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Hematology > NCC Content > Flashcards

NCC Content Flashcards

1
Q

Pluripotent stem cells

A

all blood cells are made from these

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2
Q

bone hematopoiesis

A

predominates from 22 weeks gestation forward

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3
Q

liver hematopoiesis

A
  • established by 9 weeks gestation
  • peaks at 4-5 months gestation
  • regresses as bone marrow production increases
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4
Q

when and where does hematopoiesis start?

A

starts in the yolk sac during the 3rd week of gestation

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5
Q

what is hematopoiesis?

A

the formation, production, and maintenance of blood cells

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6
Q

Name the extramedullary sites of hematopoiesis

A
  • spleen
  • lymph nodes
  • thymus
  • kidneys
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7
Q

What factors influence hematopoiesis?

the rate of differentiation of pluripotent cells

A

hypoxia, bacterial infection, physiologic stress

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8
Q

What is erythropoiesis?

A

the production of erythrocytes

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9
Q

Where is erythropoietin produced?

A

prenatally: in the liver
postnatally: in the kidneys

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10
Q

What factors increase erythropoiesis?

A
  • anemia
  • low oxygen availability to tissues
  • down syndrome
  • IUGR
  • infants born to women with diabetes or PIH (placental insufficiency)
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11
Q

What is erythropoietin?

A

the hormone that regulates erythropoiesis & hemoglobin synthesis

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12
Q

Describe hemoglobin

A
  • major iron-containing component of RBC

- carries oxygen from the lungs to the tissue cells

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13
Q

What is a normal hemoglobin level?

A

normal 14-20 g/dL in infants > 34 weeks, slightly lower in preterm infants

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14
Q

Describe the lifespan of fetal hemoglobin (HbF)

A
  • begins around 14 days of life
  • starts to decline at 30-32 weeks
  • drops dramatically by 6 months of life
  • virtually gone at 1 year of life
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15
Q

At term, how much HbF do babies have?

A

RBCs contain 70-90% at birth

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16
Q

What is special about HbF?

A
  • it has a higher affinity for oxygen than adult hemoglobin

- this is why babies can tolerate lower levels of oxygen in utero

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17
Q

When does adult hemoglobin start production (HbA)?

A

-begins at the end of fetal life

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18
Q

What factors affect a baby’s Hgb and Hct level?

A
  • gestation
  • placental transfusion
  • blood sampling site (if capillary Hgb is high, recheck w/ central lab draw)
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19
Q

What is hematocrit (Hct)?

A

-% of RBCs in a unit volume of blood

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20
Q

What are reticulocytes?

A

immature RBCs

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21
Q

Describe the life span of a reticulocyte.

A
  • 1 to 2 days in the marrow and 1 more day in the circulation before full maturation
  • this cycle takes longer when stress is present
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22
Q

Reticulocyte count

A
  • the lower the gestation, the higher the count
  • 3-7% during first 24-48 hrs of life, can be as high as 10% in preterm infant
  • falls to
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23
Q

How do reticulocytes function?

A
  • oxygen and carbon dioxide transport

- buffer

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24
Q

What does an increased retic count suggest?

A

-chronic blood loss or hemolysis

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25
Q

Life span length of a RBC

A
  • term infant 60-70 days

- preterm infant 35-50 days

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26
Q

Why do premature infants often need RBC transfusions?

A
  • shortened life span of RBC in preemies

- lab draws

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27
Q

What is a nucleated red blood cell?

A

circulating immature (prereticulocyte) red cells

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28
Q

What do nucleated RBCs indicate or suggest?

A
  • indicative of hemolysis, acute blood loss, hypoxemia, congenital heart disease, infection
  • indicative of stress
  • their presence suggest problems regarding long-term neurodevelopmental outcomes
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29
Q

If you know your Hct, Hgb or RBC count, you can figure out the other two…

A

Example:
Hgb 15%
multiply by 3 = Hct
divide by 3 = RBC count

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30
Q

About WBCs…

A
  • also called leukocytes
  • mature in bone marrow and lymphatic tissues
  • react to foreign protein in extravascular space
  • premature babies have 30-50% less than term
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31
Q

What are the three types of WBCs?

