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FIRST AID ONE LINERS > HEME ONC 1 REDS > Flashcards

HEME ONC 1 REDS Flashcards

1
Q

This is the most common porphyria ________ and this enzyme is defective ___________

A

Porphyria cutanea tarda; uroporphyrinogen decarboxylase

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2
Q

What enzymes are affected by lead poisoning and what substrates accumulate?

A

Ferrochetalase and delta-ALA dehydratase; protoporphyrin and delta ALA

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3
Q

Macrophages are activated by this cytokine _________ and are identified by this cluster of differentiation marker_____________

A

IFN-gamma; CD14

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3
Q

What is defective in Bernard Soulier syndrome?

A

GpIb on the platelet, which binds to vWF on the damaged endothelium

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4
Q

Which RBC transporter allows them to to sequester HCO3?

A

HCO3/Cl antiporter, also responsible for chloride shift

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5
Q

Which disease involves an absence of GpIb? What process is affected?

A

Bernard-Soulier Syndrome (big suckers)? Platelet adhesion to vWF is affected

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5
Q

Name 3 causes of megaloblastic anemia

A

B12 deficiency, folate deficiency, orotic aciduria

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6
Q

What are the NAACP causes of eosinophilia?

A

Neoplasia, Asthma, Allergies, Collagen Vascular Disease, Parasites

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6
Q

With which disease are macro-ovalocytes associated?

A

Megaloblastic anemia (along with hypersegmented polys) and marrow failure

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6
Q

What is defective in thrombotic thrombocytopenic purpura?

A

ADAMTS-13 a vWF metalloprotease; since vWF multimers not broken down, leads to excessive binding up of platelets causing obstruction in the vessel = microangiopathic hemolytic anemia

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7
Q

What is defective in Glanzmann’s thrombasthenia?

A

Defective GpIIb/IIIa leading to defective aggregation

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8
Q

Which proteins are defective in hereditary spherocytosis? Why is there anemia? What is the Tx?

A

Spectrin, ankyrin, band 3, and protein 4.2; there is anemia because the defective shape leads to early removal by the spleen; tx = splenectomy

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9
Q

Multiple myeloma is the most common primary tumor of this tissue_________ and is composed of these cells ___________

A

bone; plasma cells

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10
Q

Define poikilocytosis

A

varying shapes of blood cells

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11
Q

Where do prothrombin gene mutations occur?

A

3’ UTR, leads to venous thrombosis

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12
Q

What are 3-4 disease processes that you may see schistocytes in?

A

DIC, TTP (thrombotic thrombocytopenic purpura, ADAMTS-13), HUS (hemolytic uremic syndrome, shiga-like toxin), mechanical heart valves and aortic stenosis

Note: TTP involves this d/t shearing across the platelet aggregates, ITP on the other hand is actually an extravascular hemolysis occuring in the spleen

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13
Q

Where is the location of a large amount of the platelet pool?

A

the spleen… 1/3 of platelets are stored in the spleen

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14
Q

Differentiate the signs of a platelet disorder vs. a coagulation disorder

A

platelet disorders present with petechiae and mucosal bleeding whereas coagulation disorders present with deep bleeding into joints

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15
Q

What cellular inclusions are you likely to see in sickle cell patients and why?

A

Howell-Jolly bodies because these are nuclear remnants that are normally removed by the spleen but in Sickle Cell they get autosplenectomy

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15
Q

What disease does ecluzimab treat?

A

paroxysmal nocturnal hemoglobinuria

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16
Q

Who are the cis and trans deletions of alpha thalassemia the most common in?

