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HEM Exam 1 > Hypoproliferative Anemias > Flashcards

Hypoproliferative Anemias Flashcards

1
Q

reticulocyte index for hypo proliferative anemias

A
  • below 2%
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2
Q

absolute retic count for hypoproliferative anemias

A
  • below 75,000
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3
Q

definition of megaloblastic anemias

A
  • group of disorders characterized by a defect in DNA synthesis leading to a characteristic morphology of bone marrow cells
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4
Q

most common causes of megaloblastic anemias

A
  • B12

- folate deficiency

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5
Q

typical presenting feature of megaloblastic anemia

A
  • anemia
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6
Q

hematologic presentation of megaloblastic anemia

A
  • elevated MCV and RDW
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7
Q

peripheral smear of megaloblastic anemia shows

A
  • hypersegmented PMNs (neutrophils)
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8
Q

non-hematologic presentation of megaloblastic anemia

A
  • beefy red smooth tongue

- neuro/psychiatric features

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9
Q

beefy red smooth tongue seen with which deficiencies

A
  • B12

- folate

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10
Q

neuro/psychiatric features seen with which deficiencies

A
  • B12 only
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11
Q

if you are folate deficient, what precursor will build up?

A
  • homocysteine
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12
Q

if you are B12 deficient, what precursor will build up

A
  • homocysteine

- methylmalonic acid

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13
Q

homocysteine important for

A
  • DNA synthesis
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14
Q

methylmalonic acid important for

A
  • myelin synthesis
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15
Q

homocysteine path

A
  • homocysteine
  • methionine
  • DNA synthesis
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16
Q

methylmalonic acid path

A
  • methylmalonic acid
  • succinic acid
  • myelin synthesis
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17
Q

how does B12 get into the body

A
  • diet

- animal products

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18
Q

parietal cells in stomach secrete

A
  • intrinsic factor
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19
Q

what does B12 bind to

A
  • salivary R protein in acidic environment
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20
Q

role of pancreatic enzymes

A
  • degrade R protein

- frees B12

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21
Q

B12-IF complex taken up by cells in

A
  • distal ileum
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22
Q

B12 carried in blood by ____ to ____

A
  • transcobalamin

- tissues

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23
Q

causes of B12 deficiency

A
  • inadequate dietary intake
  • inadequate absorption
  • reduced B12 absorption in ileum
  • pancreatic insufficiency
  • competition for B12
  • inactivation of cobalamin
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24
Q

inadequate dietary intake - B12 deficiency

A
  • vegan diet

- no animal products

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25
Q

inadequate absorption - B12 deficiency

A
  • lack of gastric acids
  • destruction or removal of parietal cells
  • gastric bypass
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26
Q

destruction/removal of parietal cells by

A
  • pernicious anemia

- gastrectomy

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27
Q

reduced B12 absorption in the ileum

A
  • Crohn’s disease
  • Sprue
  • Metformin
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28
Q

Sprue

A
  • celiac

- tropical

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29
Q

competition for B12

A
  • fish tapeworm,

- bacterial overgrowth

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30
Q

what inactivates cobalamin

A
  • nitrous oxide
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31
Q

neurologic changes of B12 deficiency

A
  • loss of vibration and position sense - subacute degeneration of the spinal cord
  • dementia “megaloblastic mania”
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32
Q

laboratory findings of B12 deficiency

A
  • macrocytosis
  • hypersegements PMNs
  • pancytopenia
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33
Q

lab findings with severe B12 deficiency

A
  • elevated bilirubin and LDH

- due to intramedullary hemolysis

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34
Q

diagnosing B12 deficiency

A
  • low B12 level
  • elevated homocysteine
  • elevated methylmalonic acid
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35
Q

how long does B12 deficiency take to develop

A
  • years
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36
Q

treat B12 deficiency

A
  • treat underlying cause

- IM injection of B12 lifelong

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37
Q

folic acid is absorbed where

A
  • through the small intestine
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38
Q

folate is found in what food products

A
  • uncooked leafy green vegetables

- bread, cereal products

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39
Q

enterohepatic circulation important for

A
  • folate absorption
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40
Q

biliary drainage causes

A
  • profound folate deficiency that occurs within hours
41
Q

extra folate should be given to these people

A
  • patients with hemolytic anemias and psoriasis
  • women contemplating pregnancy
  • pregnant/lactating women
42
Q

folate stores in liver for how long

A
  • 2-5 months only
43
Q

most common cause of folate deficiency

A
  • malnutrition
44
Q

why do we give folate to women contemplating pregnancy

A
  • prevent neural tube defects
45
Q

definition of aplastic anemia

A
  • pancytopenia in peripheral blood w hypocellularity in marrow
46
Q

pancytopenia

A
  • decreased WBCs, RBCs, and platelets
47
Q

bone marrow biopsy of aplastic anemia shows

A
  • hypocellularity

- deficiency of hematopoietic stem cells

48
Q

aplastic anemia can also be considered

A
  • an autoimmune condition
49
Q

secondary causes of aplastic anemia

A
  • ionizing radiation
  • cytotoxic chemotherapy
  • benzene exposure
  • viruses
  • PNH
50
Q

