Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Hematology Exam 1 > Hypoproliferative Anemias > Flashcards

Hypoproliferative Anemias Flashcards

1
Q

What is the first lab value that would explicitly denote a hypo-proliferative anemia?

A

Low retic count

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

B12 and folate deficiency are the most common causes of what kind of anemia?

A

Hypoproliferative macrocytic megaloblastic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

A retic index of less than 2% or absolute retic count less than 75,000 is indicative of a ____ proliferative anemia

A

Hypo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Cobalamine = Vitamin ___

A

B12

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Megaloblastic anemias are defined as a group of disorders characterized by a defect in _______ that leads to an immature nucleus and mature cytoplasm

A

DNA synthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

On a peripheral smear of someone with megaloblastic anemia, what will be the morphology of the neutrophils?

A

Hyper-segmented (so more than 5 lobes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

In megaloblastic anemias, the MCV is ____ and the RDW is ____

A

Elevated; elevated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are some non-hematologic findings of someone with megaloblastic anemia?

A

Glossitis (beefy red smooth tongue) seen in both B12 and folate deficiency and neuro/psychiatric features only seen in B12 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Would you see neuro/psychiatric features in someone that is folate deficient?

A

No, just B12 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Homocysteine is needed to form ______ that is needed for DNA synthesis

A

Methionine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Folate is needed to form ______

A

Methionine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

B12 is needed to form ______ and succinic acid

A

Methionine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

B12 is needed to form methionine and _______ acid

A

Succinic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Methylmalonic acid forms ______ acid

A

Succinic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

______ acid forms succinic acid

A

Methylmalonic acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Methylmalonic acid forms ______ acid that then goes to aid in myelin synthesis

A

Succinic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

B12 from the diet binds to what protein in the saliva?

A

Protein R

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

_____ cells in the stomach secrete intrinsic factor

A

Parietal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Parietal cells in the _____ secrete ____ ____

A

Stomach; intrinsic factor (IF)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Enzymes produced by the ____ free B12 from being bound to protein R

A

Pancreas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

B12-IF complex is taken up in the distal _____

A

Ileum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

B12 is bound to [protein R/ IF] in the distal illeum

A

IF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

In the blood, B12 is carried by _______ to the tissues

A

Transcobalamin II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What type of diet would cause B12 deficiency?

A

Vegan diet (no animal products)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Inadequate absorption of B12 may be due to what causes?

A
  • Lack of gastric acid (meds, atrophic gastritis) - Destruction/removal of parietal cells (pernicious anemia, gastrectomy) - Gastric bypass surgery
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

In pernicious anemia, what is causing the lack of absorption of B12?

A

Autoimmune loss of parietal cells that produce IF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Where in the body is most B12 stored?

A

Liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is necessary for B12 to be absorbed?

A

Intrinsic factor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What enzymes are needed to free up B12 to bind IF?

A

Pancreatic enzymes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What are some conditions that can reduce B12 absorption in the ileum?

A
  • Crohn’s disease - Sprue (celiac or tropical) - Metformin (diabetes treatment)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

If there is a problem with the pancreas, what step of B12 absorption will be affected?

A

The step where pancreatic enzymes cleave protein R from B12

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What are some competitors of B12 absorption that can be in the body?

A

Fish tapeworm, bacterial overgrowth

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What molecule may inactivate cobalamin?

A

Nitrous oxide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What neurological condition can be caused by B12 deficiency and reversible if caught early?

A

Dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

In B12 deficiency there will be subacute combined degeneration of the _____ and lateral spinal columns

A

Dorsal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

There is a loss of _____ and position sense in B12 deficiency that is presented with tripping and falling and having problem buttoning shirt

A

Vibration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Pernicious anemia runs in families and on a smear would present with

A

Pancytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

In someone who is B12 deficient, what would you expect their lab values would be?

A
  • Increased MCV (macrocytic) - Hypersegmented PMNs (neutrophils) - There could be pancytopenia if severe - Increased LDH and bilirubin due to intramedullary hemolysis in severe cases (pernicious anemia)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

How would you diagnose B12 deficiency?

A
  • Low B12 level - Elevated homocysteine - Elevated methylmalonic acid
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

If have someone who underwent gastric bypass surgery two months ago and is not taking B12 supplements, would you expect them to be severely B12 deficient?

A

No, it takes years (10) to develop B12 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Should you just go ahead and give B12 to someone with a deficiency first?

A

No, you should try to treat the underlying cause first

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

B12 deficiency generally requires lifelong _______ shots of B12

A

Intramuscular (IM)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Where is folate absorbed?

A

Small intestine (mainly to duodenum and jejunum)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Where in the diet is folate found?

A

Uncooked green leafy veggies, bread and cereals

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

_______ hepatic circulation is important for folate absorption and if a bile drain is put in, all folate may be lost in 6 hours

A

Enterohepatic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Cooking destroys _____ in veggies

A

Folate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

What is the daily folate requirement for adults?

A

100 mcg

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What is the daily folate requirement for children?

A

50 mcg

49
Q

What is the daily folate requirement for pregnant or lactating women?

A

400 mcg

50
Q

Someone with psoriasis or hemolysis should take what kind of supplements?

A

Folate

51
Q

Where in the body is folate stored and for how long?

A

Liver; 2-5 months

52
Q

Which parts of the small intestine are iron and folate absorbed?