A

cis = Asian; trans = African

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18
Q

Name the WBC differential form highest cell concentration to the lowest cell concentration

A

Neutrophil, Lymphocyte, Monocyte, Eosinophil, Basophil

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18
Q

Liver disease and abetalipoproteinemia can lead to this spiny looking RBC

A

acanthocyte (spur cell)–deposition of cholesterol into the plasma membrane

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19
Q

Describe the 1,2,3, and 4 gene deletions of the alpha thalassemias

A

1-2 have little significance, 3 gene deletion leads to HbH disease in which you actually get a beta-tetramer (= HbH), and the 4 gene deletion results in gamma tetramers i.e. hemoglobin barts = hydrops fetalis

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19
Q

What is the risk of administering warfarin in a person with protein C/S deficiency?

A

Thrombotic skin necrosis with hemorrhage, this is because factors V and VIII are inactivated by proteins C and S so there will be hypercoagulability in these patients

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20
Q

What are the clinical uses for giving cryoprecipitate?

A

treating coagulation factor deficiencies involving fibrinogen and factor VIII

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21
Q

Which 2 enzymes are inhibited in lead poisoning? What is a major risk factor?

A

heme synthesis proteins = ferrochetalase and ALA dehydratase; homes with chipped paint

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22
Q

Name 4 causes of renal papillary necrosis

A

Sickle cell disease, Obstructive pyelonephritis, DM, and Analgesics (SODA mneomonic)

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23
Q

What is the clinical use for administering packed RBC’s? (2)

A

Acute blood loss or severe anemia

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24
Q

Which disease is associated strongly with dacryocytes?

A

myelofibrosis (teardrop cells) causes a myelophthisic anemia by forcing the RBC’s out of the fibrous marrow

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25
Q

How are the actions of ticlopidine/clopidogrel similar and different from abciximab?

A

All ultimately end up blocking platelet aggregation via messing with GpIIb/IIIa; however clopidogrel/ticlopidine inhibit the ADP receptor so that GpIIb/IIIa cannot be expressed whereas abciximab binds directly to GpIIb/IIIa

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26
Q

What is the triad of paroxysmal nocturnal hemoglobinuria?

A

hemolytic anemia, pancytopenia, venous thrombosis

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27
Q

Why is there anemia in chronic disease (i.e. anemia of chronic disease)?

A

Because the chronic inflammation leads to an increase in the release of hepcidin from the liver which binds ferroportin on intestines thus inhibiting the uptake of iron. It also does this on macrophages which decreases the TIBC

Evolutionarily, this may be to keep iron away from bugs.

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28
Q

Which 4-5 anemias are microcytic, hypochromic?

A

IRON DEFICIENCY ANEIMA (MOST COMMON BY FAR)? Alpha thalassemia, beta thalassemia, sideroblastic anemia, and Lead poisoning (which causes sideroblastic anemia)

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29
Q

When present in the skin, these cells are called Langerhans cells

A

dendritic cells

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30
Q

Triad of Plummer Vinson

A

Iron deficiency anemia (microcytic, hypochromic), atrophic glossitis, and esophageal webs

31
Q

What blood type is the universal donor of PLASMA? Why?

A

AB because they have no antibodies to either A or B

32
Q

2 diseases associated with spherocytes

A

hereditary spherocytosis, autoimmune hemolysis

33
Q

Name 4 diseases associated with target cells (i.e. HALT said the hunter to his target)

A

HbC disease, Asplenia, Liver disease, Thalassemia

34
Q

What is the pentad of Sx in TTP?

A

NEUROLOGIC (not in HUS), renal (but more in HUS), fever, thrombocytopenia, microangiopathic hemolytic anemia

35
Q

Why do infants initially have asymptomatic sickle cell? How does the drug hydroxyurea work to improve the disease?

A

Because they initially have more fetal hemoglobin than than HbS? Hydroxyurea acetylyates histones to open them up so that gamma globin can be made so that more HbF can be present in the adult than HbS

36
Q

How does pyruvate kinase deficiency present?

A

Hemolytic anemia of the newborn

37
Q

G6PD deficient patients can have exacerbations from what 3 sources? What will you see on a peripheral blood smear?

A

Fava beans, sulfonamides + anti-malarials, infections, also dapsone; heinz bodies in RBC’s and bite cells

38
Q

Name 4 causes of cold autoimmune hemolytic anemia. What is the antibody isotype?

