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Peds Exam 1 (Lauren) > Hematology 💉 > Flashcards

Hematology 💉 Flashcards

1
Q

Is ITP preceded by any event?

A

Usually viral infection

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2
Q

How do you treat hereditary spherocytosis?

A

Supportive care

Blood transfusion

Splenectomy

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3
Q

What are the sx of megaloblastic anemia (both folate and B12)

A

Pallor

Glossitis*****

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4
Q

Do kids with chronic anemia have any symptoms?

A

Not usually because they are used to it and have been compensating

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5
Q

How do you diagnose ITP?

A

You exclude every other possibility ~diagnosis of exclusion~~~

If there’s bleeding and platelets are low and there’s no other explanation= ITP

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6
Q

When i say frequent or severe infections, you say

A

Leukocytopenia

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7
Q

What is the most common bleeding disorder of childhood?

A

Idiopathic Thrombocytopenic Purpura (ITP)

*****

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8
Q

How do you treat von Willebrand disease?

A

Desmopressin*** (causes release of vWF and factor VIII from endothelium stores)

vWF replacement

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9
Q

What medication increases the risk of venous thromboembolism in people with Factor V Leiden mutation?

A

Oral contraceptives

35x risk for heterozygous

80x risk for homozygous

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10
Q

What kind of anemia does lead poisoning cause?

A

Mild normocytic anemia

Anemia is due to hemolysis

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11
Q

How do you treat sickle cell anemia?

A

Hydroxyurea*****

Treat pain of vaso-occlusive episodes**

Avoid precipitating factors

Stem cell transplant

Supportive

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12
Q

How do you diagnose sickle cell anemia?

A

Hemoglobin electrophoresis: must see HbS

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13
Q

What happens with von Willebrand disease?

A

Decrease/impairment of von Willebrand Factor (vWF)

=platelets don’t clump

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14
Q

Do people with G6PD deficiency have hemolysis going on all the time?

A

No, it occurs episodically due to oxidative stress

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15
Q

What does protein C do?

A

It deactivates factors V and VIII when you don’t need them anymore

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16
Q

What is the most common ~inherited~ bleeding disorder?

A

von Willebrand disease****

On test!!

(ITP was most common bleeding disorder)

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17
Q

How do you treat DIC?

A

Treating the triggering event

Replacement of coagulant factors

Anticoagulation therapy when indicated

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18
Q

How do you treat Fanconi anemia?

A

Supportive care for the anemia, low platelets, and low WBCs

Eventually you need a stem cell transplant

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19
Q

What is hereditary spherocytosis?

A

A Red blood cell membrane defect that causes the RBCs to have increased osmotic fragility and spherical shapes.

Leads to increased cell lysis and thus, hemolytic anemia

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20
Q

Which pathways are measured with PT (prothrombin time)?

A

Extrinsic and common pathways

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21
Q

If your 4 year old patient has frequent nosebleeds, what diagnosis should you consider

A

ITP

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22
Q

How could someone become defeicient in B12

A

Intestinal MALABSORPTION (crohn, UC, celiac, etc)

Diet insufficiency (vegans)

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23
Q

What factor is deficient in hemophilia B?

A

Factor IX

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24
Q

How do you treat thrombotic disorders?

Protein C, Protein S, Factor V Leiden, Antithrombin

A

If they have an episode of VTE, they need to be on anticoagulant prophylaxis for at least 3 months

Anticoagulant prophylaxis:
Unfractionated heparin
LMW heparin
Warfarin (unless they’re pregnant)

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25
Q

What Hgb measurement qualifies as

“anemia” in children 6 months to 5 years?

A

<11 g/dL

Both male and female

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26
Q

What are the ONLY 2 causes of Megaloblastic anemia?

A

Vitamin B12 (cobalamin) deficiency

Folic acid deficiency

You already know this but he said it 3 times I don’t want you to forget

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27
Q

If you have Factor V Leiden Mutation you are prone to (Bleeding/clotting)

A

Clotting

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28
Q

What will happen to PT and aPTT in liver disease or vitamin K deficiency?

A

Both will be prolonged

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29
Q

What kind of symptoms will be seen with primary polycythemia?

A

Headache

Lethargy

Splenomegaly

Plethora ?

Hgb as high as 27

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30
Q

What platelet count will you see in Henoch-Schonlein Purpura?

A

NORMAL or elevated

The Purpura are caused by immune complexes, NOT platelets

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31
Q

What is the most common reason for pain in sickle cell disease?

