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Pediatrics > BRS Hematology > Flashcards

BRS Hematology Flashcards

0
Q

when does fetal hemoglobin go away

A

by 6-9 months of age

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1
Q

describe hgb levels at birth and afterwards

A

high at birth and normally declines, reaches low between 2-3 months in term infant and between 1-2 months in a premie
-hgb reaches adult level after puberty

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2
Q

usually, retics make up ___% of RBCs

A

1%

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3
Q

MC blood disease during infancy and childhood

A

iron deficiency anemia mostly 2/2 inadequate intake

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4
Q

nutritional iron deficiency most common in these two age groups

A
  1. 9-24 months- inadequate intake, low iron stores

2. adolescent girls- poor diet, rapid growth, loss of iron in menstrual blood

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5
Q

spoon-shaped nails, diminished attention and ability to learn, pale

A

anemia

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6
Q
  • early finding of iron deficiency anemia

- as serum iron decreases, you see these lab findings

A
  • low serum ferritin- b/c iron stores disappear first
  • iron binding capacity increases –> increased transferrin and decreased transferrin saturation
  • may note increased free erythrocyte protoporphyrin
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7
Q

how to tx iron deficiency anemia

A

elemental iron; give with vitamin C to enhance absorption
dietary counseling
if anemia doesn’t improve, then you have to look for another cause

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8
Q

normally, most hemoglobin is hemoglobin ______, which is a tetramer containing _____ and ______

A

A1

2 alpha chains and 2 beta chains

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9
Q

what is wrong in alpha-thalassemia

what is wrong in beta-thalassemia

A

defective alpha-globin chain synthesis

defective beta-globin chain synthesis

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10
Q

how does thalassemia result in abnormal bones?

A

hemolysis –> increased bone marrow activity –> marrow space enlarges –> large bones in the face, skull, etc

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11
Q

alpha-thalassemia primarily occurs in this ethnic group

beta-thalassemia primarily occurs in this ethnic group

A

SE Asian

mediterranean

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12
Q

4 types of alpha-thalassemia

A
  1. silent carrier- 1 alpha gene deleted, no anemia, no sxs
  2. alpha thalassemia minor- 2 alpha genes deleted, mild anemia
  3. hgb H disease- 3 alpha genes deleted, pts have severe anemia at birth with elevated Hgb Bart’s (binds oxygen very strongly and does not release it to the tissue), anemia is lifelong and severe
  4. fetal hydrops- 4 alpha genes deleted, only Hgb Bart’s is formed which leads to in utero anemia, CHF, and death
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13
Q

2 types of beta-thalassemia

A

beta thalessemia major and minor

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14
Q

hemolytic anemia in infancy, marked HSM, bone marrow hyperplasia –> frontal bossing, maxillary hyperplasia with prominent cheekbones, skull deformities

A

beta-thalassemia major (Cooley’s anemia or homozygous beta-thalassemia)
-total absence of beta chains or deficient beta chain production

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15
Q

lab findings of beta-thalassemia major

A
  • severe hypochromia and microcytosis, target cells and poikilocytes
  • elevated unconjugated bili, serum iron, and LDH
  • low or absent Hgb A and elevated Hgb F
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16
Q

how to tx beta thalassemia major

A

lifelong transfusions, splenectomy, +/- BMT

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17
Q

a potential complication of beta thalassemia major

how do you tx it

A

hemochromatosis

  • increased iron absorption from intestine
  • more iron in transfused RBCs

tx with deferoxamine (chelator)

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18
Q

this causes mild asymptomatic anemia with Hgb levels 2-3 g/dL below age-appropriate norms
-hypochromia and microcytosis with target cells and anisocytosis

A

beta thalassemia minor

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19
Q

how to tx beta thalassemia minor

A

no tx needed

*note, this is often mistaken for iron deficiency anemia… however, iron levels are actually normal or elevated

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20
Q

ring sideroblasts in the bone marrow 2/2 accumulation of iron in the mitochondria of RBC precursors
what is it and what might it be caused by?