A
  • granulocytes (3 types)
  • lymphocytes
  • monocytes
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32
Q

What are the three types of granulocytes?

A
  • basophils
  • eosinophils
  • neutrophils
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33
Q

What do basophils do?

A

-allergic and inflammatory responses

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34
Q

What do eosinophils do?

A
  • allergic and anaphylactic responses, parasitic destruction
  • similar to neutrophils, but less effective in response
  • prolonged survival in extravascular space
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35
Q

What do neutrophils do?

A
  • phagocytes
  • physiological stress can increase production and bone marrow release of immature forms (bands, myelocytes, metamyelocytes, promyelocytes)
  • may be increased at birth but decrease during first week of life
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36
Q

Explain T-lymphocytes

A
  • thymus derived

- graft vs. host disease, delayed hypersensitivity reactions

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37
Q

Explain B-lymphocytes

A
  • bone marrow derived

- production and secretion of immunoglobulins and antibodies

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38
Q

What do monocytes do?

A
  • immature circulating macrophages
  • mature once in tissues
  • “The cleaners”
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39
Q

Describe platelets.

A
  • small, non nucleated disk-shaped cells

- functions: hemostasis, coagulation, thrombus formation

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40
Q

What stimulates platelets to work?

A

response stimulated by disruption of the endothelium

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41
Q

Where are platelets produced?

A
  • derived from megakaryocytic in the bone marrow

- circulate in the blood for 7-10 days before being removed by the spleen

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42
Q

What population of newborns have lower platelet counts?

A

-SGA infants have 20-25% lower platelet counts, probably from stress

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43
Q

How much blood volume does a term baby have?

A

around 80-100 ml/kg

44
Q

How much blood volume does a preterm baby have?

A

around 90-105 ml/kg

45
Q

What is the definition of anemia?

A

-central venous hgb

46
Q

What can cause anemia?

A
  • hemorrhage
  • hemolysis
  • prematurity
  • iatrogenic
47
Q

What clotting factors require Vitamin K?

A

II, VII, IX, X

48
Q

What are the types of hemostasis reactions?

A
  • vascular (vessel contraction)
  • intravascular (platelet plug formation)
  • extravascular (compression by surround tissue, release of thromboplastin)
49
Q

What is the prothrombin time? (PT)

A

extrinsic (faster) and common portions of coagulation cascade, requires factor VII which requires vitamin K
-time it takes for plasma to clot after the addition of tissue factor

50
Q

What is the partial thromboplastin time? (PTT)

A

intrinsic (slower) and common portions of the coagulation cascade
-used to monitor treatment effects of heparin

51
Q

What is d-dimer?

A

fibrin degradation product

52
Q

What is hemorrhagic disease?

A

hemorrhagic tendency caused by vitamin K deficiency and decreased activity of factors II, VII, IX, X

53
Q

What is vitamin K affected by?

A

-lack of bacterial presence (intestinal flora is needed for vitamin K synthesis)

54
Q

Describe EARLY hemorrhagic disease

A
  • least common
  • bleeding with 1st 24 hours of life
  • typically associated with maternal anticonvulsant therapy
  • cannot be prevented with administration of vitamin K
  • therapy=treatment of mother with large doses of vitamin K > or = 10 days prior to delivery
55
Q

Describe CLASSIC hemorrhagic disease

A
  • onset 2-5 days of life
  • generalized and occasional dramatic bleeding
  • typically breast fed infant who has not received prophylactic vitamin K and is not taking adequate amounts of EBM
56
Q

Describe LATE hemorrhagic disease

A
  • onset after 7 days
  • more devastating d/t increased incidence of intracranial hemorrhage, permanent sequelae, mortality rate
  • associated with chronic diseases that interfere with fat absorption or performance of intestinal flora
57
Q

what supplement is important for babies with liver dysfunction?

