BRS- Heme Flashcards Preview

Pediatrics > BRS- Heme > Flashcards

Flashcards in BRS- Heme Deck (114):
1

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

2-3 months= nadir
6-9 months= HbF disappearance

2

How common is anemia in kids?

20% US kiddos
80% worldwide kiddos

3

Reticulocyte count reflects _____.
Normal percent blood count made up of retics?

number of immature RBCs/ activity of bone marrow
normally 1%.

4

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

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

5

Causes of IDA in kiddos

-lack of dietary iron
-early ingestion of cows milk
-occult blood loss
-menstruation

6

2 weird key symptoms of anemia

spoon shaped nails
diminished attention
also paleness, fatigue etc

7

Labs assc with IDA:

-low ferritin (early)
-increased transferrin
-decreased transferrin saturation
-increased free erythrocyte protoporphyrin

8

3 causes of macrocytic anemia

-B12
-folate
-thiamine
deficiencies

9

Normocytic normochromic anemia with low retic counts

-malignancy
-fanconis
-red cell aplasias

10

Three types of red cell aplasia

TEC
Diamond Blackfan
Parvo B19

11

When should workup to determine cause of anemia begin?

When anemia is not responsive to iron therapy.

12

HbA1 makeup

A2B2

13

PE finding in thalassemias

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

14

What populations are predisposed to a/b thal?

A: Asians
B: Meditteraneans

15

How many types of A thal exist?
B thal?

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

16

What are the two most severe types of a thal?

-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

Labs assc with B Thal

-increased HbF
-low HbA1
-target cells
-high bili/LDH (hemolysis)

18

Complication assc with thalassemia treatment and how to prevent it?

-hemochromatosis due to chronic transfusion
-prevent with deferoxamine

19

Iron level in B thal minor

-normal to high

20

Sideroblastic anemia:
basic pathologic cause

iron in the mitochondria

21

4 causes of of acquired sideroblastic anemia

-lead
-isoniazid
-alcohol
-chloramphenicol

22

B12:
-cogactor for absorption + source
-site of absorption

-intrinsic factor, gastric parietal cells
-terminal ileum

23

2 weird manifestations of B12 def

-beefy red tongue
-neuro findings

24

Three classes of normocytic anemia + how to distinguish them?

-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