A

Cold weather, CLL, EBV, and Mycoplasma pneumoniae? IgM

39
Q

Which 2 drugs are 1st line for lead poisoning in adults? Kids?

A

Adult = Dimercaprol and EDTA; Kid = succimier (sucks to be a kid who eats lead)

41
Q

With which disease are bite cells associated? Explain how they come to be.

A

Bite cells are RBC’s with chunks missing from G6PD deficiency. The oxidation of ferrous to ferric ends up denaturing hemoglobin. This damages the membrane of the RBC and when the cell gets to the spleen, the resident MO take a “bite” out of it; G6PD deficient RBC’s bite the dust.

42
Q

What enzyme is defective in acute intermittent prophyria?

A

Porphobilinogen deaminase

43
Q

What are 2 infectious etiologies associated with cold autoimmune hemolytic anemia? Ab isotype?

A

EBV and Mycoplasma pneumoniae? IgM

44
Q

What is the Tx for von Willebrand disease? How is it diagnosed?

A

Desmopressin (DDAVP)!!!!! Causes release of vWF from Weibel-Palade bodies? Ristocetin cofactor assay

45
Q

Which receptor on the platelet binds to vWF on the endothelium?

A

GPIb

46
Q

What 2 ways are normochromic anemias classified?

A

hemolytic and non hemolytic

46
Q

What causes paroxysmal noctural hemoglobinuria?How is it treated?

A

A deficiency in CD55/59 which is a GPI anchor that helps to prevent complement mediated lysis ends up leading to excess hemolysis by complement, ccluzimab

48
Q

How are the Sx of megaloblastic different when caused by folate vs. B12

A

B12 has neuro Sx

49
Q

The hereditary cause of sideroblastic anemia is caused by a defect in this enzyme_________ , is inherited in this fashion _________ and is treated with ____________.

A

Delta-ALA synthase; X-linked recessive; pyridoxine (B6) because it is a cofactor for delta-ALA synthase!

50
Q

What is the difference between beta thalassemia minor and major? What untoward effect from blood transfusions might you see in thalassemia major? What does an X-ray of the skull look like?

A

Beta minor is a heterozygous condition in which there is some beta globin produced, beta major is absent beta globin? The treatment is infusions and may lead to secondary hemochromatosis? The skull may look like a crew cut

51
Q

What is the issue in factor V leiden?

A

Mutation that leads to inability of factor V to be degraded by Protein C leading to hypercoagulability

53
Q

Which active factor is destroyed by protein C but resistant in this disease _____________ leading to hypercoagulability?

A

factor V; Factor V Leiden

54
Q

What are the clinical uses for giving Fresh Frozen plasma?

A

DIC, cirrhosis, and warfarin overdose (immediate, vitamin K for later)… All of these situations need coagulation factors

56
Q

Which IBD causes megaloblastic anemia and why?

A

Crohn’s because it affects the entire GI system and B12 absorbed in distal ileum whereas UC only affects colon

57
Q

Which co-stimulatory molecule is present on the T cell? Which is present on the APC?

A

CD28; B7

58
Q

The exposure of this molecule on the surface of injured endothelium is key to initiating platelet adhesion

A

vWF, binds to GpIb on platelet; vWF is stored along with P-selectin in Weibel-Palade bodies of endothelial cells

59
Q

What cell type is identified in microangiopathic hemolytic anemias?

A

schistocytes

60
Q

What are 2 clinical uses for giving platelets?

A

To stop significant bleeding or qualitative platelet defects (Bernard Soulier, Glanzmann’s)

61
Q

With which disease are elliptocytes associated?

A

hereditary elliptocytosis

61
Q

What is a Howell-Jolly Body? What do they indicate?

A

It is a basophilic nuclear remnant in an otherwise mature RBC. They are normally removed by the spleen, so their presence indicates asplenism or hyposplenia. Could also be from ingestion of NAPTHALENE i.e. mothballs

62
Q

Which receptor on the platelet binds to fibrinogen for secondary hemostasis?