A

Vaso-occlusion

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32
Q

Predict the lab values for hemophilia:

Platelet:

PT:

aPTT:

A

Platelet: normal

PT: normal

aPTT: prolonged

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33
Q

Which kind of macrocytuic anemia will have a high methylmalonic acid?

A

B12 deficient only

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34
Q

What are 2 pieces of evidence that someone has thrombotic lesions (like in DIC)

A

Major vessel thrombosis (ex whole leg is thrombosed)

Purpura fulminans (acute, usually fatal, thrombotic disorder that manifests as blood spots, bruising, and discoloration of the skin)

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35
Q

What are the 2 other names for primary polycythemia?

A

Congenital erythrocytosis

Familial polycythemia

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36
Q

What is the treatment for primary polycythemia aka congenital erythrocytosis aka familial polycythemia?

A

Phlebotomy ****

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37
Q

What does protein S do?

A

It is a cofactors for protein C, and facilitates the action of activated protein C

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38
Q

What are the symptoms of Henoch-Schonlein Purpura?

A

Palpable Purpura ** (immune complexes stuck under skin)

Arthritis

Abdominal pain

Renal disease

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39
Q

If you’re deficient in protein C or Protein S, you will have too much (bleeding/clotting)

A

Clotting

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40
Q

Who gets hemophilia more often, men or women?

A

Men. It is X-linked

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41
Q

How will platelet count differ in liver disease vs Vitamin K deficiency?

A

Liver disease: normal/low

Vitamin K: normal

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42
Q

What drug therapy is monitored by INR?

A

Warfarin

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43
Q

What sx ONLY occur with B12 deficiency and NOT folate deficiency>

A

Neurologic signs

Paresthesias, weakness, unsteady gate, decreased vibratory sensation and proprioception on neurologic exam

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44
Q

What causes acquired aplastic anemia?

A

50% we don’t know

50% medications, toxic exposures, viruses (mono, HIV, hep)

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45
Q

What is vWF?

A

A protein that binds to factor VIII and is a cofactors for platelet adhesion to the endothelium

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46
Q

What are some pieces of evidence that someone has diffuse bleeding tendency (like in DIC)?

A

Hematuria

Melena

Purpura/petechiae

Persistent oozing from needle puncture sites

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47
Q

How do you treat ITP?

A

Avoid medications that mess with your platelets (Aspirin, NSAIDS)**

Prednisone***** (this is autoimmune)

Bleeding precautions

IVIG

Splenectomy

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48
Q

What is the most common nutritional deficiency in children/

A

Iron

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49
Q

What are the 2 possible causes of macrocytic anemia

A

B12 deficiency

Folate deficiency

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50
Q

What disorders will cause an increased bleeding time?

A

Platelet disorders (von Willebrand)

Severe thrombocytopenia

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51
Q

What is INR?

A

A more accurate PT (standardized)

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52
Q

What will you see on CBC of someone with acquired aplastic anemia?

A

Anemia, usually normocytic

Low WBC with marked neutropenia

Thrombocytopenia

Low retic count- Bone Marrow is NOT working duh

53
Q

What are the 2 days someone might become hypoxic leading to secondary polycythemia?

A

Cyanotic congenital heart disease (Tetralogy of Fallot, TGA, etc)

Chronic pulmonary disease (COPD, CF, etc)

54
Q

What happens with Henoch-Schonlein Purpura?

A

IgA immune complexes deposit in the small vessels of the skin, GI tract, and kidneys

IgA IgA IgA IgA IgA IgA IgA IgA

55
Q

What will you see on the bloodwork in Fanconi anemia?

A

Progressive Pancytopenia

ALL cell lines are scanty

56
Q

What are the symptoms of von Willebrand factor disease?

A

Prolonged bleeding from mucosa: nosebleeds, heavy periods, GI bleeding

Easy bruising

57
Q

What is DIC?

A
  1. Triggering event
  2. Activation of coagulation cascade
  3. Microthrombi everywhere
  4. Massive consumption of platelets, fibrin, and coagulation factors
  5. Severe bleeding

BASICALLY bleeding out and clotting small vessels all at the same time

58
Q

Antithrombin makes this drug not work

A

Heparin

59
Q

What kind of lab values will you see with ITP?

Platelets:

WBC:

Hgb:

PT:

aPTT:

A

Platelets: LOW

WBC: normal

Hgb: normal

PT: normal

aPTT: normal

All tests are normal except for platelet count

60
Q

What will you see on the peripheral smear in lead poisoning?

A

Basophilic stippling ****

61
Q

What is NEEDED to diagnose thalassemia?

A

Hemoglobin electrophoresis

62
Q

How do you treat lead poisoning?

