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Red Blood Cells and Bleeding Disorders

Red Blood Cells and Bleeding Disorders

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Published by Pinay Yaun

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Published by: Pinay Yaun on Aug 28, 2011
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 1 
Bloodlines
 
Above:
From pluripotent cells arise the WBC and RBC and lymphoid series. Note that some cells will arise fromthe same mother cell.
Anemias
y
 
Reduction below normal limits of the totalcirculating red cell mass
y
 
Reduced oxygen transport capacity of theblood
y
 
Reduction below normal in the volume of packed red cell as measured by hematocritor hemoglobin concentration
y
 
IO
W, the patient will appear pale and weakfrom lack of oxygen.
C
lassification of Anemia According to UnderlyingMechanism
y
 
Blood loss
y
 
I
ncreased rate of destruction (hemolyticanemias)
y
 
I
mpaired red cell production
Anemia of Blood Loss
A
cute blood loss (microcytic, hypochromatic RBCsmay not be evident)
y
 
Reflect loss of blood volume
may lead toshock, death
y
 
H
emodilution
shift of water frominterstitial fluid compartment intointravascular space
y
 
E
rythropoietin production
reticulocytosis(
I
mmature RBC containing remnants of nuclei seen only in special stain. Biggerthan usual RBC. Polychromatophilic
bluish  red hue) reaching 10  15%
y
 
Reticulocyte count normally 0.5  1.5%Chronic blood loss (microcytic, hypochromic RBCsare more evident in chronic blood loss)
y
 
G
IT
bleeding: gastric ulcer, hematemesis,hemorrhoids
o
 
S
triking reticulocytosis may not be seen.
y
 
G
ynecologic causes
Subject: PathologyTopic: RBCs and Bleeding DisordersLecturer: Dr. CagampanDate of Lecture: August 9, 2011Transcriptionist: Mopster and Pinay
 Editor: Mopster and Pinay
 Pages: 15
   S   Y   2   0   1   1  -   2   0   1   2
 
 2 
I
ncreased Rate of Destruction (Hemolytic Anemia)
y
 
I
ntrinsic (intracorpuscular) abnormalities of red cells
 
o
 
H
ereditary
 
 
Red cell membrane disorders
 
 
D
isorders of membranecytoskeleton: spherocytosis,elliptocytosis
 
 
D
isorders of lipid synthesis:selective increase in membranelecithin
 
 
Red cell enzme deficiencies
 
 
G
lycolytic enzymes: pyruvatekinase deficiency, hexokinasedeficiency
 
 
E
nzymes of hexosemonophosphate shunt:
G6
P
D
,glutathione synthetase
 
 
D
isorders of hemoglobin synthesis
 
 
D
eficient globin synthesis:thalassemia syndromes
 
 
S
tructurally abnormal globinsynthesis(hemoglobinopathies): sicklecell anemia, unstablehemoglobin
 
o
 
A
cquired
 
 
M
embrane defect: paroxysmalnocturnal hemoglobinuria
 
y
 
E
xtrinsic (extracorpuscular) abnormalities
 
o
 
A
ntibody mediated
 
 
I
sohemagglutinins: transfusionreactions, erythroblastosis fetalis
 
 
A
utoantibodies: idiopathic(primary), drug associated,
SLE
,malignant neoplasm, mycoplasmalinfections
 
o
 
M
echanical trauma to red cells
 
 
M
icroangiopathic hemolyticanemia: thromboticthrombocytopenic purpura,
D
I
C
 
 
Cardiac traumatic hemolytic anemia
 
 
I
nfections: malaria
 
 
Chemical injury: lead poisoning
 
 
S
equestration in mononuclearphagocyte system: hypersplenism
 
I
mpaired Red
C
ell Production
y
 
D
isturbance of proliferation anddifferentiation of stem cells: aplasticanemia, pure red cell aplasia, anemia of renal failure, anemia of endocrine disorders
y
 
D
isturbance of proliferation and maturationof erythroblasts:
o
 
D
efective
D
N
A
synthesis: deficiency orimpaired use of vitamin B12 and folicacid (megaloblastic anemia)
o
 
D
efective hemoglobin synthesis
 
D
eficient heme synthesis: irondeficiency
 
D
eficient globin synthesis:thalassemias
 
U
nknown or multiple mechanisms:sideroblastic anemia, anemia of chronic infections, myelophthisicanemia due to marrow infiltration
Hemolytic Anemia
y
 