A

sideroblastic anemia

-inherited or caused by toxins

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21
Q 
iron deficiency anemia
thalassemia
sideroblastic anemia
lead toxicity
chronic disease
A

causes of microcytic, hypochromic anemia

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22
Q

B12 deficiency
thiamine deficiency
folate deficiency

A

causes of macrocytic anemia

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23
Q

macrocytic anemias have large RBCs and MCV > ______

A

95

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24
Q

causes of folic acid deficiency

A
  • decreased folic acid intake (no fresh fruits and beggies, exclusive goat’s milk)
  • decreased intestinal absorption of folic acid- small intestine dzs, anticonvulsants, OCPs
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25
Q

how to dx and tx folic acid deficiency

A

dx with low serum folic acid

tx with dietary folic acid and tx the underlying cause

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26
Q

describe normal vitamin B12 absorption

A

B12 combines with intrinsic factor and then is absorbed in terminal ileum

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27
Q

causes of B12 deficiency

A
  • inadequate dietary intake (vegan diet)
  • inability to secrete intrinsic factor (juvenile pernicious anemia)
  • inability to absorb B12 (ex. Crohn’s disease)
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28
Q

anemia, smooth red tongue, neuro issues (ataxia, hyporefliexia, positive Babinski)

A

B12 deficiency

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29
Q

how to dx and tx B12 deficiency anemia

A

dx with low serum B12 level

tx with monthly IM B12 injections

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30
Q

splenomegaly by age 2-3 years, pallor, weakness, pigmentary gallstones, aplastic crises assoc with parvovirus B19 infection

what is it, what’s the demographic, how is it inherited, what protein is affected

A

hereditary spherocytosis
Northern Europeans
AD disorder
spectrin abnormality or deficiency

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31
Q

how to dx hereditary spherocytosis

A

spherocytes on blood smear

osmotic fragility studies

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32
Q

how to tx hereditary spherocytosis

A

transfusions

splenectomy is curative but should be delayed until > 5 years 2/2 risk of infection with encapsulated bacteria

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33
Q

mostly asymptomatic, elliptical RBCs on blood smear

what is it, how is it inherited, what protein is messed up, how do you tx it

A

hereditary elliptocytosis
AD
spectrin
tx: splenectomy for pts with chronci hemolysis; if no hemolysis, no tx is needed

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34
Q

AR disorder that results in decreased production of an enzyme, leading to ATP depletion and decreased RBC survival
what is it, what are the clinical features, what do you see on blood semar

A

pyruvate kinase deficiency- pallor, jaundice, splenomegaly

blood smear shows polychromatic RBCs

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35
Q

how to dx and tx pyruvate kinase deficiency

A

dx with decreased PK activity in RBCs

tx with transfusions and splenectomy for severe disease

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36
Q

acute hemolytic disease induced by infection or medications or chronic hemolytic disease

A

glucose-6-phosphate dehydrogenase deficiency (G6PD)

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37
Q

triggers for G6PD deficiency

A

infections
fava beans
drugs- sulfa, salicylates, antimalarials

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38
Q

hemolysis with abdominal pain, V/D, fever, and hemoglobinuria followed by jaundice, +/- HSM 24-48 hours after exposure to oxidant

A

G6PD deficiency

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39
Q

bite cells, hemighosts, Heinz bodies
hemoglobinuria
increased retic count

A

G6PD deficiency

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40
Q

how to dx and tx G6PD deficiency

A

dx- low G6PD in RBCs

tx- transfusions as needed; splenectomy not helpful

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41
Q

2 types of autoimmune hemolytic anemia

A

fulminant acute type

  • infants and young children
  • preceded by respiratory infection
  • acute onset of apllor, jaundice, hemoglobinuria, splenomegaly
  • complete recovery is expected

prolonged type
-protracted course, high mortality, underlying disease is often present

