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Year 2 Heme lymph > RBC I, HS and Sickle Cell Putthoff > Flashcards

RBC I, HS and Sickle Cell Putthoff Flashcards

1
Q

definition of anemia

A

reduction of total circulating red cell mass below normal limits

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2
Q

microcytic normochromic anemias are generally what disorders

A

disorders of Hb synthesis

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3
Q

MCV tells you what in CBC

A

microcytic, normo or macro

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4
Q

What is Mean cell Hb [ ]

A

average concentration of Hb in given volume of packed RBC

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5
Q

what is RDW

A

ratio of largest RBC to smalles

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6
Q

signsof anemia

A

pale, weakness, malaise and fatigue

dyspnea on mild exertion

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7
Q

what are normal Hb limits

A

male 13-17 female 12-15

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8
Q

what are normal MCV limits

A

80-100

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9
Q

what are normal MCHC limits

A

30-40

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10
Q

what is normal RDW limits

A

11-15

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11
Q

hypoxia due to anemia causes what changes in liver, myocardium and kidney

A

fatty changes

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12
Q

what are complications from acute blood loss

A

oliguria and anuria from renal hypoperfusion

HA dizziness and faintness

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13
Q

what are causes of chronic blood loss

A

GI lesions

gynecologic disturbances

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14
Q

how many days for reticulocytes to be made

A

5

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15
Q

difference in blood cell response to minimal acute bleeding and severe

A

minimal basically only reticulocytosis

severe is reticulocytosis and thrombocytosis

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16
Q

what are common features with hemolytic anemias

A

shortened red cell life span below 120 days
elevated EPO levels
accumulation of Hb degradation products

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17
Q

what is extravascular hemolysis

A

when RBC are destroyed in phagocytes (this is normal)

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18
Q

clinical features of extravascular hemolysis

A

anemia, splenomegaly and jaundice

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19
Q

what is haptoglobin

A

alpha2 globulin that binds free Hb and prevents its excretion in urine (decreased in hemolytic anemia)

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20
Q

what is intravascular hemolysis

A

mechanical injury, complement fixation, intracellular parasites or exogenous toxic factors

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21
Q

causes of intravascular hemolysis

A

cardiac valve damage, thrombotic narrowing

repetative physical trauma

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22
Q

signs of intravascular hemolysis

A

anemia, hemoglobinemia, hemoglobinuria, hemosidinuria and jaundice
NOT splenomegaly

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23
Q

what bilirubin is elevated in hemolytic anemias

A

unconjugated

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24
Q

when is conjugated bilirubin elevated?

