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
2
Q
How common is anemia in kids?
A
20% US kiddos
80% worldwide kiddos
3
Q
Reticulocyte count reflects _____.
Normal percent blood count made up of retics?
A
number of immature RBCs/ activity of bone marrow
normally 1%.
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)
5
Q
Causes of IDA in kiddos
A
- lack of dietary iron
- early ingestion of cows milk
- occult blood loss
- menstruation
6
Q
2 weird key symptoms of anemia
A
spoon shaped nails
diminished attention
also paleness, fatigue etc
7
Q
Labs assc with IDA:
A
- low ferritin (early)
- increased transferrin
- decreased transferrin saturation
- increased free erythrocyte protoporphyrin
8
Q
3 causes of macrocytic anemia
A
-B12
-folate
-thiamine
deficiencies
9
Q
Normocytic normochromic anemia with low retic counts
A
- malignancy
- fanconis
- red cell aplasias
10
Q
Three types of red cell aplasia
A
TEC
Diamond Blackfan
Parvo B19
11
Q
When should workup to determine cause of anemia begin?
A
When anemia is not responsive to iron therapy.
12
Q
HbA1 makeup
A
A2B2
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)
14
Q
What populations are predisposed to a/b thal?
A
A: Asians
B: Meditteraneans
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)
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!)
17
Q
Labs assc with B Thal
A
- increased HbF
- low HbA1
- target cells
- high bili/LDH (hemolysis)
18
Q
Complication assc with thalassemia treatment and how to prevent it?
A
- hemochromatosis due to chronic transfusion
- prevent with deferoxamine
19
Q
Iron level in B thal minor
A
-normal to high
20
Q
Sideroblastic anemia:
basic pathologic cause
A
iron in the mitochondria
21
Q
4 causes of of acquired sideroblastic anemia
A
- lead
- isoniazid
- alcohol
- chloramphenicol
22
Q
B12:
- cogactor for absorption + source
- site of absorption
A
- intrinsic factor, gastric parietal cells
- terminal ileum
23
Q
2 weird manifestations of B12 def
A
- beefy red tongue
- neuro findings
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)
25
Three types of aplastic anemia
- malignancy
- red cell aplasia
- drug suppression
26
Hereditary spherocytosis:
- inheritance pattern
- assc protein
- AD
| - spectrin
27
3 features assc with hereditary spherocytosis:
- pigmentary gallstones
- aplastic crises
- splenomegaly
28
Test assc with hereditary spherocytosis:
-osmotic fragility studies
29
Condition aside from hereditary spherocytosis that is AD And assc with spectrin?
hereditary elliptocytosis
30
Enzymatic defects of RBCs:
| + which is most common?
- G6PD*
| - pyruvate kinase
31
Enzymatic and RBC structural defects are classified as what type of anemia?
hemolytic
32
PK deficiency:
- leads to depletion of ____.
- RBC appearance ______.
- treatment
- ATP
- polychromic
- transfusion/ splenectomy
33
Three drugs assc with G6PD crisis + how far from exposure do symptoms begin?
nitrofurantoin
sulfa drugs
antimalarials
fava beans
24-48 hours
34
G6PD smear appearance
- bite cells
| - Heinz bodies
35
AIHA:
- frequent cause of acute AIHA
- assc test
- treatment
- respiratory infection, virus, drugs
- positive direct coombs
- steroids = rapid complete recovery
36
Secondary AIHA/ chronic causes:
- lymphoma
- SLE
- Immunodeficiency
37
Two most common causes of alloimmune hemolytic anemia
- Rh Hemolytic Disease (mom type -)
| - ABO hemolytic Disease (mom type O)
38
Test that distinguishes Rh Hemolytic Disease from ABO -
direct coombs
- strongly positive in Rh
- weakly positive in ABO
39
Microangiopathic Hemolytic Anemia:
- type of damage
- four assc conditions
- mechanical damage
| - HUS, artificial heart valves, hemangioma, DIC
40
Two types of cells seen on Micro HA smear:
- burr cells
| - target cells
41
Gene mutation assc with sickle cell
VGB6
| valine --> glutamic acid B6 chain
42
When does sickle cell become symptomatic?
