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RBC disorders Flashcards

1
Q

what are the two parts of Anemia

A

Reduction of red cell mass

Impaired tissue oxygenation

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2
Q

how can reduction of RBC mass be shown

A

Red cell count

Hemoglobin/hematocrit

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3
Q

what are the signs of impaired tissue oxygenation

A

Shortness of breath
Weakness
Fatigue
Pallor

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4
Q

what do the normal levels of rbc’s depend on

A

Age
sex
geographic location

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5
Q

what is polycythemia

A

increase in red cell mass

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6
Q

what are the categories of Anemia

A

RBC loss (blood loss)
Decreased RBC survivial
Decreased RBC production

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7
Q

what causes RBC (blood) loss

A

Hemorrhage (acute)
Trauma (acute)
GI or GYN disease (acute or chronic, occult blood loss)

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8
Q

what causes decread RBC survival

A 
HEMOLYSIS
Mechanical trauma
transfusion reaction
Herediatry spherocytosis
Hemoglobinopathies
Thalassemias
G6PD deficiency
Erythroblastosis fetalis
Malaria
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9
Q

what causes Decreased RBC production

A 
Iron deficiency anemia
Vitamin B12 deficinecy
folate Deficiency
Aplastic anemia
Myelophthisic anemias
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10
Q

what does a CBC test for

A 
RBC count
Hemoglobin
Hematocrit
Mean cell volume, Hb, and Hb concentration
WBC
Platelts
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11
Q

what tests are done for anemia

A

Blood smear review
CBC
REticulocyte count
Iron indicies

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12
Q

what does an iron indice test for

A

Serum Fe, Iron-binding capacity
transferrin saturation
ferritin concentration
Vitamin B12 and Folate (also)

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13
Q

what is included in a hemolysis work-up

A 
Dilirubin (direct and indirect)
Haptoglobin
LDH
coomb's test
Plasma hemoglobin
Hemoglobin electrophoresis
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14
Q

how much blood loss can a young healthy indivudal tolerate with minimal symptoms

A

Up to 1000ml of rapid blood loss

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15
Q

what loss of blood results in shock

A

2000ml of rapid loss

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16
Q

what helps to re-expand vascular volume once blood loss in controle

A

Interstitial fluid redistribution

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17
Q

what are the symptoms of 500-1000ml of blood loss

A

vasovagal response

  • sweating
  • weakness
  • nausea
  • slow heart rate
  • hypotension
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18
Q

what are the symptoms of 1000-1500ml of blood loss

A

Lightheadedness

orthostatic hypotension

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19
Q

what are the symotoms of 1500-2000ml of blood losss

A 
thirst
shortness of breath
loss of consciousness
sweating
rapid pulse
decreaed blood pressure clammy skin
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20
Q

what stimulates RBC production

A

erythropoietin

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21
Q

what is found in the blood at higher rates when lots of erythropoietin is made

A

lots of reticulocyte

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22
Q

when does chronic blood loss cause anemia

A

rate of loss exceeds capacity for RBC regeneration,

when iron reserves are depeleted

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23
Q

what are the important cuasese of iron deficiency

A 
chronic GI hemorrhage due to ulcer or neoplasm
GYN hemorrhage (menorrhagia)
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24
Q

what causes intravascular decrease in RBC survival

A

Destruction of RBC within circulation

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25
Q

what causes extravascular decrease in RBC survivial

A

Destruction of RBC within reticuloendothelial system

Tissue macrophages of the spleen and liver

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26
Q

what happens to hemoglobin released from RBC into circulation in intravascular hemolysis

A

bound to haptoglobin and cleared from ciculation by the liver

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27
Q

what is haptoglobin

A

binding protein for hemoglobin

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28
Q

what is a key feature of intravascular hemolysis

A

Decrease in serum haptoglobin

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29
Q

what happnes when plasma hemoglobin exceeeds haptoglobin

A

free hemoglobin is peded out (hemoglobinuria)

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30
Q

what is hemoglobin’s effect on the kidney

A

toxic due to iron accumulation in proximal tubular cells when the cells are shed

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31
Q

what is the shedding of cells lost when hemoglobin is peed out

A

Hemosiderinuria

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32
Q

what does conversion of heme to bilirubin lead to

A

Hyperbilirubinemia and jaundice

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33
Q

what determines the degree of jaundice

A

dependent on functional capacity of liver

rate of hemolysis

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34
Q

what are the immune causes of intravasvular hemolysis

A

Transfusion reaction

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35
Q

what are the non-immune causes of intravascular hemolysis

A

Mechanical trauma (defective heart valve

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36
Q

what all is affected in intravascular hemolysis

A 
decreased:
haptoglobin
Hemoglobinemia
Hemoglobinuria
Hemosiderinuria
Hyperbilirubinemia
Reticulocytosis
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37
Q

what hapens to damaged or abnormal RBC’s

A

Removed in speen

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38
Q

what happens to hemoglobin in the spleen

A

Broken down intracellularily (not released directly into blood and urin)