A

biliary tract obstruction

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25
what type of Hb abnormalities lead to hemolytic anemia
deficient production | structurally anormal
26
What is hereditary spherocytosis
inherited disorder caused by intrinsic defects in RC membrane skeleton that render red cells spheroid, less deformable and vulnerable to splenic sequestration and destruction
27
What is inheritance pattern of hereditary spherocytosis
autosomal dominant
28
what is compound heterozygosity in hereditary spherocytosis
inheritance of 2 different defects | most severe form
29
red brown urine from hemolytic anemia is from what
methemoglobin because dec haptoglobin
30
gallstones in a patient with hemolytic anemia are most likley composed of what
heme pigments
31
what is hemosiderosis
when there is iron accumulation | hemosiderin (iron containing pigment)
32
what is life span RBC in HS
10-20 days
33
what are the common mutations of HS
ankyrin, band 3 | spectrin or band 4.2
34
do HS cells have normal mean cell Hb [ ]
no it is increased
35
clinical findings of HS
anemia, splenomegaly and jaundice
36
what do the RBC look lik ein HS
spherocytosis (dark staining hyperchromic RBC without central zone pallor)
37
HS get stuck where
in cords of billroth in the spleen and thus prone to macrophages
38
HS are more prone to destruction by hypotonic solutions why
osmotic lysis because influx of water with little room to expand
39
what occurs if the RBC destruction overweighs RBC production
chronic hemolytic anemia
40
what usually causes an aplastic crisis in HS
parvovirus
41
how does parvovirus affect RBC
kills RBC progenitors ceasing RBC production for 1-2 weeks
42
what is Tx for HS patient in aplastic crisis
transfusions until immune response clears the infection
43
what are hemolytic crisis in HS patients
intercurrent events leading to increased splenic destruction of red cells
44
what is Tx for Hs patients
splenectomy
45
What deficiency is an example of hemolytic disease due to RBC enzyme defect
G6PD deficiency
46
what occurs in G6Pd
abnormalities in the hexos monophosphate shunt or glutathione metabolism resulting from deficient or impaired enzyme function reduce ability of red cells to protect themselves against oxidative injuries and lead to hemolysis
47
what causes the episodic hemolysis of G6PD deficiency
exposures that generat oxidant stress like infections
48
What infections can trigger hemolysis in G6Pd
viral hepatitis, pneumonia, thyphoid fever
49
what is characterisitc of G6Pd on peripheral smear
red cells with heinze bodies and bite cells
50
inheritance pattern of G6Pd
recessive X linked trait
51
distribution of G6Pd
mediterannean and men | middle eash
52
what drugs can cause hemolysis in G6Pd
sulfonamides, antimilarials, nitrofurantoins
53
what type of hemolysis occurs in G6Pd
intra and extravascular
54
which variant of G6PD has greater hemolysis
Mediterranean
55
why don't G6PD patients present with splenomegaly or cholelithiasis
because the hemolytic episodes occur intermittently
56
how long does it take for acute intravascular hemolysis to occur in G6PD
2-3 days following exposure to inciting agent
57
What is Sickle cell disease
hereditary hemoglobinopathy caused by point mutation in beta globin that promotes polymerization of deoxygenated Hb leading to RBC distortion, hemolytic anemia, microvascular obstruction and ischemic tissue damage
58
What are the main types adult Hb
HbA (2 alpha 2 beta) | HbA2 (2 alpha 2 gamma)
59
what is Hbf
2 alpha 2 lambda
60
what point mutation is in sickle cell disease
glutamate for valine on 6th codon of beta globulin
61
characteristics of PS of sickle cell
anisocytosis and poikilocytosi
62
what is anisocytosis
different sizes of RBC
63
what is poikilocytosis
different shapes of RBCs
64
2 heterozygotes of sickle cell trait have a child. change of child haveing homozygous?
1/4
65
what is thoughs on why africans have higher HbS
protection against falciparum malaria
66
What are major pathologic findings with HbS
stack into polymers when deoxygenated | cause chronic hemolysis, microvascular occlusions and tissue damage
67
what are clinical signs of sickle cell disease
severe hemolytic anemia assoc with reticulocytosis, hyperbilirubinemia and presence of irreversibly sickled cells
68
what are the "pain crisis" assoc with sickle cell disease
vaso-occlusive crises that are episodes of hypoxic injury and infarction that cause severe pain in affected region
69
most common sites of vaso occlusive crisis in Sickle cell disease
``` bones lungs liver brain spleen penis ```
70
what are the clinical manifestations of the pain crisis in sickle cell
hand foot syndrome or dactylitis
71
why do infants with sickle cell disease not become symptomatic until 5-6 mo old
because HbF prevents polymerization of HbS
72
what is HbS HbC
disease with sickling but not as severe as sickle cell
73
how does mean cell Hb [ ] affect sickle cell disease
increased MCHC facilitates sickling | dec opposite
74
how does pH affect HbS
low pH reduces O2 affinity for Hb | more sickling
75
where does sickling tend to occur
in microvascular beds with slow transit times spleen and bone marrow inflammed tissues
76
what is Acute Chest Syndrome
particularly dangerous type of vaso occlusive crisis involving the lungs which typically presents with fever, cough and chest pain and pulmonary infiltrates
77
severity of hemolysis in sickle cell disease relates to what
the orecentage of irreversibly sickled cells
78
how does sickle cell affec tNO levels
fre eHb released from lysed cells binds and inactivates NO
79
how do the vasoocclusive crisis affect the eyes in sickle cell disease
loss of visual acuity, even blindness
80
what are Howell Jolly bodies
small nuclear remnatns in red cells due to asplenia
81
"crew-cut" on XRay is indicative of what
expansion of marrrow
82
what is autosplenectomy and when is it seen
enlargement of spleen early childhood that leads to infarction, fibrosis etc
83
what are sequestration crisis
children with intact spleens that have rapid enalrgement leading to hypovolemia and sometimes shock
84
what are aplastic crisis in sickle cell disease
infection of RBC progenitors with parvovirus B19
85
what does the chronic hypoxia fro sickle cell cause
impairment of growth and development organ damage of spleen heart kidneys and lungs hypertropy renal medulla that could lead to hyposthenuria
86
what infections are sickle cell patients mroe prone to
pneumococcus, meningococcus, H influenza
87
how is fetal Dx of sickle cell known
amniocentesis or chorionic biopsy
88
Tx for sickle cell
DNA synthesis inhibitor hydroxyurea | stem cell transplantation maybe cure

Year 2 Heme lymph (28 decks)

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