L4 Peds Hematology Flashcards
1
Q
anemia definition
A
2 standard deviations below normal for age and sex
2
Q
anemia definition 6 months - 5 years
A
Hgb concentration <11g/dl
3
Q
Low reticulocyte count means
A
bone marrow isn’t producing RBC to keep up with anemia, “trouble”
4
Q
High reticulocyte count means
A
bone marrow is working
5
Q
Normocytic normochromic anemia
A
anemia of chronic disease
6
Q
Microcytic hypochromic anemia
A
iron defienciy
thalassemia
lead intoxication
7
Q
macrocytic anemia
A
vitamin B12/folate deficiency
8
Q
iron deficiency anemia RBCs
A
Microcytic hypochromic anemia
9
Q
thalassemia anemia RBCs
A
Microcytic hypochromic anemia
10
Q
lead intoxication RBCs
A
Microcytic hypochromic anemia
11
Q
anemia of chronic disease RBCs
A
Normocytic normochromic anemia
12
Q
acute signs of anemia
A
lethargy
tachycardia
pallor
13
Q
acute signs of anemia in infants
A
irritability and poor oral intake
14
Q
chronic anemia presentation
A
may present with few or no symptoms at all
15
Q
other findings in anemia
A
jaundice, gallstone disease, petechiae, purpura, ecchymosis, bleeding
16
Q
Bone marrow failure (2)
A
Fanconi Anemia
Acquired aplastic anemia
17
Q
Fanconi Anemia
A
Inherited bone marrow failure syndrome
Autosomal recessive, defective DNA repair
Majority develop in first 10 years of life
18
Q
Fanconi Anemia Congenital malformations:
A
abnormal pigmentation of skin
short stature
skeletal malformations
19
Q
Progressive pancytopenia
A
Fanconi Anemia
20
Q
Non-anemia symptoms of fanconi anemia
A
Increased incidence of malignancies: Myelodysplastic syndrome (MDS), Acute myeloid leukemia (AML)
21
Q
often misdiagnosed as Idiopathic thrombocytopenic purpura (ITP)
A
Fanconi Anemia
22
Q
Lab findings of fanconi anemia
A
Thrombocytopenia leukopenia
severe aplastic anemia
bone marrow hypoplasia/aplasia
23
Q
which lab finding of fanconi anemia usually occurs before the anemia
A
Thrombocytopenia leukopenia
24
Q
most common nutritional deficiency
A
iron deficiency anemia
25
most common bleeding disorder
idiopathic thrombocytopenic purpura (ITP)
26
fanconi anemia prognosis
mortality due to bleeding, infection, malignancy in adolescence/early adulthood
27
Fanconi Anemia vs Acquired aplastic anemia: pancytopenia
Fanconi Anemia: progressive
| Acquired aplastic anemia: peripheral with a hypocellular bone marrow
28
Causes of Acquired aplastic anemia
Idiopathic (50%), toxic exposure to insecticides, viruses (EBV, Hepatitis, HIV)
29
Signs/symptoms of Acquired aplastic anemia
weakness, fatigue, pallow, frequent/severe infections, purpura, petechiae, bleeding
30
bone marrow failure vs. idiopathic thrombocytopenic purpura (ITP)
ITP gets better after a transfusion, bone marrow failure does not
31
Lab findings in Acquired aplastic anemia
Anemia, usually normocytic
Low WBC with marked neutropenia
Thrombocytopenia
Low reticulocyte count
32
Leading causes of death for acquired aplastic anemia
infection
| severe hemorrhage
33
greatest prevalence of iron deficiency anemia
```
children 1-2 years
Females of childbearing age
African american children
Hispanic children
Poverty
```
34
signs/symptoms of iron defieciency anemia
varies with severity: asymptomatic, pallor, fatigue, irritability, delayed motor development, pica
35
when/how to screen for iron deficiency anemia
12 months: hgb concentration, risk factors
36
at risk for iron deficiency anemia
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
37
labs for iron deficiency anemia