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42
Q

lab findings of autoimmune hemolytic anemia

A

anemia, spherocytes, reticulocytosis, direct Coombs test

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43
Q

how to tx autoimmune hemolytic anemia

A

transfusions for transient relief

steroids- esp good for acute form of AIHA

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44
Q

mother’s second baby has severe jaundice, anemia, HSN, hydrops fetalis, strongly positive Coombs

A

Rh hemolytic disease

-mom is Rh negative and produces Rh antibodies from the first pregnancy

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45
Q

in ABO hemolytic disease, a direct Coombs test is _______

it can occur during the first pregnancy

A

weakly positive

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46
Q

tx for Rh and ABO hemolytic disease

A

phototherapy if mild-moderate jaundice
exchange transfusion for severe jaundice
**prevent kernicterus

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47
Q

severe HTN, HUS, artificial heart valves, giant hemangioma, DIC can all cause ______
lab findings of this condition
how to tx

A

MAHA
anemia (burr cells, target cells, irregularly shaped RBCs), thrombocytopenia
supportive care; tx the underlying cause

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48
Q

sickle cell disease is caused by a single AA substitution of _____ for _______ on the number 6 position of the ______ chain of Hgb

A

valine for glutamic acid

beta-globin chain

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49
Q

pathophys of sickle cell disease

A

Hgb stacks when exposed to low O2 or acidosis –> distorted RBC shape –> hemolysis and occlusion of small vessels

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50
Q

persons with SS trait have these types of Hgb

clinical features of SS trait

A 
Hgb A (50-60%)
Hgb S (35-45%)
Hgb F (small amount)
-pts usually asymptomatic unless exposed to severe hypoxemia
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51
Q

how to dx sickle cell disease

A

electrophoresis shows Hgb S and Hgb F in the newborn

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52
Q

SS disease does not present until _____ of age 2/2 protective effects of Hgb F

A

6 months

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53
Q

most common crisis in SS disease

how to manage it

A

vaso-occlusive crisis/painful bone crisis

-pain control, IVF, +/- partial exchange transfusion

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54
Q

what is acute abdominal crisis in SS disease

how to tx it

A

abdominal pain and distention 2/2 sickling within mesenteric artery
tx with pain control, IVF, +/- partial exchange transfusion

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55
Q

dysarthrai, hemiplegia in SS disease patient

what is it and how to tx

A

stroke

tx with urgent exchange transfusion and start chronic transfusion program to prevent recurrence

56
Q

always consider _____ in any pt presenting with priapism

A

SS disease

57
Q

acute chest syndrome in SS disease

define it and tx it

A

new pulmonary infiltrate associated with respiratory sxs

-hydration, pain management, abx, O2, incentive spirometry, partial exchange transfusion

58
Q

sequestration crisis in SS disease

what is it and how to tx

A

rapid accumulation of blood in spleen or less commonly the liver
tx with transfusion of RBCs +/- splenectomy

59
Q

aplastic crisis in SS disease

what is it and how to tx

A

temporary cessation of RBC production often caused by parvovirus B19 or other infectious agent
-tx with transfusion of RBCs

60
Q

hyperhemolytic crisis in SS disease

what is it and how to tx

A
  • rapid hemolysis –> fatigue, pallor, tachycardia, jaundice
  • tx with transfusion of RBCs
61
Q

which SS disease crises can you do an RBC transfusion and not exchange transfusion

A

sequestration crisis
aplastic crisis
hyperhemolytic crisis

62
Q

_____ is the leading cause of death from SS disease

what are the 4 main causes of this

A

infection 2/2 decreased splenic function

-HIB, strep pneumo, salmonella, Neisseria meningitidis

63
Q

osteomyelitis in SS disease may mimic painful bone crisis and is usually caused by this organism

A

salmonella from GI tract

less likely is staph aureus

64
Q

preventative care for SS disease (3 drugs and 2 other interventions)

A
  • hydroxyurea- increases Hgb F –> decrease incidence of vasoocclusive crises
  • daily PCN ppx against strep pneumo infection
  • folic acid supplementation
  • routine vaccines + prevnar/pneumovax and menactra
  • serial transcranial doppler US or MRA to look for pts at risk for stroke
66
Q

sickle cell-hemoglobin C disease is less severe/more severe than SS disease

A

less severe

67
Q

symptomatic anemia within first year of life
rapid onset
often with physical findings: craniofacial, renal, cardiac anomalies; short stature; triphalangeal thumbs
decreased Hgb and retics
what is it and how to tx?