A

chelation

63
Q

What does the severity of thalassemia depend on

A

Number of gene deletions

64
Q

What is the treatment of secondary polycythemia?

A

Correction of underlying hypoxic disorder

Phlebotomy

65
Q

Which clotting factors are in the intrinsic pathway?

A

8

9

11

12

66
Q

What is fanconi anemia?

A

An inherited bone marrow failure

67
Q

What is the most common type of small vessel Vasculitis?

A

Henoch-Schonlein Purpura (HSP)

68
Q

If you’re giving your patient heparin and its not working what disease should you consider that they might have

A

Antithrombin deficiency

69
Q

Where do hemophiliacs commonly bleed?

A

INTO joints and muscles***

Hemarthrosis= bleeding into a joint

70
Q

What usually precedes Henoch-Schonlein Purpura?

A

Upper respiratory infection

Same as ITP

71
Q

What are the lab findings of iron deficiency anemia:

RBC

MCV

Hgb

Ferritin

RDW

A

RBC: low

MCV: low

Hgb: <11 g/dL

Ferritin: < 12mcg/L

RDW: Increased ****

72
Q

What is the difference between primary and secondary polycythemia?

A

Primary- congenital

Secondary- acquired due to hypoxemia

73
Q

Predict the lab values for von Willebrand disease:

Platelet:

PT:

aPTT:

A

Platelet: normal or low

PT: normal

aPTT: normal or prolonged (vWF works with factor VIII)

74
Q

What will you see in a peripheral smear of G6PD deficienty?

A

HEINZ BODIES***** (denatured hemoglobin)

“Bite” deformities

75
Q

Which is more common: hemophilia A or B

A

A

76
Q

What does a high vs low reticulocyte count tell you

A

High: bone marrow is working

Low: trouble

77
Q

What can happen if you give Warfarin to someone with Protein C deficiency?

A

Warfarin-induced skin necrosis***

78
Q

What kinds of things can cause oxidative stress and precipitate a hemolytic episode in someone with G6PD deficiency?

A

Infection

Drugs

Food

79
Q

Which factors are in the common pathway?

A

1

2

5

10

80
Q

Why does hereditary spherocytosis cause anemia, jaundice, gallstones, and splenomegaly?

A

The RBCs are delicate and sphere shaped, causing them to rupture all the time.
Many get stuck in the trabeculae of the spleen= splenomegaly

81
Q

Why is Fanconi anemia often misdiagnosed as ITP?

A

Because Fanconi’s starts with either low platelets or low WBC, and if they have low platelets, they’ll be bleeding a lot, so ITP explains the easy bleeding.

(But then the anemia and leukopenia appear which is not part of ITP)

82
Q

What happens with ITP?

A

**IMMUNE MEDIATED attack against your own platelets:

  1. The body makes antibodies that bind to platelets
  2. Platelets are phagocytized by splenic macrophages
  3. Decreased platelet lifespan
83
Q

Which specific clotting factors are measured with PTT/aPTT?

A

1

2

5

8**

9**

10

11**

12**

**indicated intrinsic pathway

84
Q

What are the risk factors for iron deficiency anemia?

A

Low SES

Premature/low birth weight

Lead exposure

Exclusive breast feeding beyond 4 months

Weaning to foods that don’t have iron

Feeding problems

85
Q

How do you get Fanconi anemia?

A

You inherit it from both of your parents

Autosomal recessive

86
Q

What are the 3 possible causes of microcytic anemia?

A

Iron deficiency

Thalassemia

Lead poisoning

87
Q

What does bleeding time measure?

A

The time it takes for hemostasis

88
Q

What are the clinical manifestations of ITP?

A

Petechiae

Bruising

Nosebleeds

89
Q

What labs do you need to order for Henoch-Schonlein Purpura?

A

Platelets (normal or high)

Antistreptolysin O (ASO) titer (high)

Serum IgA (high)

Hemoccult (blood in poo)

Urinalysis (hematuria)

90
Q

Which specific clotting factors are measured with PT

A

1

2

5

7***

10

Tissue Factor**

**indicates extrinsic pathway

91
Q

What is the prognosis for fanconi anemia?

A

Many succumb to bleeding, infection or malignancy in adolescence

High risk of developing myelodysplastic syndrome or AML

92
Q

How do you treat hemophilia?

A

Desmopressin (for Hemophilia A)

Factor replacement (VIII and IX)

93
Q

Predict the lab values for ITP:

Platelet:

PT:

aPTT:

A

Platelet: low

PT: normal

aPTT: normal

94
Q

Which factors are in the extrinsic pathway?

A

7

Tissue factor

95
Q

What will you see in the labs of someone with megaloblastic anemia?