Microcytic, hypochromic anemia
Hemoglobin <11 g/dL
Ferritin <12 mcg/L
38
If hemoglobin is 10-11 mg/dL at 12 month screening
closely monitored or empirically treated
| recheck hemoglobin in 1 month
39
if iron deficiency anemia is diagnosed, tx
iron 6 mg/kg/day, divided into 3 doses
40
if your patient eats a lot of ice chips, think
iron deficiency anemia
41
2 megaloblastic anemias
```
Vitamin B12 (cobalamin) deficiency
Folic Acid deficiency
```
42
megaloblastic anemia aka
macrocytic anemia
43
Vitamin B12 aka
cobalamin
44
causes of B12 defieciency
```
intestinal malabsorption (IBD)
dietary insufficiency
```
45
causes of Folic acid deficiency
increased folate requirement, malabsorptive syndromes, inadequate dietary intake (rare), medications
46
increased folate requirement is seen in
rapid growth, chronic hemolytic anemia
47
signs/symptoms of megaloblastic anemia
Glossitus
| Pallor
48
neurologic signs such as ________ ONLY occur with _______
decreased vibratory sensation and proprioception, parethsesias, weakness, unsteady gait
**B12 deficiency**
49
Labs of megaloblastic anemia
Elevated MCV, MCH
Larger, hypersegmented neutrophils
Macro-ovalocytes (RBC)
Elevated homocysteine
50
Labs of B12 deficiency
elevated methylmalonic acid and homocysteine
51
labs of folate deficiency
elevated homocysteine without elevated methylmalonic acid
52
if you give folate to a B12 deficient patient
clinical symptoms and anemia will improve, but it will not avoid permanent neurological deficity
53
Red cell membrane defect→ hemolytic anemia → jaundice, splenomegaly, gallstones
Spherocytes
Increased osmotic fragility
Hereditary spherocytosis
54
may be treated with splenectomy
Hereditary spherocytosis
| +/- thalassemia
55
Thalassemia RBC
Microcytic, hypochromic anemia
56
needed for diagnosis of thalassemia
hemoglobin electrophoresis
57
when would a thalassemia patient get a hematopoietic stem cell transplant
severe beta thalassemia
58
pain in sickle cell is caused by
vaso-occlusion
59
Splenomegaly
| Splenic infarcts → functional asplenia
sickle cell anemia
60
+/- growth failure and delayed puberty
sickle cell anemia
61
Hydroxyurea
treatment of sickle cell anemia
62
Episodic hemolysis: exposure to oxidant stress of infection, certain drugs and foods
G6PD
63
Red cell enzyme defect→ hemolytic anemia→ pallor, jaundice
| X linked recessive inheritance
G6PD
64
Labs:
Hyperbilirubinemia
episodic hemolysis → hemoglobinuria
G6PD
65
```
Peripheral smear:
Bite like deformities
Heinz bodies (denatured hemoglobin)
```
G6PD
66
lead poisoning anemia is
Mild, hemolytic, normocytic anemia
67
Peripheral smear: Basophilic stippling
lead poisoning
68
lead poisoning results from
lead paint in old houses
69
Familial Polycythemia aka
Congenital Erythrocytosis
or
Primary polycthemia
70
Familial polycythemia affects
ONLY RBC
71
Congenital Erythrocytosis symptoms
Headache, lethargy
| +/- plethora (swelling), splenomegaly
72
Causes of secondary polycythemia
Occurs in response to hypoxemia:
Cyanotic congenital heart disease
Chronic pulmonary disease (cystic fibrosis)
73
Initial screening tests for bleeding disorders
CBC, Peripheral smear, PT/INR, aPTT, +/- bleeding time
74
Normal platelet count
150,000-400,000/mm3
75
platelet count at risk of spontaneous bleeding
<20,000
76
measure extrinsic and common pathways
PT
77
measures intrinsic and common pathways
PTT/aPTT
78
extrinsic and common pathways
I, II, V, VII, X, Tissue factor
79
intrinsic and common pathways