A

GpIIb/IIIa

63
Q

With which Dz are ringed sideroblasts associated? What is going on?

A

Sideroblastic anemia resulting from iron overload in the mitochondria of developing RBC’s, Fe = mitochondrial toxin… Although it is excess iron it is actually assoicated with lead poisoning since it affects the synthesis of heme which involves iron

64
Q

Which active clotting factors are inactivated by antithrombin III?

A

factors II, VII, IX, X, XI, and XII

65
Q

What is indicated by a hypersegmented neutrophil?

A

megaloblastic anemia (B12 or folate deficiency, or both, OR orotic aciduria)

67
Q

Which type of hemolytic anemia is associated with chronic lymphocytic leukemia (CLL)?

A

BOTH warm (IgG) and cold (IgM) autoimmune hemolytic anemia

68
Q

Which disease involves an absence of GPIIb/IIIa? What process is affected?

A

Glanzmann’s thrombasthenia; platelet aggregation

69
Q

Which 2 products of eosinophils help to limit the reaction following mast cell degranulation? Which is also deficient in a hereditary CNS disorder?

A

Histaminase and Arylsulfatase; Arylsulfatase A is deficient in metachromatic leukodystrophy

70
Q

Basophilic stippling of RBCs’ involves the inhibition of rRNA degradation leading to basophilic pieces of rRNA in RBC’s and can by caused by these 3 things

A

Thalassemias, Anemia of chronic disease, lead poisoning

71
Q

What is HbH disease?

A

A three gene deletion of the alpha globin gene, a form of alpha thalassemia in which beta globin tetramers form and these are called HbH

72
Q

Which form of vitamin K is required for activating these 6 factors___________.

A

reduced, epoxide reductase reduces the oxidized form of vitamin K? Factors II, VII, IX, X, and proteins C/S

74
Q

This drug helps to prevent mast cell degranulation by inhibiting a chloride channel

A

cromolyn sodium

75
Q

How do Heinz bodies form?

A

In G6PD, the ROS that accumulate oxidize ferrous to ferric leading to denaturation of hemoglobin which then sticks in the membrane

77
Q

Name 3 major causes of warm autoimmune hemolytic anemia. What is the antibody isotype?

A

SLE, CLL, and alpha-methyldopa; IgG

78
Q

What is the issue in idiopathic thromocytopenic purpura?

A

there is an antibody to GpIIb/IIIa which marks the platelet for destruction by the spleen, i.e. thrombocytoPENIA

79
Q

What are 2 infectious agents that primarily infect RBC’s?

A

Babesia microtic (co-infect with Lyme); Malaria

80
Q

How does megaloblastic anemia caused by orotic aciduria differ in the Tx? What enzyme is deficient?

A

it is Tx’d with uridine monophosphate; it presents as a megaloblastic anemia in children that is not cured by B12 admin? UMP synthase

82
Q

Absence of this receptor leads to complete resistance to malaria

A

Duffy receptor on RBC

83
Q

Why might the PTT be elevated in vWF disease?

A

Because vWF binds and protects factor VIII, since PTT tests factor VIII among many factors, a lower level of factor VIII can raise the PTT? However, the PTT can be normal

84
Q

Define anisocytosis

A

varying sizes of cells

85
Q

What is the mutation in sickle cell anemia?

A

substitution of glutamic acid with valine at position 6 on the Beta globin chain

86
Q

What blood disease can be treated with desmopressin and why?

A

DDAVP causes release of vWF from Weibel-Palade bodies which is used to Tx von Willebrand’s disease

87
Q

Ingestion of napthalene (mothballs) may cause this inclusion body in RBC’s

A

Howell-Jolly bodies

88
Q

What hematologic side effect may alpha-methyldopa have?

A

Warm autoimmune hemolytic anemia (IgG)

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