A

congenital hypoplastic anemia (Diamond-Blackfan anemia)

-tx with RBC transfusion, steroids, and BMT if no response to steroids

68
Q

slow onset of symptomatic anemia > 1 year of age
decreased Hgb and retics
what is it and how to tx?

A

transient erythroblastopenia of childhood (TEC)

-no treatment needed… spontaneous recovery

69
Q

asymptomatic anemia after URI ad slapped cheek
can present as aplastic crisis in kids with SS disease
decreased Hgb and retics
what is it and how to tx?

A

parvovirus B19 associated pure RBC aplasia

  • tx with RBC transfusion for SS disease pts in aplastic crisis
  • otherwise, no tx needed
70
Q

-pancytopenia
-ecchymosis and petechiae around age 7
-short stature, absence of hypoplasia of the thumb and radius
-skin hyper pigmentation
-renal abnormalities
what is it

A

Congenital aplastic anemia (Fanconi anemia)

AR inheritance

71
Q

how to tx fanconi anemia

A

RBC and platelet transfusions PRN
BMT from HLA-compatible person if possible
immunosuppressives like steroids may help

72
Q

causes of acquired aplastic anemia

A 
drugs- sulfonamides, anticonvulsants, chloramphenicol
infections- HIV, EBV, CMV
chemicals
radiation 
idiopathic
73
Q

how to tx acquired aplastic anemia

A

stop the causative agent
transfusions PRN
BMT
immunosuppressive therapy

74
Q

define polycythemia

A

increase in RBCs relative to total blood volume

Hct > 60%

75
Q

what is polycythemia vera

A

malignancy involving the RBC that causes polycythemia

76
Q

causes of secondary polycythemia

appropriate and inappropriate

A
  • appropriate: chronic hypoxemia (cyanotic congenital heart disease, pulmonary disease, residence at high altitudes)
  • inappropriate: benign of malignant tumors, excess hormone production, kidney abnormalities
77
Q

ruddy facial complexion with normal size liver and spleen

A

sign of polycythemia

78
Q

EPO level in secondary polycythemia

A

high

79
Q

when do you most commonly see relative polycythemia

A

dehydration

80
Q

complications of polycythemia (2)

A

thrombosis- vasoocclusive crisis, stroke, MI

bleeding

81
Q 
factor VIII and IX deficiency 
PTT
PT
bleeding time
plt count
petechiae 
hemarthroses
A 
prolonged
normal
normal
normal
no 
yes
82
Q 
von Willebrand's 
PTT
PT
bleeding time
plt count
petechiae 
hemarthroses
A 
prolonged
normal
prolonged
normal
no
rare
83
Q 
thrombocytopenia 
PTT
PT
bleeding time
plt count
petechiae 
hemarthroses
A 
normal
normal
prolonged
low
yes
no
84
Q 
platelet function defect 
PTT
PT
bleeding time
plt count
petechiae 
hemarthroses
A 
normal 
normal
prolonged
normal
yes
no
85
Q 
vitamin K deficiency
PTT
PT
bleeding time
plt count
petechiae 
hemarthroses
A 
prolonged 
prolonged
normal
normal
yes
yes
86
Q 
DIC and liver disease 
PTT
PT
bleeding time
plt count
petechiae 
hemarthroses
A 
prolonged
prolonged
prolonged
low
yes
sometimes
87
Q