A

ADEKs

58
Q

What has “virtually” eliminated hemorrhagic disease?

A

prophylactic vitamin K!!

-0.5 to 1 mg IM, or IV in ELBW

59
Q

what is in cryoprecipate?

A

factor VIII and fibrinogen

60
Q

What is DIC?

A

an acquired hemorrhagic disorder associated with an underlying disease manifested as uncontrolled activation of coagulation & fibrinolysis

61
Q

What are the warning signs for DIC?

A

Hi-YA

  • hemorrhage
  • ischemia
  • anemia
62
Q

Pathophysiology for DIC

A
  • depleted clotting factors and platelets
  • blood loss & red cell fragmentation
  • microvascular thrombi lead to ischemia and necrosis of any organ (like the kidneys)
63
Q

How do you treat DIC?

A

provide supportive therapy while attempting to treat the underlying disease
this is the only treatment!

64
Q

What is thrombocytopenia?

A

a platelet count of

65
Q

What is autoimmune thrombocytopenia? (80%)

A

THIS IS A MATERNAL AUTOANTIBODY CONDITION

-mom has idiopathic thrombocytopenia purpura, systemic lupus erythematosus

66
Q

What is the pathophysiology of autoimmune thrombocytopenia?

A

maternal IgG antibodies cross the placenta and destroy fetal platelets

  • nadir occurs on DOL 2
  • counts are low as long as antibodies are present, typically as long as 4 months
67
Q

is maternal platelet count low, normal, or high with autoimmune thrombocytopenia?

A

LOW

68
Q

What is alloimmune thrombocytopenia? (20%)

A
  • similar to Rh incompatibility
  • fetal platelets contain antigen that is lacking in mother (inherited from father)
  • fetal platelets enter maternal circulation and antibodies are created
  • Antibodies cross into fetal circulation and destroy fetal platelets
69
Q

Characteristics of alloimmune thrombocytopenia

A
  • nadir occurs in first few days of life; normalizes by 1 month
  • fetal thrombocytopenia can occur as early as 20 weeks’ gestation
  • more severe sequelae than autoimmune
70
Q

Is maternal platelet count low, normal or high in alloimmune thrombocytopenia?

A

normal

71
Q

Treatment for alloimmune thrombocytopenia?

A

give maternal platelets or matched platelets

72
Q

Why does birth depression (apgar

A

fetal megakaryocytic have increased sensitivity to hypoxic injury

73
Q

What are rare causes of impaired production of platelets?

A
  • trisomy 13, 18: bone marrow hypoplasia
  • TAR syndrome (thrombocytopenia w/absent radii): megakaryocytic progenitor cell is defective, presents at birth but improvement follows
  • fanconi anemia: rarely presents in neonatal period; thumb, skeletal, renal, CNS anomalies w/ cafe-au-lait spots
  • rare syndromes: unusually small or large platelets
74
Q

Platelet problems from maternal drugs

A

interferes with platelet aggregation

  • demerol
  • phenergan
  • ASA
  • sulfonamides
  • quinidine
  • quinine
  • thiazides
75
Q

Why are neonates at a greater risk for thrombosis?

A

d/t diminished fibrinolysis r/t decreased plasminogen levels

76
Q

About renal vein thrombosis

A
  • most common, often in LGA babies

- often from indwelling umbilical venous catheter

77
Q

symptoms of renal vein thrombosis

A
  • flank mass/enlarged kidney
  • hematuria
  • hypertension
  • renal failure
  • proteinuria
  • thrombocytopenia (platelets are used up on clots)
  • depletion of coagulation factors
78
Q

about renal artery thrombosis

A
  • often from indwelling umbilical arterial catheter

- often less common and less severe than renal vein thrombosis

79
Q

symptoms of renal artery thrombosis

A
  • flank mass/enlarged kidney
  • hematuria
  • renal failure
  • may have hypertension
80
Q

What is purpura fulminans and what is it associated with?