~6-9 months when HbF declines
43
Leading cause of death in sickle cell
-infection by encapsulated bacteria due to decreased splenic function
44
Bacteria dangerous to SS patients
- strep pneumo
- H flu
- Neisseria
45
5 weird sickle cell complications
- dactylitis
- salmonella osteomyelitis
- stroke
- priapism
- acute chest syndrome
46
SCA Sequestration crisis:
- labs
- symptoms
- low Hb
- high retics
- abdominal pain and distention
- shock
- usually kiddos under
47
Blood smear findings assc with SCA
- Howell jolly bodies
- sickled cells
- target cells
48
5 Preventative measures in SCA
- hydroxyurea to increase HbF
- penicillin px
- folic acid
- immunization (esp flu, pneumo)
- serial transcranial DUS
49
Bone complication aside from osteo in sickle cell anemia
avascular necrosis of the femoral head
50
Three nonmalignant RED cell aplasias (not pancytopenia
- transient erythroblastopenia of childhood (TEC)
- congenital hypolpastic anemia (Diamond Blackfan)
- parvovirus B19
51
CHA? Diamond Blackfan:
- inheritance pattern
- lab value that is increased
- physical exam findings (3)
- two other involved organ systems
- AD/AR
- high HbF
- short, triphalangeal thumbs, craniofacial changes
- renal, cardiac changes
52
Possible cause of transient erythroblastopenia of childhood + prognosis
-viral infection --> spontaneously resolves, requires no treatment
53
Findings assc with parvovirus B19 aside from RBC aplasia? Another name?
- slapped cheeks
- lacy rash
- fifths disease
54
Congenital cause of pancytopenia +inheritance pattern
-fanconi anemia, AR
55
Skeletal +renal abnormalities assc with Fanconis
- absence/ hypoplasia of thumb + radius
- type 2/ proximal RTA (inability to reabsorb bicarb)
**also see skin pigmentation
56
Drugs causing acquired aplastic anemia
- anticonvulsants
- chloramphenicol
- sulfas
57
Infections causing acquired aplastic anemia
- HIV
- EBV
- CMV
58
Two types of polycythemia:
- primary (poly vera, malignant)
| - secondary (increased EPO)
59
Cause of appropriate polycythemia:
- hypoxemia
| - pulm disease
60
EPO/ inappropriate poly can be cause by malignancy of which organs?
- cerebellum/ kidney
- ovary
- adrenal
- liver
(C/KOAL)
61
Appearance assc with polycythemia + 2 complications:
- ruddy complexion
- thrombosis
- bleeding
62
Cause of relative polycythemia
-dehydration most commonly
63
Three factors assc with hemostasis
- vessels
- platelets
- clotting factors
64
Hemophilia A and vWF are both considered _____.
What are their inheritance patterns?
Which is most common?
Factor VIII disorders
A: X linked; vWF: AD
vWF = MC bleeding disorder
65
Contrast lab findings in Hemophilia A/ vWF Disease
- both have prolonged PTT, normal PT
- only vWF has prolonged bleeding time
- both have normal platelet count
66
Which two bleeding disorders are assc with hemarthroses?
- Factor VIII/ Hemophilia A
| - Vitamin K deficiency
67
Order of intrinsic pathway clotting
-12,11,9,8,10
68
Order of extrinsic pathway clotting
7,10
69
Order of common pathway clotting
10,2 (thrombin),1 (fibrin)
70
Factor required for making fibrin polymer
XIII
71
What are the three types of vWF disease?
| Which is most common?
type 1: classic, quantitative***
type 2: qualitative
type 3: absence
72
Test for vWF activity?
| Treatment?
ristocetin cofactor
| DDAVP + cryo
73
Hemophilia B:
- aka
- cause
- inheritance
- Christmas disease
- factor IX deficiency
- X linked
74
Three acquired clotting factor disorders
- vitamin K def
- liver disease
- DIC
75
Three congenital clotting factor disorders
- Hemo A/ VIII
- Hemo B/ IX
- vWF (most common)
76
Clotting factors that require vitamin K
-2,7,9,10, C, S
| PT, PTT both affected by vit K deficiency
77
Meds that classically depletes vitamin K
warfarin
78
Signs of vitamin K def in newborn
- bleeding from circumcision + umbilical stump
| - hematemesis
79
Hematologic findings in liver disease/DIC
- ^^ PT,PTT
- ^^fibrin degradation products
- thrombocytopenia
80
Findings specific to DIC:
-helmet cells
-fragmented RBCs
(not seen in liver disease)
81
Thrombocytopenia findings
- increased bleeding time
- low platelets
- petechiae
82
Which of the platelet disorders are assc with petechiae?