I, II, V, VIII, IX, X, XI, XII
80
common pathway
II, IIa, I, V, X, phospholipids, XIII
81
Intrinsic pathway
VIII, IX, XI, XII
82
Extrinsic pathway
Tissue factor
| VII
83
more accurate reflection of PT used to monitor warfarin treatment
INR
84
measure of time for hemostasis, screening test for platelet dysfunction. Prolonged in platelet disorders
Bleeding time
85
Disorders with prolonged bleeding times
von Willebrand disease, severe thrombocytopenia
86
Most common bleeding disorder of childhood, most frequently 2-5 years old
Idiopathic Thrombocytopenic Purpura (ITP)
87
Idiopathic Thrombocytopenic Purpura (ITP) cause
Often follows infection with viruses→ immune mediated: autoantibodies bind to platelets → phagocytized by splenic macrophages → decreased platelet lifespan
88
Idiopathic Thrombocytopenic Purpura (ITP) prognosis
90% have spontaneous remission
89
Idiopathic Thrombocytopenic Purpura (ITP) signs/symptoms
multiple petechiae, ecchymosis, epistaxis
90
Your patient had a viral infection and now is getting a bunch of nosebleeds, what's your ddx?
Idiopathic Thrombocytopenic Purpura (ITP)
91
Idiopathic Thrombocytopenic Purpura labs
Thrombocytopenia
Normal WBC, hemoglobin, PT, aPTT
Hemorrhage → low hemoglobin
92
How to diagnose Idiopathic Thrombocytopenic Purpura
diagnosis of exclusion
93
if petechiae are observed, always
check platelets to check for Idiopathic Thrombocytopenic Purpura
94
medications that compromise platelet function
Aspirin
| NSAIDS
95
Idiopathic Thrombocytopenic Purpura treatment
```
avoid meds that compromise platelet function
Bleeding precautions
Prednisone
IVIG
Splenectomy
```
96
who to observe with Idiopathic Thrombocytopenic Purpura
asymptomatic children
97
most common inherited bleeding disorder
Von Willebrand Disease
| autosomal dominant, 1% prevalence
98
Von Willebrand disease pathophysiology
Decrease in level of or impairment in the action of von Willebrand Factor (vWF)
99
most common type of Von Willebrand disease
type 1 (of 3 major types)
100
von Willebrand factor
binds to factor VIII and is a cofactor for platelet adhesion to the endothelium
101
Von willebrand disease presentation
prolonged bleeding from mucosal surfaces, epistasis, menorrhagia, GI, easy bruising
102
Von willebrand disease labs
Prolonged/normal aPTT
Decreased/normal Factor VIII
Decreased/normal vWF
Prolonged bleeding time
103
Von Willebrand disease vs hemophilia: bleeding time
Von Willebrand disease: prolonged
| Hemophilia: normal
104
Desmopressin MOA
Causes release of vWF and factor VIII from endothelial stores
105
Von Willebrand disease treatment
Desmopressin
| vWF replacement therapy
106
factor VIII deficiency
Hemophilia A
107
factor IX deficiency
Hemophilia B
108
Christmas disease aka
Hemophilia B
109
factors VIII and IX are found in the
intrinsic pathway
110
most common sites of bleeding in hemophilia
into joints and muscles
111
severe hemophilia can present with
spontaneous hemarthrosis→ joint destruction
112
Hemophilia labs
Normal platelet count, PT, bleeding time, vWF
Prolonged aPTT (can be normal in mild disease)
113
Hemophilia A treatment
desmopressin
114
to achieve sufficient hemostasis in patients with hemophilia, treat with
Factor replacement (VII, IX)
115
Disseminated intravascular coagulation pathophysiology
Triggered by sepsis, trauma/tissue injury, or malignancies→ widespread activation of coagulation cascade → microthrombi→ consumption of platelets → hemorrhage
116
Disseminated intravascular coagulation labs