A
  • life threatening problem with an acute onset characterized by cutaneous hemorrhage and necrosis of tissue, low blood pressure, fever and DIC
  • associated with congenital and acquired thrombophilias
81
Q

Describe congenital thrombophilias

A
  • deficiencies of protein C, protein S, antithrombin, activated protein C resistance, MTHFR deficiency
  • look for family history, early age of onset, recurrent disease, unusual/multiple locations of thrombosis
82
Q

Describe acquired thrombophilias

A

-coagulation factor deficiency r/t placental transfer of maternal anti-phospholipid antibodies (i.e. maternal lupus)

83
Q

What is the other name for Christmas disease?

A

hemophilia B

84
Q

How is hemophilia passed on?

A

X-linked recessive inheritance
(gene is on X chromosome)
-females are carriers only due to XX
-males are affected due to XY - no normal X to counterbalance bad X

85
Q

What is von Willebrand disease?

A
  • component of factor VIII functioning as ligand between the platelet and vessel
  • autosomal dominant or recessive inheritance
  • males and females affected equally
  • each pregnancy with 50% chance of occurrence
86
Q

How does von Willebrand disease present in the neonatal period?

A

-it rarely does; may present with mucus membrane bleeding

87
Q

How is von Willebrand disease diagnosed?

A

with ristocetin factor

88
Q

What is factor XIII deficiency?

A
  • autosomal recessive inheritance; males and females equally affected
  • symptoms are prolonged bleeding from umbilical stump or several days after circumcision
  • treat with cryo, factor XIII concentrate
  • most likely to present in the neonatal period??
89
Q

which cell is the most sensitive indicator of sepsis?

A

the neutrophil

90
Q

what are the mature neutrophils called?

A

polymorphonuclear (PMNs) or segs?

91
Q

what are the immature neutrophils called?

A

promyelocytes, myelocytes, metamyelocytes, bands

92
Q

define neutropenia

A

ANC

93
Q

is neutrophilia predictive of infection?

A

-somewhat, not as reliable as neutropenia

94
Q

What is the most common transfusion reaction in a neonate?

A

fever

95
Q

Why is graft vs host disease rare now with blood transfusions?

A

d/t irradiation of blood products

96
Q

What is the HCT in whole blood vs. PRBCs respectively?

A

35%; 60-90%

97
Q

Describe a partial exchange transfusion

A
  • done with normal saline to combat polycythemia
  • goal is to decrease HCT without decreasing blood volume
  • calculation: blood volume x (measured HCT - desired HCT) divided by measured HCT
98
Q

Describe PRBC administration for hydrops

A
  • goal is to correct anemia without increasing blood volume

- calculation: blood volume x (desired HCT - measured HCT) divided by PRBC HCT - measured HCT

99
Q

Describe a complete exchange

A
  • removes 70-85% of infants blood

- may cause hypoglycemia, hypocalcemia, hypomagnesemia

100
Q

How much should platelet count rise with a transfusion?

A

around 75K-100K

-lack of rise is indicative of destruction

101
Q

albumin for volume expansion

A

5% in increments of 10 ml/kg

102
Q

albumin for oncotic pressure

A

25% in increments of 4 ml/kg

103
Q

erythropoietin for anemia of prematurity

A
  • 500-1400 units/kg/week

- need to be on iron supplement to support increased erythropoiesis!

104
Q

ferrous sulfate administration

A
  • 2-5 mg/kg/day elemental iron daily

- iron fortified formula can provide up to 2 mg/kg/day

105
Q

How long should preterm infants receive iron supplements?

A

until 12 months of age

106
Q

What is the antidote for heparin overdose?

A

protamine sulfate

Hematology (4 decks)

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