viruses associated with aplastic anemia

A
  • EBV

- non Hep A, B, C, D, or E

51
Q

anemia in aplastic anemia symptoms

A
  • weakness

- fatigue

52
Q

low platelets in aplastic anemia symptoms

A
  • bruising

- oozing

53
Q

low neutrophil counts in aplastic anemia symptoms

A
  • infection fever
54
Q

is there splenomegaly or adenopathy in aplastic anemia

A
  • no!
55
Q

making diagnosis of aplastic anemia

A
  • rule out other causes of pancytopenia
  • think about exposures to drugs, test for viruses
  • test for PNH
  • bone marrow biopsy
56
Q

rule out other causes of pancytopenia

A
  • B12/folate deficiency
  • splenomegaly
  • other marrow toxins such as alcohol
  • autoimmune conditions such as lupus
57
Q

what to look for in bone marrow biopsy

A
  • degree of cellularity
  • rule out cancer, infiltrative disorders
  • rule out dysplasia
58
Q

infiltrative disorders

A
  • sarcoidosis

- fibrosis

59
Q

dysplasia disorders

A

MDS

60
Q

treatment of aplastic anemia

A
  • supportive care

- definitive therapy

61
Q

supportive care of aplastic anemia

A
  • transfusions
  • antibiotics
  • growth factors
62
Q

definitive care of aplastic anemia

A
  • stem cell transplantation

- T cell directed immunosuppression with antithymocyte globulin (ATG) and cyclosporine

63
Q

stem cell transplantation mostly used in

A
  • younger patients
64
Q

T cell directed immunosuppression with antithymocyte globulin (ATG) and cyclosporine mostly used in

A
  • older patients

- those without donor match

65
Q

paroxysmal nocturnal hemoglobinuria is what kind of disorder

A
  • clonal hematologic disorder
66
Q

abnormal clone for paroxysmal nocturnal hemoglobinuria is missing which gene

A
  • PIG-A
67
Q

purpose of PIG-A

A
  • makes GPI
68
Q

purpose of GPI

A
  • anchors several proteins to cell surface
69
Q

most important GPI proteins

A
  • CD55

- CD59

70
Q

function of CD55 and CD59

A
  • inhibit complement on surface of red cells
71
Q

PNH clinical manifestations

A
  • hemolysis at night
  • thrombosis
  • bone marrow failure
72
Q

hemolysis at night in paroxysmal nocturnal hemoglobinuria leads to

A
  • free hemoglobin lost in urine

- leads to development of iron deficiency

73
Q

types of thrombosis in paroxysmal nocturnal hemoglobinuria

A
  • arterial and venous
74
Q

think of paroxysmal nocturnal hemoglobinuria when you see

A
  • Budd Chiari syndrome
75
Q

Budd Chiari syndrome

A
  • thrombosis of the hepatic veins

- leads to hepatic failure

76
Q

PNH leads to predisposition of other diseases such as

A
  • MDS

- AML

77
Q

intravascular hemolysis leads to

A
  • low nitric levels

- defect in smooth muscle relaxation

78
Q

defects in smooth muscle relaxation

A
  • esophageal spasm
  • erectile dysfunction
  • pulmonary hypertension
79
Q

how to treat hemolysis in PNH

A
  • a drug!

- eculizumab

80
Q

MOA of eculizumab

A
  • targets C5

- blocks terminal complement activation

81
Q

eculizumab will increase susceptibility to infection with

A
  • Neisseria organisms
82
Q

most common form of inherited aplastic anemia

A
  • Fanconi’s anemia
83
Q

Fanconi’s anemia genetic linkage

A
  • autosomal recessive

- X-linked

84
Q

Fanconi’s anemia due to mutation in

A
  • 1 of 16 genes for DNA repair
85
Q

clinical manifestations of Fanconi’s

A
  • pancytopenia with macrocytic anemia

- other somatic abnormalities

86
Q

increased incidence of ____ in Fanconi”s

A
  • AML

- solid tumors

87
Q

Fanconi’s usually presents in

A
  • early childhood
88
Q

characteristic congenital mutations of Fanconi’s

A
  • hypo-pigmented spots and cafe-au-lait macules

- abnormalities of thumbs and radii

89
Q

diagnosis of Fanconi’s

A
  • culture lymphocytes or fibroblasts with DEB (diepoxybutane)
  • look for abnormal chromosomal breakage
90
Q

treatment of Fanconi’s

A
  • allogenic transplant

- not much chemotherapy and radiation

91
Q

Dyskeratosis congenita inherited disorder of

A
  • telomere shortening

- inherited form of aplastic anemia

92
Q

Dyskeratosis congenita clinical characteristics

A
  • bone marrow failure

- predisposition to AML and other solid tumors

93
Q

classic triad of Dyskeratosis congenita

A
  • mottled hyperpigmentation of skin involving arms, shoulders, neck, torso
  • abnormal nails of fingers and toes
  • mucosal leukoplakia - white lesions
94
Q

additional findings in Dyskeratosis congenita

A
  • pulmonary fibrosis
  • prematurely gray hair
  • cirrhosis of liver
95
Q

treatment of Dyskeratosis congenita

A
  • allogenic transplant with not much chemotherapy and radiation
96
Q

differential diagnosis of hypoproliferative anemias

A
  • acute blood loss or destruction
  • nutritional anemia
  • bone marrow depression
  • defective RBC production
  • destruction of erythroid precursors
  • replacement of normal marrow

BAD DNR

97
Q

nutrients necessary for normal erythropoesis

A
  • iron
  • B12
  • folic acid
98
Q

defective RBC production by

A
  • kidney disease

- myelodysplasia

99
Q

destruction of marrow erythroid precursors by

A
  • aplastic anemia

- parvovirus infection

HEM Exam 1 (22 decks)

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