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39
Q

what does breakdown of hemoglobin lead to an increase in the blood

A

hemoglobin breakdown products increase in the blood

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40
Q

what does the presence of hemoglobin breakdown products in the blood lesad to

A

hyperbilirubinemia

Jaundice

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41
Q

what organs are affected by extravascular hemolysis

A

Spleen and liver enlarrged

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42
Q

what does elevated levels of bilirubin lead to

A

Formation of gallstones

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43
Q

what are the intrisic defects that cause extravascular hemolysis

A

Non-immune

  • RBC membrane defects
  • Hemoglobinopathies
  • metabolic fects
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44
Q

what kind of defect is hereditary spherocytosis

A

Membrane defect for extravascular hemolysis

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45
Q

what causes hereditary spherocytosis

A

less defomrability of the RBC so that they are sequested and destroyed in the spleen

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46
Q

what protein is affected in herditary spherocytosis

- is it quantitative or qualitative

A

quantitative or qualitative spectrin (structural protein)

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47
Q

how is hereditary spherocytosis transmitted

A

Autosomal dominant

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48
Q

when does hereditary Spherocytosis manifest it self

A

adult life

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49
Q

how does removal of spleen affect herediatry spherocytosis

A

normal RBC survival

does not give normal RBC morphology

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50
Q

what kind of problem is sickle cell anemai

A

Abronal hemoglobin extravascular hemolysis

(hemoglobinopathies)

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51
Q

how is sickle cell anemia transported

A

Autosomal codominant

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52
Q

what causes sickle cell anemia

A

the strucutre of globin chain causes hemoglobin to gel upon deoxygenation

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53
Q

what is the mutation that causes sickle cell anemia

A

one single base pair substitaion at position 6 (valine for gluatmic acid)

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54
Q

what happens in sickle cell anemia in low O2 conditions

A

abnormal hemoglobin polymerizes causing RBC to create a sickle shape

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55
Q

problem with sickle cell shape

A

Rigid and vulberable to splenic sequestation (decreased survival)
block microciuluation
- ischemia and or infarcause

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56
Q

how many alleles do you ned for sickle cell disease

A

homozygous

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57
Q

characteristics of homozygous sickle cell disease

A

severe anemia
Vaso-occlusive crises
- acute chest syndrome
- stroke

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58
Q

what are the complication of homozygous sickle cell disease

A

autospenectormy
painful crises
leg ulcers
retinal and renal thrombosis

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59
Q

what are the odds of having at least one sickle cell genee

A

8% of blacks

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60
Q

blood effect of sickle cell anemai

A

Moderate to severe anemia
Hyperbilirubinemia
Reticulocytosis

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61
Q

what is the type of anemia that is thalassemia

A

extravascular hemolysis due to lack of globin chains

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62
Q

how is thalassemia transmitted

A

Autosomal codominant

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63
Q

what causes thalassemia

A

Diminished or absent synth of alpha or beta globin chains of hemoglobin

64
Q

what happens to the gene in thalassemia

A

whole or partial gene deletion
mutation of coding sequence/promoter
mRNA instability

65
Q

what does decreased globin from thalassemia lead to

A

Decreased hemoglobin prudction with anemia

66
Q

what does the production of excess of other globulin chains in Thalassemia lad to

A

membrane damage and premature destruction of RBC precursors in the marrow and sleen

67
Q

what are the clinical manifestations of Thalassemia

A 
Varibale anema (asymptomatic to severe)
moderate to marked microcytosis with target cells and basophilic stipping of the REd cells present in a blood smear
68
Q

where is Thalassemia common

A

mediterranean
african
southeast asia

69
Q

what kind of defect is Glucos-6-phosphate dehydrogenase deficiency

A

Metabolic Defect

70
Q

how is G6PD transmitted

A

X linked inheritance

71
Q

what ahppens to red cells susceptible to oxidance injury

A

usually precipiated by drugs/toxins or infections

72
Q

what happens to oxidized hemoglobin from G6PD

A

precipitates within the cell and attaches to the RBC membrane
- reduced membrane flexibility

