q

Factor required for making fibrin polymer

A

XIII

71
Q

What are the three types of vWF disease?

Which is most common?

A

type 1: classic, quantitative***
type 2: qualitative
type 3: absence

72
Q

Test for vWF activity?

Treatment?

A

ristocetin cofactor

DDAVP + cryo

73
Q

Hemophilia B:

  • aka
  • cause
  • inheritance
A
  • Christmas disease
  • factor IX deficiency
  • X linked
74
Q

Three acquired clotting factor disorders

A
  • vitamin K def
  • liver disease
  • DIC
75
Q

Three congenital clotting factor disorders

A
  • Hemo A/ VIII
  • Hemo B/ IX
  • vWF (most common)
76
Q

Clotting factors that require vitamin K

A

-2,7,9,10, C, S

PT, PTT both affected by vit K deficiency

77
Q

Meds that classically depletes vitamin K

A

warfarin

78
Q

Signs of vitamin K def in newborn

A
  • bleeding from circumcision + umbilical stump

- hematemesis

79
Q

Hematologic findings in liver disease/DIC

A
  • ^^ PT,PTT
  • ^^fibrin degradation products
  • thrombocytopenia
80
Q

Findings specific to DIC:

A

-helmet cells
-fragmented RBCs
(not seen in liver disease)

81
Q

Thrombocytopenia findings

A
  • increased bleeding time
  • low platelets
  • petechiae
82
Q

Which of the platelet disorders are assc with petechiae?

A
  • DIC, liver, vit K, thrombocytopenia

- not vWF/ hemophilias

83
Q

Which of the platelet disorders have normal bleeding times?

A

-all prolonged except

Heme A, vitamin K= normal

84
Q

Kasabach-Merritt Syndrome:

describe

A

Large hemangioma –> DIC

85
Q

Five Vessel abnormalities that present with bleeding:

A
  • HSP
  • HHT
  • Scurvy
  • Collagen D/O (i.e. ED)
  • Malnutrition/steroids
86
Q

Hereditary Hemorrhagic Telangiectasia

Inheritance pattern

A

AD

87
Q

Most common cause of bleeding

A

thrombocytopenia

88
Q

Two congenital disorders causing low platelets

A
  • Wiskott Aldrich

- TAR

89
Q

Wiskott Aldrich:
inheritance pattern
3 findings

A
  • X linked
  • thrombocytopenia
  • low B/T cell immunity
  • eczema
90
Q

TAR syndrome:

  • inheritance pattern
  • 4 findings
A
  • thrombocytopenia
  • absent radius
  • cardiac disease
  • renal disease
91
Q

Two diseases causing increased platelet destruction

A
  • ITP

- Neonatal Immune mediated thrombocytopenia

92
Q

Immune thrombocytopenic purpura:

  • when does it most commonly occur?
  • prognosis?
A
  • 1-4 weeks after a viral infection
  • 70-80% resolve spontaneously w/in months
  • 10-20% become chronic (6+ mos), more commonly in ages 10+
93
Q

Smear finding in ITP

A

few large sticky platelets

94
Q

Two types of neonatal immune thrombo

A
  • passive (mother has low platelets)

- isoimmune (mother has normal platelets)

95
Q

Two “syndromes” assc with low platelets

A
  • Kasabach Merritt

- HUS

96
Q

Three misc causes of low platelets

A
  • DIC
  • large spleen
  • drugs
97
Q

Two drugs that interfere with platelet function

A
  • aspirin

- valproate

98
Q

Two systemic diseases that impair platelet function

A
  • liver disease

- uremia

99
Q

Two congenital disorders effecting platelet function + their inheritance pattern

A

-Glanzmann
-Bernard Soulier
both AR

100
Q

Defect in Glanzman/ Bernard Soulier

A
  • Glanzmann: lack of IIb/IIIa, = no aggregation

- BS: lack of membrane glycoprotein= no adhesion

101
Q

Most common childhood platelet disorder

A

ITP

102
Q

Four factors most commonly altered in hypercoagulable disorders

A
  • protein C
  • protein S
  • factor V
  • antithrombin III
103
Q

Presentations for homozygotes lacking Protein C

A

-purpura fulminans early in life (rapidly spreading bleeding)

104
Q

Neutropenia predisposes to what infections?

A
  • moderate: mucus membrane/ skin infection

- severe: sepsis, gram neg or s. aureus

105
Q

What is considered severe neutropenia?

A

ANC less than 500 cells/mm^3

106
Q

Most common cause neutropenia in childhood

A

infection

107
Q

Chronic benign neutropenia of childhood:
age group
prognosis
key distinguishing feature of disease

A
  • less than 4
  • resolves spontaneously within months –> years
  • neutropenia is NONCYCLICAL
108
Q

Kostmann syndrome:

  • aka
  • inheritance pattern
A
  • severe congenital agranulocytosis

- AR

109
Q

Cyclical neutropenia:

  • inheritance pattern
  • features during neutropenic episode
A
  • AD

- Fever, ulcers, stomatitis

110
Q

Three genetic “syndromes” assc with neutropenia + inheritance pattern

A
  • Chediak Higashi
  • Cartilage hair hypoplasia syndrome
  • Schwachmann Diamond
  • both are AR
111
Q

Chediak Higashi

  • PE findings
  • Smear findings
A
  • albinish

- blue granules in neutrophil cytoplasm + neutropenia

112
Q

Four features of Cartilage hair hypoplasia syndrome

A
  • immunodeficiency
  • thin hair
  • short stature
  • neutropenia
113
Q

Schwachman Diamond Syndrome:

-4 features

A
  • exocrine pancreatic insufficiency
  • short stature
  • neutropenia
  • metaphyseal chrondrodysplasia
114
Q

Five causes of increased neutrophil destruction

A

1) infection
2) drugs
3) hypersplenism
4) autoimmune neutropenia
5) isoimmune neutroplenia

Pediatrics (12 decks)

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