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Unit 1 Peds II > L4 Peds Hematology > Flashcards

L4 Peds Hematology Flashcards

1
Q

anemia definition

A

2 standard deviations below normal for age and sex

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2
Q

anemia definition 6 months - 5 years

A

Hgb concentration <11g/dl

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3
Q

Low reticulocyte count means

A

bone marrow isn’t producing RBC to keep up with anemia, “trouble”

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4
Q

High reticulocyte count means

A

bone marrow is working

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5
Q

Normocytic normochromic anemia

A

anemia of chronic disease

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6
Q

Microcytic hypochromic anemia

A

iron defienciy
thalassemia
lead intoxication

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7
Q

macrocytic anemia

A

vitamin B12/folate deficiency

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8
Q

iron deficiency anemia RBCs

A

Microcytic hypochromic anemia

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9
Q

thalassemia anemia RBCs

A

Microcytic hypochromic anemia

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10
Q

lead intoxication RBCs

A

Microcytic hypochromic anemia

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11
Q

anemia of chronic disease RBCs

A

Normocytic normochromic anemia

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12
Q

acute signs of anemia

A

lethargy
tachycardia
pallor

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13
Q

acute signs of anemia in infants

A

irritability and poor oral intake

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14
Q

chronic anemia presentation

A

may present with few or no symptoms at all

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15
Q

other findings in anemia

A

jaundice, gallstone disease, petechiae, purpura, ecchymosis, bleeding

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16
Q

Bone marrow failure (2)

A

Fanconi Anemia

Acquired aplastic anemia

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17
Q

Fanconi Anemia

A

Inherited bone marrow failure syndrome
Autosomal recessive, defective DNA repair
Majority develop in first 10 years of life

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18
Q

Fanconi Anemia Congenital malformations:

A

abnormal pigmentation of skin
short stature
skeletal malformations

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19
Q

Progressive pancytopenia

A

Fanconi Anemia

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20
Q

Non-anemia symptoms of fanconi anemia

A

Increased incidence of malignancies: Myelodysplastic syndrome (MDS), Acute myeloid leukemia (AML)

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21
Q

often misdiagnosed as Idiopathic thrombocytopenic purpura (ITP)

A

Fanconi Anemia

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22
Q

Lab findings of fanconi anemia

A

Thrombocytopenia leukopenia
severe aplastic anemia
bone marrow hypoplasia/aplasia

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23
Q

which lab finding of fanconi anemia usually occurs before the anemia

A

Thrombocytopenia leukopenia

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24
Q

most common nutritional deficiency

A

iron deficiency anemia

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25
Q

most common bleeding disorder

A

idiopathic thrombocytopenic purpura (ITP)

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26
Q

fanconi anemia prognosis

A

mortality due to bleeding, infection, malignancy in adolescence/early adulthood

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27
Q

Fanconi Anemia vs Acquired aplastic anemia: pancytopenia

A

Fanconi Anemia: progressive

Acquired aplastic anemia: peripheral with a hypocellular bone marrow

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28
Q

Causes of Acquired aplastic anemia

A

Idiopathic (50%), toxic exposure to insecticides, viruses (EBV, Hepatitis, HIV)

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29
Q

Signs/symptoms of Acquired aplastic anemia

A

weakness, fatigue, pallow, frequent/severe infections, purpura, petechiae, bleeding

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30
Q

bone marrow failure vs. idiopathic thrombocytopenic purpura (ITP)

A

ITP gets better after a transfusion, bone marrow failure does not

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31
Q

Lab findings in Acquired aplastic anemia

A

Anemia, usually normocytic
Low WBC with marked neutropenia
Thrombocytopenia
Low reticulocyte count

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32
Q

Leading causes of death for acquired aplastic anemia

A

infection

severe hemorrhage

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33
Q

greatest prevalence of iron deficiency anemia

A 
children 1-2 years 
Females of childbearing age
African american children 
Hispanic children
Poverty
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34
Q

signs/symptoms of iron defieciency anemia

A

varies with severity: asymptomatic, pallor, fatigue, irritability, delayed motor development, pica

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35
Q

when/how to screen for iron deficiency anemia

A

12 months: hgb concentration, risk factors

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36
Q

at risk for iron deficiency anemia

A

low socioeconomic status, prematurity/low birth weight, lead exposure, exclusive breast feeding after 4 months without iron supplementation, weaning to whole milks/foods not containing iron, feeding problems, poor growth, inadequate nutrition