_______ = defect in factor VIII procoagulant activity. Plt function is normal

A

Hemophilia A

88
Q

________ = factor VIII procoagulant activity is variable but pot function is defective b/c of a decrease of defect in von Willebrand’s factor (needed for pot adhesion to blood vessel walls)

A

von Willebrand’s disease

89
Q

inheritance of hemophilia

A

X linked

90
Q

hemarthroses and deep soft tissue bleeding are hallmarks of _____
risk of serious and life threatening hemorrhage is lifelong

A

hemophilia A and B

91
Q

3 categories of hemophilia severity

A

severe- spontaneous bleeding, < 1% activity
moderate- bleeding only w/ trauma (1-5%)
mild- bleeding only after surgery or major trauma (> 5%)

92
Q

how to manage hemophilia A

A

factor VIII

DDAVP can be useful in mild hemophilia

93
Q

inheritance of von Willebrand’s disease

A

AD

94
Q

3 types of von Willebrand’s disease

A 
type I (classic)- mild quantitative deficiencies of vWF and factor VIII protein... this is the most common form
type II- qualitative abnormality in vWF
type III- absence of vWF; most severe form
95
Q

common sxs of von Willebrand’s disease

A

epistaxis, menorrhagia, bruising, bleeding, mucocutaneous bleeding

96
Q

how to dx von Willebrand’s disease

A

quantitative assay for vWF antigen and activity (ristocetin cofactor assay)

97
Q

how to tx von Willebrand’s disease

A

DDAVP for mild to moderate bleeding and for ppx

cryoprecipitate for serious bleeding or type III disease

98
Q

vitamin K dependent factors

A

II, VII, IX, X, protein C and S

99
Q

causes of vitamin K deficiency

A

inadequate intake is unusual

  • mediations- cephalosporins, riframpin, isoniazid, warfarin
  • decreased absorption- pancreatic insufficiency, prolonged diarrhea, biliary obstruction
  • hemorrhagic dz of the newborn- within 24 hrs - 3 months
100
Q

serious bleeding in new born
-cutaneous bleeding, hematemesis, bleeding from circumcision site or umbilical cord

how do you prevent this?

A

hemorrhagic dz of newborn

give IM vitamin K after birth

101
Q

how to tx vitamin K deficiency bleeding

A

vitamin K

if severe disease, give FFP as needed

102
Q

prolonged PT and PTT, increased fibrin degradation products, thrombocytopenia
this could be 2 things
how do you tx it?

A

DIC- fibrinogen, FFP, plts as needed

severe liver dz- vitamin K, FFP, plts as needed

103
Q

what shape RBCs do you see on smear of DIC

A

fragmented and helmet shaped RBCs

104
Q

palpable purpura on LE and buttocks, renal insufficiency, arthritis, abdominal pain
-normal plt count

A

Henoch-Schonlein purpua (IgA mediated vasculitis)

105
Q

locally dilated and tortuous veins and capillaries of the skin and mucous membranes
-AD disorder

A

hereditary hemorrhagic telangiectasia

106
Q

impaired collagen synthesis –> weakened blood vessels

due to a vitamin deficiency

A

vitamin C

scurvy

107
Q

steroids can cause blood vessel weakness and bleeding (T/F)

A

T

108
Q

X linked disorder: thrombocytopenia with unusually small plts, eczema, defects in T and B cell immunity

A

Wiskott-Aldrich syndrome

109
Q

AR disorder: thrombocytopenia, limb abnormalities like absence of radius (however, compared to falcon anemia, the thumb is present here)

A

thrombocytopenia-absent radius syndrome (TAR)

110
Q

cutaneous bleeding or mucous membrane bleeding 1-4 weeks after a viral infection
-thrombocytopenia, few large “sticky” plts

A

immune thrombocytopenia purpura (ITP)

111
Q

how to tx ITP

A

supportive care

  • plts < 20,000 or active bleeding warrant IVIG or steroids
  • generally do not transfuse plts b/c it will be rapidly destroyed anyways
  • can try anti-D immunoglobulin as second line
112
Q

prognosis and tx of ITP

A

70-80% resolve spontaneously within months

-if it continues, splenectomy is often curative but due to infection risk, we hesitate to do that in young kids