73
Q

what does reduced membrane flexibility lead to in glucose-6-phosphate dehydrogenase Deficiency

A

Extravascular hemolysis in the sleen

74
Q

what is needed to make Glucose-6-phosphate Dehydrogenase symptomatic

A

Oxidant is present

75
Q

what is the cytomorphologic halmmark of glucose-6-phasophate

A

Bite cells

76
Q

what extrinisic defect is the hemolytic disease type of the newborn

A

Autoimmune

77
Q

what type of hemolysis is Erythroblastosis fetalis

A

Immune destruction extravascular hemolysis

78
Q

what causese erythroblastosis featlis

A

blood group incompatibility between mom and fetus

79
Q

when does erythroblosis featilis occur

A

in utero when fetus has antigens from dad that the mom does not have

80
Q

what cause sensitization in the mother in erythroblosis featalis

A

Fetal RBC during 3rd trimester during pregnancy or childbirth entering materal circulation leads t

81
Q

when do the full effects of Erythroblosis featalis affect featus

A

second pregnancy antibodies go into the featus leading to extravascular hemolysis

82
Q

what antigens are the most important for erythroblosis featalis

A

ABO and Rh antigens

83
Q

what is done in Rh mothers to prevent Erythroblastosis fetalis

A

given anti-D within 72 hours of delivery of Rh-positive featus

84
Q

what is the action of Anti-D in preventing Erythroblastosis

A

binds to Rh-positive fetal cells and removes them from circulation before antibodies can form and mom becomes sensitized

85
Q

when does ABO hymolytic disease occur

A

In A or B infants with O moms

86
Q

why is lysis of featl RBC miniminal during ABO hymolytic disase

A

Fetal cells Present some A weakly

other cells express A and B antigens and soak up the antibody

87
Q

what kind of RBC disorder is Hemolytic Tranfusion reaction

A

Intravascular hemolysis autoimmune

88
Q

what happens in hemolytic transfusion reaction

A

incompatable RBC into a sensitized patient causes antibody binding to antigen

  • activates complement
  • immediate intravascular hemolysis
89
Q

what are the problems with hemolytic transfusion reaction

A

Bleeding
renal failure
Shock
Death

90
Q

what antigens are important for hemolytic transfuction reactions

A

ABO (others can cause problems through)

91
Q

what kind of problem is Autoimmune hemolysis

A

extravascular hemolysis

92
Q

what causes autoimmune hemolysis

A

Patient makes antibodies to own RBC

93
Q

what does autoimmune hemolysis lead to

A
  • anitbody coated cells are lysed(complemt) or removed by retriculoendo system
94
Q

phargocytic removal of RBC from autoimmune hemolysis leads to

A

partial loss of red cell membrane (Spherocytes)

- sequester in spleen (anemia)

95
Q

what kind of disorder can be cause by caridac valve prosthesis

A

Intravscular hemolsys

96
Q

what happens in cardiac valve prosthesis hemolysis

A

RBC disrupted by phsycial trauma as pass through turbulance and abnormal pressure

97
Q

how can DIC lead to RBC disorders

A

RBC’s lysed as they pass through fibrin clots and strands and produce schistrocytes

98
Q

what kind of Disorder does Malaria cuase

A

Intravascular hemolysis

99
Q

what does Malaria fur

A

Infect RBC to cause lysis of RBC during maturation

100
Q

what is the hallmark of autoimmune hemolytic anemai

A

Spherocytes

101
Q

what test is done for Autoimmune hemolysis

A

Positive direct Coomb’s test

102
Q

what does the presence of schistocytes show

A

Indicuate hemolysis due to mech trauma

103
Q

what can causes a nutrition decrease in RBC production

A

IRon deficincy

B12 an dFolate deficiency (megaloblastic anemia)

104
Q

what is the most common basis of anemia world wide

A

Iron deficicy

105
Q

how does IRon deficiency anemia usually develop

A

Insidiously

106
Q

how tends to suffer from inadequate intake of iron

A

Infants

107
Q

who has icnreased demand for iron

A

Pregnancy

Adelescencts

108
Q

what can cause an increased loss of iron

A

Bleeding

109
Q

does an iron deficincy often stand a lone

A

No, may be related to a more serious condition

110
Q

what happens to the RBC in lab during iron deficincy

A

Microcytic (low MCV)- smaller)