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
Disseminated intravascular coagulation treatment
Replacement therapy
| Anticoagulant therapy
118
the liver synthesizes
prothrombin
fibrinogen
Factors V, VII, IX, X, XII, XIII
119
hallmark of Disseminated intravascular coagulation on labs
Elevated D dimer
| Elevated fibrin degradation product (FDPs)
120
vitamin-K dependent factors
II, VII, IX, X
121
vitamin K deficiency vs liver disease on labs:
vitamin K: normal platelet count
liver disease: normal/low platelet count
both: prolonged PT, aPTT
122
the one bleeding disorder with a prolonged PT
Disseminated intravascular coagulation
123
3 bleeding disorders with decreased platelet counts
Disseminated intravascular coagulation
Idiopathic Thrombocytopenic Purpura
+/- liver disease
124
3 bleeding disorders with prolonged aPTT
Disseminated intravascular coagulation
Hemophilia
von Willebrand Disease
125
Inherited thrombotic disorders (4)
Protein C deficiency
Protein S deficiency
Antithrombin deficiency
Factor V Leiden mutation
126
activated protein C
inactivates activated factors V and VIII
127
may develop warfarin-induced skin necrosis
Protein C deficiency patients
128
may be Homozygous or Heterozygous
Protein C deficiency
| Protein S deficiency Factor V Leiden mutation
129
Protein S
a cofactor for protein C, facilitates its action
130
Factor V Leiden mutation is what kind of mutation
point mutation
131
Factor V Leiden mutation pathophysiology
mutation makes factor V resistant to inactivation by activated protein C
132
Risk of venous thromboembolism increased
| → even more so if taking oral contraceptives
Factor V Leiden mutation
133
antithrombin
inhibits thrombin
134
presents with venous thromboembolism and significantly dimished efficiency of heparin
antithrombin deficiency
135
Inherited thrombotic disorders as a group:
Hypercoagulable → thrombosis, DVTs, PEs, + family history
136
Inherited thrombotic disorders treatment
prophylaxis: unfractionated heparin, low molecular weight heparin, warfarin
First episode VTE: 3 months anticoagulation
+/- long term anticoagulation
137
most commmon small vessel vasculitis
Henoch-Schonlein Purpura
138
Henoch-Schonlein Purpura pathophysiology
Deposition of IgA immune complexes→ small vessels of skin, GI tract, kidneys
139
Risk for Henoch-Schonlein Purpura
Boys aged 2-7 years, highest incidence spring and fall
| URI often precedes diagnosis
140
**palpable purpura**, arthritis/arthralgias, abdominal pain, renal disease
Henoch-Schonlein Purpura
141
Henoch-Schonlein Purpura labs
Elevated/normal platelets
Antistreptolysin O (ASO) titer elevated
+/- elevated serum IgA, + hemoccult
Urinalysis: hematuria +/- proteinuria
142
who can't take warfarin
pregnant patients
| Protein C deficiency patients
143
diseases that are preceded by URI/viral infection
Henoch-Schonlein Purpura
| Idiopathic thrombocytic purpura
144
bone marrow hypoplasia/aplasia
fanconia anemia
145
large neutrophils with hypersegmented nuclei
megaloblastic anemia
146
macro-ovalocytes
megaloblastic anemia
147
everything is normal except thrombocytopenia
idiopathic thrombocytopenic purpura
148
everything is more or less normal except prolonged bleeding time
von Willebrande disease
| *vWF may be normal or decreased*
149
prolonged aPTT, but it could be normal in mild disease
hemophilia
150
elevated antistreptolysin O titer
Henoch-Schonlein Purpura
151
+/- serum IgA elevated
| +/- +hemoccult
Henoch-Schonlein Purpura