113
Q

mother has ITP –> antibodies against her own plts cross the placenta –> antibodies destroy fetus’s plts
-mom has thrombocytopenia

A

passive autoimmune thrombocytopenia

114
Q

mother makes antibodies against fetus’s plts –> antibodies cross placenta and destroy fetus’s plts
-mom has normal pot count

A

isoimmune thrombocytopenia

115
Q

enlarging hemangioma, MAHA, thrombocytopenia, consumptive coagulopathy

A

Kasabach-Merritt syndrome

116
Q

AR disorder: diminished ability of plts to aggregate 2/2 deficient adhesive glycoprotein IIb/IIIa on the plt cell membrane

A

Glanzmann’s thrombasthenia

117
Q

AR disorder: decreased plt adhesion as a result of absence of plt membrane glycoproteins

A

bernard-soulier syndrome

118
Q

causes of acquired qualitative plt disorders

A

drugs- aspirin, depakote

uremia and severe liver disease

119
Q

purpura fulminans- nonthrombocytopenic purpura (fever, shock, rapidly spreading skin bleeding and intravascualr thrombosis )

A

presentation of homozygous protein C deficiency

120
Q

how do protein C heterozygotes present

A

later with DVT or CNS thrombosis

121
Q

how to tx protein C deficiency

A

heparin, FFP, warfarin, protein C

122
Q

define neutropenia levels (3)

A

mild- ANC 1000-1500
moderate- ANC 500-1000, infection of mucous membranes and skin
severe- ANC <500, severe infections esp with staph aureus and gram negative bacteria

123
Q

_______ are the most common cause of neutropenia during childhood

A

infections

  • HIV, EBV, EMV, Hep A and B, influenza, parvovirus
  • typhus, RMSF
  • protozoans
124
Q

increased incidence of mild infections
noncyclic neutropenia
otherwise healthy with normal appearance and growth

A

chronic benign neutropenia of childhood (CBN)

125
Q

what does the bone marrow look like in chronic benign neutropenia of childhood

A

immature neutrophil precursors

126
Q

prognosis of chronic benign neutropenia of childhood

A

resolves spontaneously in months to years

127
Q

AR disorder where ANC is usually < 300

A

severe congenital agranulocytosis (Kostmann syndrome)

128
Q

how to dx cyclic neutropenia

how long are the cycles

A

dx with serial neutrophils counts during a 2-3 months period

cycles are usually 21 days

129
Q

AR disorder: oculocutaneous albinism, large blue-gray granules in cytoplasm of neutrophils, *neutropenia, blond or brown hair with silver streaks

A

chediak-higashi syndrome

130
Q

AR disorder: short stature, immunodeficiency, fine hair, *neutropenia

A

cartilage-hair hypoplasia syndrome

131
Q

exocrine pancreatic insufficiency with malabsorption, short stature 2/2 metaphyseal chondrodysplasia, *neutropenia

A

schwachman-diamond syndrome

132
Q

isoimmune neutropenia

A

mother makes antibodies to baby’s neutrophils –> causes neutropenia in baby that resolves by 8 weeks of life

133
Q

autoimmune neutropenia can happen 2/2 infection, drugs, SLE, JRA, etc (T/F)

A

T

134
Q

in iron deficiency anemia, the free erythrocyte protoporphyrin is elevated/decreased

A

elevated

135
Q

pts with SS trait may have these sxs

A

inability to concentrate urine, hematuria

136
Q

beta thalassemia puts you at increased risk for hemochromatosis (T/F)

A

T

137
Q

goat’s milk only leads to ____ deficiency

A

folic acid

138
Q

spoon shaped nails seen in ____ deficiency

A

iron

139
Q

smooth red tongue seen in _____ deficiency

A

B12

Pediatrics (14 decks)

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