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37
Q

labs for iron deficiency anemia

A

Microcytic, hypochromic anemia
Hemoglobin <11 g/dL
Ferritin <12 mcg/L

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38
Q

If hemoglobin is 10-11 mg/dL at 12 month screening

A

closely monitored or empirically treated

recheck hemoglobin in 1 month

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39
Q

if iron deficiency anemia is diagnosed, tx

A

iron 6 mg/kg/day, divided into 3 doses

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40
Q

if your patient eats a lot of ice chips, think

A

iron deficiency anemia

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41
Q

2 megaloblastic anemias

A 
Vitamin B12 (cobalamin) deficiency
Folic Acid deficiency
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42
Q

megaloblastic anemia aka

A

macrocytic anemia

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43
Q

Vitamin B12 aka

A

cobalamin

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44
Q

causes of B12 defieciency

A 
intestinal malabsorption (IBD)
dietary insufficiency
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45
Q

causes of Folic acid deficiency

A

increased folate requirement, malabsorptive syndromes, inadequate dietary intake (rare), medications

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46
Q

increased folate requirement is seen in

A

rapid growth, chronic hemolytic anemia

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47
Q

signs/symptoms of megaloblastic anemia

A

Glossitus

Pallor

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48
Q

neurologic signs such as ________ ONLY occur with _______

A

decreased vibratory sensation and proprioception, parethsesias, weakness, unsteady gait
B12 deficiency

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49
Q

Labs of megaloblastic anemia

A

Elevated MCV, MCH
Larger, hypersegmented neutrophils
Macro-ovalocytes (RBC)
Elevated homocysteine

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50
Q

Labs of B12 deficiency

A

elevated methylmalonic acid and homocysteine

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51
Q

labs of folate deficiency

A

elevated homocysteine without elevated methylmalonic acid

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52
Q

if you give folate to a B12 deficient patient

A

clinical symptoms and anemia will improve, but it will not avoid permanent neurological deficity

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53
Q

Red cell membrane defect→ hemolytic anemia → jaundice, splenomegaly, gallstones
Spherocytes
Increased osmotic fragility

A

Hereditary spherocytosis

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54
Q

may be treated with splenectomy

A

Hereditary spherocytosis

+/- thalassemia

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55
Q

Thalassemia RBC

A

Microcytic, hypochromic anemia

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56
Q

needed for diagnosis of thalassemia

A

hemoglobin electrophoresis

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57
Q

when would a thalassemia patient get a hematopoietic stem cell transplant

A

severe beta thalassemia

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58
Q

pain in sickle cell is caused by

A

vaso-occlusion

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59
Q

Splenomegaly

Splenic infarcts → functional asplenia

A

sickle cell anemia

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60
Q

+/- growth failure and delayed puberty

A

sickle cell anemia

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61
Q

Hydroxyurea

A

treatment of sickle cell anemia

62
Q

Episodic hemolysis: exposure to oxidant stress of infection, certain drugs and foods

A

G6PD

63
Q

Red cell enzyme defect→ hemolytic anemia→ pallor, jaundice

X linked recessive inheritance

A

G6PD

64
Q

Labs:
Hyperbilirubinemia
episodic hemolysis → hemoglobinuria

A

G6PD

65
Q 
Peripheral smear:
Bite like deformities
Heinz bodies (denatured hemoglobin)
A

G6PD

66
Q

lead poisoning anemia is

A

Mild, hemolytic, normocytic anemia

67
Q

Peripheral smear: Basophilic stippling

A

lead poisoning

68
Q

lead poisoning results from

A

lead paint in old houses

69
Q

Familial Polycythemia aka

A

Congenital Erythrocytosis
or
Primary polycthemia

70
Q

Familial polycythemia affects

A

ONLY RBC

71
Q

Congenital Erythrocytosis symptoms

A

Headache, lethargy

+/- plethora (swelling), splenomegaly

72
Q

Causes of secondary polycythemia

A

Occurs in response to hypoxemia:
Cyanotic congenital heart disease
Chronic pulmonary disease (cystic fibrosis)