- DIC, liver, vit K, thrombocytopenia
| - not vWF/ hemophilias
83
Which of the platelet disorders have normal bleeding times?
-all prolonged except
| Heme A, vitamin K= normal
84
Kasabach-Merritt Syndrome:
| describe
Large hemangioma --> DIC
85
Five Vessel abnormalities that present with bleeding:
- HSP
- HHT
- Scurvy
- Collagen D/O (i.e. ED)
- Malnutrition/steroids
86
Hereditary Hemorrhagic Telangiectasia
| Inheritance pattern
AD
87
Most common cause of bleeding
thrombocytopenia
88
Two congenital disorders causing low platelets
- Wiskott Aldrich
| - TAR
89
Wiskott Aldrich:
inheritance pattern
3 findings
- X linked
- thrombocytopenia
- low B/T cell immunity
- eczema
90
TAR syndrome:
- inheritance pattern
- 4 findings
- thrombocytopenia
- absent radius
- cardiac disease
- renal disease
91
Two diseases causing increased platelet destruction
- ITP
| - Neonatal Immune mediated thrombocytopenia
92
Immune thrombocytopenic purpura:
- when does it most commonly occur?
- prognosis?
- 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
Smear finding in ITP
few large sticky platelets
94
Two types of neonatal immune thrombo
- passive (mother has low platelets)
| - isoimmune (mother has normal platelets)
95
Two "syndromes" assc with low platelets
- Kasabach Merritt
| - HUS
96
Three misc causes of low platelets
- DIC
- large spleen
- drugs
97
Two drugs that interfere with platelet function
- aspirin
| - valproate
98
Two systemic diseases that impair platelet function
- liver disease
| - uremia
99
Two congenital disorders effecting platelet function + their inheritance pattern
-Glanzmann
-Bernard Soulier
both AR
100
Defect in Glanzman/ Bernard Soulier
- Glanzmann: lack of IIb/IIIa, = no aggregation
| - BS: lack of membrane glycoprotein= no adhesion
101
Most common childhood platelet disorder
ITP
102
Four factors most commonly altered in hypercoagulable disorders
- protein C
- protein S
- factor V
- antithrombin III
103
Presentations for homozygotes lacking Protein C
-purpura fulminans early in life (rapidly spreading bleeding)
104
Neutropenia predisposes to what infections?
- moderate: mucus membrane/ skin infection
| - severe: sepsis, gram neg or s. aureus
105
What is considered severe neutropenia?
ANC less than 500 cells/mm^3
106
Most common cause neutropenia in childhood
infection
107
Chronic benign neutropenia of childhood:
age group
prognosis
key distinguishing feature of disease
- less than 4
- resolves spontaneously within months --> years
- neutropenia is NONCYCLICAL
108
Kostmann syndrome:
- aka
- inheritance pattern
- severe congenital agranulocytosis
| - AR
109
Cyclical neutropenia:
- inheritance pattern
- features during neutropenic episode
- AD
| - Fever, ulcers, stomatitis
110
Three genetic "syndromes" assc with neutropenia + inheritance pattern
- Chediak Higashi
- Cartilage hair hypoplasia syndrome
- Schwachmann Diamond
- both are AR
111
Chediak Higashi
- PE findings
- Smear findings
- albinish
| - blue granules in neutrophil cytoplasm + neutropenia
112
Four features of Cartilage hair hypoplasia syndrome
- immunodeficiency
- thin hair
- short stature
- neutropenia
113
Schwachman Diamond Syndrome:
| -4 features
- exocrine pancreatic insufficiency
- short stature
- neutropenia
- metaphyseal chrondrodysplasia
114
Five causes of increased neutrophil destruction
1) infection
2) drugs
3) hypersplenism
4) autoimmune neutropenia
5) isoimmune neutroplenia
Pediatrics
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