Hypochromic (low MCHC) - less hemoglobin

111
Q

what is lost in the serium during a iron deficincy

A

Less serum ferritin
more serum iron binding capcaicty
less serum iron

112
Q

how does the reticulocyte respond in rion defiincy anemai

A

Absent reticulocyte response

113
Q

what is rion used for

A

Hemoglobin
Myoglobin
enzymes

114
Q

what is usually done to test anemia

A

Lab work

115
Q

what causes MEgaloblasti anemia

A

Folate and B12 deficiency

116
Q

what synthesis does Megaloblastic Anemia involve

A

Synthesis of Thymidine

117
Q

how does Impaired DNA synth affect mitotic division

A

delays it

118
Q

what does megaloblastic anemia do to the nucleus and cytoplasm

A

Nuc Does not matuer appropriately

Cytoplasm does normal

119
Q

how does Megalblastic Anemia change cells

A

Large red and white cells precursors (increased MCV)

120
Q

what does Megalblastic Anemia do to the neutrophils

A

Hypersegmented

121
Q

how does MEgaloblastic Anemia affect mature RBC production

A

Decreased due to absent reticulocyte reponse

122
Q

what may severe megaloblastic anemia do

A

White cell and platelet production affected

123
Q

how does Megaloblastic anemia cause annemia

A

megaloblast Accumulate in the bone marrow, not enought RBC released

124
Q

what happens to the megaloblasts in the marrow

A

Autohemolysis or destroyed by phagocytic cells in the marrow

125
Q

what other cells can be affected by Megaloblastic anemia

A

All regularily deviding cells

126
Q

where is Folate found

A

Fesh veg

127
Q

how much folate does the body store

A

Small amounts

128
Q

what causes an increased need in Folate

A

Pregnancy
Hemolytic anemia
hell cell turnover state

129
Q

what may cause a folate deficiency

A

Increased need

Decreased dietary intake or Malabsorption

130
Q

where is B12 found

A

Animal foods

131
Q

how much B12 does your body store

A

Lots

132
Q

what causes a B12 deficiency

A

Increased need

Decreased intake/malabsorption

133
Q

what all can B12 deficiency affect besides Hematologic abrnormalities

A

Neurologic symptoms

134
Q

what is needed to absorb Vit B12

A

Intrisic factor

135
Q

where is intrisic factor found

A

Produced by parietal cells of the gastric mucosa

136
Q

what happens when Vit B12 binds to Intrinsic factor

A

passes to distal ileum to attach to receptors to absorb

137
Q

what does Absorbed B12 do

A

Binds to TRanscobalamins in plasma to deliver to liver and other cells via blood

138
Q

where is folate abosrbed

A

In proximal small intestine

139
Q

what causes Pernicious Anemia

A

Autoanitbodies to parietal cells and intrinsic factor

140
Q

what is a B12 deficiency associated with

A

Atrophic gastritis(loss of gastric parietal cells)
achloyhydria
Deficiency IF

141
Q

types of Decreased RBC production

A

Aplasitic anemia

Myelophthisic processes

142
Q

what dose a decrease in cellular elements of blood cause

A

Aplastic Anemia

143
Q

do we know the cause of more aplastic anemia s

A

no, 1/2 are idiopathic

144
Q

what causes aplastic anemia

A

Drugs (Chloramphenicol)
Toxins (benzene)
Viral hep

145
Q

what casues Aplastic Anemia

A
  • An acquired stem cell defect with decreased production of types of Blood cells
  • suppresssion of stem cells via T lymphocytes
146
Q

what surpresses stem cells in Aplastic Anemia

A

T lymphocytes

147
Q

how to treat Aplastic Anemai

A

Bone marrow transplant (less than 40 year old patients)

148
Q

what causes Myelopohthisic anemia

A

decreased production of red cells due to replacement of marrow elements

149
Q

what happens in Myelophthisic anemia

A

Normal hematopoietic cells in marrow are crowded out by tumor or fibosis

150
Q

what happens to cell shapre in myelophthisic Anemia

A

misshapen RBCs resembling teardrops on blood smear

151
Q

what else besides RBC are affected in myelophthisic anemia

A

Platelets are decreased

152
Q

what in an increase in Red Cell mass

A

Polycythemia

153
Q

what causes Relative polycythemia

A

Dehydration

Diarrhea

154
Q

what causes primary Absolute polycythemia

A

Neoplastic proliferation of Red ells

155
Q

what causes secondary absolute polycythemia

A

Icnreased Erythopoieticin fruction

156
Q

what causes an increase in erythorpoietin

A

Cyanotic heart disease
Pulmonary disease
High altitude
Erythropoietin producting tumors

157
Q

neoplastic growth of RBC and Myeloid cells

A

Polycythemia veria

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