73
Q

Initial screening tests for bleeding disorders

A

CBC, Peripheral smear, PT/INR, aPTT, +/- bleeding time

74
Q

Normal platelet count

A

150,000-400,000/mm3

75
Q

platelet count at risk of spontaneous bleeding

A

<20,000

76
Q

measure extrinsic and common pathways

A

PT

77
Q

measures intrinsic and common pathways

A

PTT/aPTT

78
Q

extrinsic and common pathways

A

I, II, V, VII, X, Tissue factor

79
Q

intrinsic and common pathways

A

I, II, V, VIII, IX, X, XI, XII

80
Q

common pathway

A

II, IIa, I, V, X, phospholipids, XIII

81
Q

Intrinsic pathway

A

VIII, IX, XI, XII

82
Q

Extrinsic pathway

A

Tissue factor

VII

83
Q

more accurate reflection of PT used to monitor warfarin treatment

A

INR

84
Q

measure of time for hemostasis, screening test for platelet dysfunction. Prolonged in platelet disorders

A

Bleeding time

85
Q

Disorders with prolonged bleeding times

A

von Willebrand disease, severe thrombocytopenia

86
Q

Most common bleeding disorder of childhood, most frequently 2-5 years old

A

Idiopathic Thrombocytopenic Purpura (ITP)

87
Q

Idiopathic Thrombocytopenic Purpura (ITP) cause

A

Often follows infection with viruses→ immune mediated: autoantibodies bind to platelets → phagocytized by splenic macrophages → decreased platelet lifespan

88
Q

Idiopathic Thrombocytopenic Purpura (ITP) prognosis

A

90% have spontaneous remission

89
Q

Idiopathic Thrombocytopenic Purpura (ITP) signs/symptoms

A

multiple petechiae, ecchymosis, epistaxis

90
Q

Your patient had a viral infection and now is getting a bunch of nosebleeds, what’s your ddx?

A

Idiopathic Thrombocytopenic Purpura (ITP)

91
Q

Idiopathic Thrombocytopenic Purpura labs

A

Thrombocytopenia

Normal WBC, hemoglobin, PT, aPTT

Hemorrhage → low hemoglobin

92
Q

How to diagnose Idiopathic Thrombocytopenic Purpura

A

diagnosis of exclusion

93
Q

if petechiae are observed, always

A

check platelets to check for Idiopathic Thrombocytopenic Purpura

94
Q

medications that compromise platelet function

A

Aspirin

NSAIDS

95
Q

Idiopathic Thrombocytopenic Purpura treatment

A 
avoid meds that compromise platelet function
Bleeding precautions
Prednisone
IVIG
Splenectomy
96
Q

who to observe with Idiopathic Thrombocytopenic Purpura

A

asymptomatic children

97
Q

most common inherited bleeding disorder

A

Von Willebrand Disease

autosomal dominant, 1% prevalence

98
Q

Von Willebrand disease pathophysiology

A

Decrease in level of or impairment in the action of von Willebrand Factor (vWF)

99
Q

most common type of Von Willebrand disease

A

type 1 (of 3 major types)

100
Q

von Willebrand factor

A

binds to factor VIII and is a cofactor for platelet adhesion to the endothelium

101
Q

Von willebrand disease presentation

A

prolonged bleeding from mucosal surfaces, epistasis, menorrhagia, GI, easy bruising

102
Q

Von willebrand disease labs

A

Prolonged/normal aPTT
Decreased/normal Factor VIII
Decreased/normal vWF
Prolonged bleeding time

103
Q

Von Willebrand disease vs hemophilia: bleeding time

A

Von Willebrand disease: prolonged

Hemophilia: normal

104
Q

Desmopressin MOA

A

Causes release of vWF and factor VIII from endothelial stores

105
Q

Von Willebrand disease treatment

A

Desmopressin

vWF replacement therapy

106
Q

factor VIII deficiency

A

Hemophilia A

107
Q

factor IX deficiency

A

Hemophilia B

108
Q

Christmas disease aka

A

Hemophilia B

109
Q

factors VIII and IX are found in the

A

intrinsic pathway

110
Q

most common sites of bleeding in hemophilia

A

into joints and muscles

111
Q

severe hemophilia can present with

A

spontaneous hemarthrosis→ joint destruction

112
Q

Hemophilia labs

A

Normal platelet count, PT, bleeding time, vWF

Prolonged aPTT (can be normal in mild disease)

113
Q

Hemophilia A treatment

A

desmopressin

114
Q

to achieve sufficient hemostasis in patients with hemophilia, treat with

A

Factor replacement (VII, IX)

115
Q

Disseminated intravascular coagulation pathophysiology

A

Triggered by sepsis, trauma/tissue injury, or malignancies→ widespread activation of coagulation cascade → microthrombi→ consumption of platelets → hemorrhage