Premature destruction of red cells and ashortened red cell life span below thenormal 120 days
y
 
E
levated erythropoietin levels and acompensatory increase in erythropoiesis
y
 
M
arkedly increased erythropoiesis withassociated reticulocytosis
y
 
A
ccumulation of hemoglobin degradationproducts released by red cell breakdownderived from hemoglobin (e.g., bilirubin)
y
 
Pigment stone formation as a result of hemoglobin degradation.
y
 
T
end to produce extravascular hemolysisalthough, on occasion, intravascularhemolysis may occur.
 
y
 
T
end to be autosomal dominant
y
 
Rare in the Philippines, except
T
halassemia.
y
 
I
ntravascular hemolysis (causes):
o
 
M
echanical injury: e.g., prostheticcardiac valves, thrombi
o
 
Complement fixation to red cells: e.g.,mismatched transfusion
o
 
T
oxic injury: e.g., malaria
y
 
M
anifestations of intravascular hemolysis:
o
 
A
nemia
o
 
H
emoglobinemia
o
 
H
emoglobinuria
o
 
J
aundice: a small percentage of gallstones are of hemoglobin origin
o
 
H
emosiderinuria
y
 
E
xtravascular hemolysis
o
 
O
ccurs in mononuclear phagocytes of spleen
o
 
Predisposing factors:
 
Red blood cell membrane injury
 
Reduced deformability
 
opsonization
o
 
S
equestration of deformed orforeign red blood cells followed byopsonization
phagocytosis as red cellsnavigate sinusoids
o
 
T
hese sequestered RBCs are renderedpalatable to macrophages due tohypoxia and
A
T
P depletion.
o
 
Clinical features
 
A
nemia
 
S
plenomegaly
 
J
aundice
y
 
M
orphology of hemolytic anemias
o
 
Normoblastic hyperplasia in marrow
o
 
Reticulocytosis in peripheral blood
o
 
Pigment gallstones
o
 
H
emosiderosis
o
 
J
aundice, anemia
 
 3 
Below:
D
efective RBCs sequestered outside arethen phagocytized.
Hereditary Spherocytosis
y
 
I
ntrinsic defect in RBC membrane
ankyrindeficiency and other (usually spectrin)skeletal membrane components
 
y
 
RBC
spheroidal, less deformable,vulnerable to splenic sequestration anddestruction
 
y
 
A
nkyrin deficiency
associated with reducedstability and loss of membrane fragments ascells traverse circulation
 
y
 
I
nherited disorder, in Northern
E
urope
y
 
A
utosomal dominant
 
y
 
M
orphology:
 
o
 
S
pheroidal RBC (normal is biconcavedisc)
 
o
 
No central pallor noted
 
o
 
M
oderate splenomegaly due to markedcongestion of the cords of Billroth
 
o
 
E
rythrophagocytes in the splenic cords
o
 
Features of hemolytic anemia
 
y
 
Clinical course: treatment is splenomegaly
 
o
 
Chronic hemolytic anemia
mild tomoderate
 
o
 
A
plastic crisis
parvovirus infection
 
o
 
H
emolytic crisis
 
o
 
D
iagnosis:
O
smotic Fragility
T
est
 Below:
Cell membrane defect leads to formation of spherocytes, which are sequestered and renderedpalatable to macrophages.
Below:
H
ow primary membrane defect leads tophagocytosis on a chemical basis.
T
hispathophysiology is common to most hemolyticanemia and needs to be known by heart.
Below (next 2 photos):
Note round shape of RBCsand absence of central pallor.
G6PD Deficiency
y
 
X
 linked
y
 
O
ne of the tests for newborn screening
y
 
I
mpaired or deficient enzyme functionwhich reduce ability of red cells to fightagainst oxidative injuries
y
 
A
bnormalities in
H
exose
M
onophosphate
S
hunt pathway or glutathione metabolism
y
 
Need reduced glutathione to protect RBCagainst oxidants
y
 
O
xidant stress:
o
 
D
rugs: antimalarials, sulfonamides, etc
o
 
I
nfection: viral hepatitis,
T
F,pneumonia
o
 
Fava bean ingestion

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