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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
Q

what type of Hb abnormalities lead to hemolytic anemia

A

deficient production

structurally anormal

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26
Q

What is hereditary spherocytosis

A

inherited disorder caused by intrinsic defects in RC membrane skeleton that render red cells spheroid, less deformable and vulnerable to splenic sequestration and destruction

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27
Q

What is inheritance pattern of hereditary spherocytosis

A

autosomal dominant

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28
Q

what is compound heterozygosity in hereditary spherocytosis

A

inheritance of 2 different defects

most severe form

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29
Q

red brown urine from hemolytic anemia is from what

A

methemoglobin because dec haptoglobin

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30
Q

gallstones in a patient with hemolytic anemia are most likley composed of what

A

heme pigments

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31
Q

what is hemosiderosis

A

when there is iron accumulation

hemosiderin (iron containing pigment)

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32
Q

what is life span RBC in HS

A

10-20 days

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33
Q

what are the common mutations of HS

A

ankyrin, band 3

spectrin or band 4.2

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34
Q

do HS cells have normal mean cell Hb [ ]

A

no it is increased

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35
Q

clinical findings of HS

A

anemia, splenomegaly and jaundice

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36
Q

what do the RBC look lik ein HS

A

spherocytosis (dark staining hyperchromic RBC without central zone pallor)

37
Q

HS get stuck where

A

in cords of billroth in the spleen and thus prone to macrophages

38
Q

HS are more prone to destruction by hypotonic solutions why

A

osmotic lysis because influx of water with little room to expand

39
Q

what occurs if the RBC destruction overweighs RBC production

A

chronic hemolytic anemia

40
Q

what usually causes an aplastic crisis in HS

A

parvovirus

41
Q

how does parvovirus affect RBC

A

kills RBC progenitors ceasing RBC production for 1-2 weeks

42
Q

what is Tx for HS patient in aplastic crisis

A

transfusions until immune response clears the infection

43
Q

what are hemolytic crisis in HS patients

A

intercurrent events leading to increased splenic destruction of red cells

44
Q

what is Tx for Hs patients

A

splenectomy

45
Q

What deficiency is an example of hemolytic disease due to RBC enzyme defect

A

G6PD deficiency

46
Q

what occurs in G6Pd

A

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
Q

what causes the episodic hemolysis of G6PD deficiency

A

exposures that generat oxidant stress like infections

48
Q

What infections can trigger hemolysis in G6Pd

A

viral hepatitis, pneumonia, thyphoid fever

49
Q

what is characterisitc of G6Pd on peripheral smear

A

red cells with heinze bodies and bite cells

50
Q

inheritance pattern of G6Pd

A

recessive X linked trait

51
Q

distribution of G6Pd

A

mediterannean and men

middle eash

52
Q

what drugs can cause hemolysis in G6Pd

A

sulfonamides, antimilarials, nitrofurantoins

53
Q

what type of hemolysis occurs in G6Pd

A

intra and extravascular

54
Q

which variant of G6PD has greater hemolysis

A

Mediterranean

55
Q

why don’t G6PD patients present with splenomegaly or cholelithiasis

A

because the hemolytic episodes occur intermittently

56
Q

how long does it take for acute intravascular hemolysis to occur in G6PD

A

2-3 days following exposure to inciting agent

57
Q

What is Sickle cell disease

A

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
Q

What are the main types adult Hb

A

HbA (2 alpha 2 beta)

HbA2 (2 alpha 2 gamma)

59
Q

what is Hbf

A

2 alpha 2 lambda

60
Q

what point mutation is in sickle cell disease

A

glutamate for valine on 6th codon of beta globulin

61
Q

characteristics of PS of sickle cell

A

anisocytosis and poikilocytosi

62
Q

what is anisocytosis

A

different sizes of RBC

63
Q

what is poikilocytosis

A

different shapes of RBCs

64
Q

2 heterozygotes of sickle cell trait have a child. change of child haveing homozygous?

A

1/4

65
Q

what is thoughs on why africans have higher HbS

A

protection against falciparum malaria

66
Q

What are major pathologic findings with HbS

A

stack into polymers when deoxygenated

cause chronic hemolysis, microvascular occlusions and tissue damage

67
Q

what are clinical signs of sickle cell disease

A

severe hemolytic anemia assoc with reticulocytosis, hyperbilirubinemia and presence of irreversibly sickled cells

68
Q

what are the “pain crisis” assoc with sickle cell disease

A

vaso-occlusive crises that are episodes of hypoxic injury and infarction that cause severe pain in affected region

69
Q

most common sites of vaso occlusive crisis in Sickle cell disease

A 
bones
lungs
liver 
brain
spleen 
penis
70
Q

what are the clinical manifestations of the pain crisis in sickle cell

A

hand foot syndrome or dactylitis

71
Q

why do infants with sickle cell disease not become symptomatic until 5-6 mo old

A

because HbF prevents polymerization of HbS

72
Q

what is HbS HbC

A

disease with sickling but not as severe as sickle cell

73
Q

how does mean cell Hb [ ] affect sickle cell disease

A

increased MCHC facilitates sickling

dec opposite

74
Q

how does pH affect HbS

A

low pH reduces O2 affinity for Hb

more sickling

75
Q

where does sickling tend to occur

A

in microvascular beds with slow transit times
spleen and bone marrow
inflammed tissues

76
Q

what is Acute Chest Syndrome

A

particularly dangerous type of vaso occlusive crisis involving the lungs which typically presents with fever, cough and chest pain and pulmonary infiltrates

77
Q

severity of hemolysis in sickle cell disease relates to what

A

the orecentage of irreversibly sickled cells

78
Q

how does sickle cell affec tNO levels

A

fre eHb released from lysed cells binds and inactivates NO

79
Q

how do the vasoocclusive crisis affect the eyes in sickle cell disease

A

loss of visual acuity, even blindness

80
Q

what are Howell Jolly bodies

A

small nuclear remnatns in red cells due to asplenia

81
Q

“crew-cut” on XRay is indicative of what

A

expansion of marrrow

82
Q

what is autosplenectomy and when is it seen

A

enlargement of spleen early childhood that leads to infarction, fibrosis etc

83
Q

what are sequestration crisis

A

children with intact spleens that have rapid enalrgement leading to hypovolemia and sometimes shock

84
Q

what are aplastic crisis in sickle cell disease

A

infection of RBC progenitors with parvovirus B19

85
Q

what does the chronic hypoxia fro sickle cell cause

A

impairment of growth and development
organ damage of spleen heart kidneys and lungs
hypertropy renal medulla that could lead to hyposthenuria

86
Q

what infections are sickle cell patients mroe prone to

A

pneumococcus, meningococcus, H influenza

87
Q

how is fetal Dx of sickle cell known

A

amniocentesis or chorionic biopsy

88
Q

Tx for sickle cell

A

DNA synthesis inhibitor hydroxyurea

stem cell transplantation maybe cure

Year 2 Heme lymph (28 decks)

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