116
Q

Disseminated intravascular coagulation labs

A

Hematuria

Decreased platelet count
Decreased fibrinogen (may be normal until late stage)
Prolonged aPTT and PT
Elevated D dimer
Elevated fibrin degradation product (FDPs)

117
Q

Disseminated intravascular coagulation treatment

A

Replacement therapy

Anticoagulant therapy

118
Q

the liver synthesizes

A

prothrombin
fibrinogen
Factors V, VII, IX, X, XII, XIII

119
Q

hallmark of Disseminated intravascular coagulation on labs

A

Elevated D dimer

Elevated fibrin degradation product (FDPs)

120
Q

vitamin-K dependent factors

A

II, VII, IX, X

121
Q

vitamin K deficiency vs liver disease on labs:

A

vitamin K: normal platelet count
liver disease: normal/low platelet count
both: prolonged PT, aPTT

122
Q

the one bleeding disorder with a prolonged PT

A

Disseminated intravascular coagulation

123
Q

3 bleeding disorders with decreased platelet counts

A

Disseminated intravascular coagulation
Idiopathic Thrombocytopenic Purpura
+/- liver disease

124
Q

3 bleeding disorders with prolonged aPTT

A

Disseminated intravascular coagulation
Hemophilia
von Willebrand Disease

125
Q

Inherited thrombotic disorders (4)

A

Protein C deficiency
Protein S deficiency
Antithrombin deficiency
Factor V Leiden mutation

126
Q

activated protein C

A

inactivates activated factors V and VIII

127
Q

may develop warfarin-induced skin necrosis

A

Protein C deficiency patients

128
Q

may be Homozygous or Heterozygous

A

Protein C deficiency

Protein S deficiency Factor V Leiden mutation

129
Q

Protein S

A

a cofactor for protein C, facilitates its action

130
Q

Factor V Leiden mutation is what kind of mutation

A

point mutation

131
Q

Factor V Leiden mutation pathophysiology

A

mutation makes factor V resistant to inactivation by activated protein C

132
Q

Risk of venous thromboembolism increased

→ even more so if taking oral contraceptives

A

Factor V Leiden mutation

133
Q

antithrombin

A

inhibits thrombin

134
Q

presents with venous thromboembolism and significantly dimished efficiency of heparin

A

antithrombin deficiency

135
Q

Inherited thrombotic disorders as a group:

A

Hypercoagulable → thrombosis, DVTs, PEs, + family history

136
Q

Inherited thrombotic disorders treatment

A

prophylaxis: unfractionated heparin, low molecular weight heparin, warfarin
First episode VTE: 3 months anticoagulation
+/- long term anticoagulation

137
Q

most commmon small vessel vasculitis

A

Henoch-Schonlein Purpura

138
Q

Henoch-Schonlein Purpura pathophysiology

A

Deposition of IgA immune complexes→ small vessels of skin, GI tract, kidneys

139
Q

Risk for Henoch-Schonlein Purpura

A

Boys aged 2-7 years, highest incidence spring and fall

URI often precedes diagnosis

140
Q

palpable purpura, arthritis/arthralgias, abdominal pain, renal disease

A

Henoch-Schonlein Purpura

141
Q

Henoch-Schonlein Purpura labs

A

Elevated/normal platelets
Antistreptolysin O (ASO) titer elevated
+/- elevated serum IgA, + hemoccult

Urinalysis: hematuria +/- proteinuria

142
Q

who can’t take warfarin

A

pregnant patients

Protein C deficiency patients

143
Q

diseases that are preceded by URI/viral infection

A

Henoch-Schonlein Purpura

Idiopathic thrombocytic purpura

144
Q

bone marrow hypoplasia/aplasia

A

fanconia anemia

145
Q

large neutrophils with hypersegmented nuclei

A

megaloblastic anemia

146
Q

macro-ovalocytes

A

megaloblastic anemia

147
Q

everything is normal except thrombocytopenia

A

idiopathic thrombocytopenic purpura

148
Q

everything is more or less normal except prolonged bleeding time

A

von Willebrande disease

vWF may be normal or decreased

149
Q

prolonged aPTT, but it could be normal in mild disease

A

hemophilia

150
Q

elevated antistreptolysin O titer

A

Henoch-Schonlein Purpura

151
Q

+/- serum IgA elevated

+/- +hemoccult

A

Henoch-Schonlein Purpura

Unit 1 Peds II (3 decks)

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