MCV:

MCH:

Neutrophil size and appearance:

Shape of RBC:

Homocysteine:

Methylmalonic acid MMA:

A

MCV: >100

MCH: high

Neutrophils: large with HYPERSEGMENTED NUCLEI**

RBC: big and oval shaped

Homocysteine: high in B12 and folate

MMA: high ONLY WITH B12 deficiency

96
Q

What is hemarthrosis and what disorder is it associated with

A

It is bleeding into a joint

Associated with hemophilia*****

**on test

97
Q

When i say purpura, petechiae and bleeding, you say

A

Thrombocytopenia

98
Q

What does the bone marrow look like in someone with aplastic anemia?

A

“Bleached”

99
Q

What kind of anemia will thalassemia cause?

A

Microcytic, hypochromic

100
Q

Predict the lab values for DIC:

Platelet:

PT:

aPTT:

A

Platelet: decreased

PT: prolonged

aPTT: prolonged

Everything is fucked up

101
Q

Which pathways are measured with PTT/aPTT (activated partial thromboplastin time)

A

Intrinsic and common pathways

102
Q

What are the sx of ACUTE anemia ?

A

Lethargy **

Tachycardia**

Irritability in infants**

Pallor

Poor oral intake

103
Q

If you have antithrombin deficiency, you are prone to (bleeding/clots)

A

Clots

104
Q

Which disorders are associated with epistaxis?

A

ITP

von Willebrand Disease

105
Q

For all thrombotic disorders (Protein C, Protein S, antithrombin, Factor V Leiden) should you expect to see a family history of thrombosis?

A

Yes

106
Q

What will happen if you have a protein C deficiency?

A

Blood clots

107
Q

What are the hallmark lab values of DIC?

A

Elevated D Dimer

Elevated fibrin degradation products

108
Q

How do you tell hemophilia apart from von Willebrand disease?

A

Bleeding time is prolonged in von Willebrand disease

109
Q

What kind of laboratory findings will you see with DIC?

A

Decreased platelet

Prolonged aPTT and PT

Decreased fibrinogen level

Elevated D-Dimer**

Elevated fibrin degradation products (FDPs)***

110
Q

What kind of lab values will you see with von Willebrand disease?

PT

aPTT

Factor VIII levels

vWF levels

Bleeding time

A

PT: normal

aPTT: normal/prolonged

Factor VIII: normal/low

vWF: normal/low

Bleeding time: PROLONGED***

111
Q

Do people with sickle cell anemia have spleen problems?

A

Yes, they have splenomegaly and can also have splenic infarcts leading to functional asplenia

112
Q

What does Bleeding Time screen for?

A

Platelet dysfunction**

113
Q

What is acquired aplastic anemia?

A

Peripheral pancytopenia with a hypocellular bone marrow***

114
Q

What kind of cells are affected by primary polycythemia?

A

RBCs ONLY

115
Q

What factor is deficient in Hemophilia A?

A

Factor VIII

116
Q

What are the 2 types of thalassemia

A

Alpha

Beta

117
Q

What is the progression of Fanconi’s anemia?

A
  1. Low platelets or low WBC
  2. Low RBCs and severe aplastic anemia
  3. Bone marrow hypoplasia/aplasia= no cell lines being produced= Emergency
118
Q

True or false:

ITP is immune mediated

A

True.

The body makes antibodies against platelets

119
Q

Screening for anemia is performed at what age?

A

12 months**

120
Q

True or false:

Sickle cell anemia causes chronic hemolysis

A

True, thats why its an anemia duh

121
Q

What will happen if you treat B12 deficiency with folate only?

A

Anemia will resolve but the patient will have PERMANENT neurological deficits**

122
Q

Which clotting factors are dependent on vitamin K?

A

2 7 9 10

123
Q

What are the leading causes of death in people with acquired aplastic anemia?

A

Overwhelming infection

Severe hemorrhage

124
Q

What lab findings will you see in hemophilia?

Platelet count:

PT

aPTT:

Bleeding TIme:

vWF:

A

Platelet count: normal

PT: normal

aPTT: Prolonged

Bleeding time: normal

vWF: normal

125
Q

What kinds of events can trigger DIC?

A

Sepsis

Trauma

Malignancies

126
Q

In Sickle Cell disease, are the RBCs always sickle shaped?

A

Only when deoxygenated

127
Q

When i say weakness, fatigue and pallor, you say

A

Anemia

128
Q

How do you treat iron deficiency in children?

A

Iron 6mg/kg

divided into 3 daily doses

Peds Exam 1 (Lauren) (4 decks)

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