A

Duodenum and jejunum

53
Q

What are some causes of folate deficiency?

A
  • Malnutrition - Malabsorption - Increased requirement (pregnancy, chronic hemolysis, psoriasis, chronic febrile illnesses) - Drugs (methotrexate, sulfa antibiotics)
54
Q

What is the most common cause of folate deficiency?

A

Malabsorption

55
Q

Patients with what kind of anemias should be supplemented with folate?

A

Hemolytic

56
Q

About how much folate should be ingested in a day?

A

1-5 mg

57
Q

______ will be increased and _______ ____ will be normal in folate deficiency

A

Homocysteine; methylmalonic acid

58
Q

Methylmalonic acid levels will be normal in _____ deficiency because only ___ is needed in the conversion to succinate

A

Folate; B12

59
Q

Aplastic anemia is defined as:

A

Pancytopenia seen in the peripheral blood smear, with a bone marrow biopsy showing hypocellularity (<25%) that arises from deficiency of hematopoietic stem cells

60
Q

What type of anemia is there pancytopenia and hypocellularity of the bone marrow?

A

Aplastic anemia

61
Q

Most aplastic anemias are ______ conditions

A

Autoimmune

62
Q

Aplastic anemia is what kind of condition?

A

Autoimmune

63
Q

What condition is depicted

A

Aplastic anemia

64
Q

This is seen in what condition?

A

Megaloblastic anemia likely caused by B12 deficiency

65
Q

What is the primary cause of aplastic anemia?

A

Idiopathic; autoimmune

66
Q

What are some main secondary causes of aplastic anemia?

A
  • Ionizing radiation
  • Cytotoxic chemotherapy
  • Benzene exposure
  • Paroxysmal Nocturnal Hemoglobinuria (PNH)
  • Viruses: EBV and hepatitis (non A, B, C, D, and E)
67
Q

In someone with aplastic anemia, will there be splenomegaly?

A

No

68
Q

The presentation of aplastic anemia is consistent with deficiencies in what cell lines?

A

Red, white, and platelets (pancytopenia)

69
Q

In someone with AA, why would the patient be more susceptible to infection and fever?

A
  • Low neutrophil count (neutropenia)
70
Q

If a patient is presenting with symptoms of AA and have pancytopenia, then what conditions should you rule out first?

A
  • B12/folate deficiency
  • Splenomegaly
  • Alcohol is a marrow toxin, so check for excess intake
  • Lupus
71
Q

In order to make a proper diagnosis of AA, you should rule out other causes of _______ and do a ___ ____ biopsy

A

Pancytopenia; bone marrow

72
Q

To make a diagnosis of pure AA, what should be done?

A
  1. Rule out other causes of pancytopenia
  2. Think about possible exposures to drugs and test for viruses
  3. Test for PNH
  4. Do a bone marrow biopsy
73
Q

When a bone marrow biopsy is done to diagnose AA, what should you be looking for?

A
  • Degree of cellularity
  • Rule out cancer, infiltrative disorders (sarcoidosis, fibrosis)
  • Rule out dysplasia (MDS)
74
Q

When diagnosing AA, what is the main test that should be done?

A

Bone marrow biopsy

75
Q

What are some treatment options for AA?

A

Supportive care

  1. Transfusions
  2. Antiobiotics
  3. Growth Factors

Definitive Care

  1. Stem cell transplant
  2. T- cell directed immunosuppression with antithymocyte globulin (ATg) and cyclosporine (relapses may occur)
76
Q

In treatment of AA, what treatment would be preferred in younger patients?

A

Stem cell transplant

77
Q

What type of stem cell transplant would be given to someone with AA?

A
  • Allogenic bc is a source of undamaged stem cells
78
Q

To treat certain cancers (leukemias), what stem cell transplant could be used when you want to do a lot of chemotherapy?

A

Autologous (patient’s own marrow) or peripheral blood stem cells (PBSC); there is no GVHD because the graft is the host

79
Q

What is hemoglobinuria?

A

Hemoglobin in the urine

80
Q

What is the definition of Paroxysmal Nocturnal Hemoglobinuria (PNH)?

A

An acquired clonal hematologic disorder in which an abnormal stem cell clone develops that gives rise to red cells, white cells, and platelets that are all missing proteins attaced to the cell surface via the GPI anchor

81
Q

The abnormal clone in PNH is missing the ____ gene that is responsible for making GPI

A

PIG-A

82
Q

The PIG-A gene encodes ___ that anchors CD55 and CD59 to the surface of red cells

A

GPI

83
Q

What are the 2 GPI anchored proteins that are missing in PNH that are important in inhibiting complement binding to red cells?

A

CD55 and CD59

84
Q

CD55 and CD59 are important in inhibiting _____ on the surface of red cells

A

Complement

85
Q

PNH red cells are extremely sensitive to what kind if red cell destruction?

A

Complement-mediated

86
Q

At what time of the day does the pH in the blood fall, which leads to more complement-mediated lysis?

A

Night

87
Q

In PNH, what happens at night?

A

Hemolysis occurs and in the morning patients urinate and they get rid of hemoglobin (hemoglobinuria) and that leads to iron deficiency

88
Q

____ deficiency is seen in patients with PNH

A

Iron

89
Q

Thrombosis in the arteries and ____ occurs in PNH

A

Veins

90
Q

Budd Chiari syndrome

A

Associated with PNH and is thrombosis of the hepatic vein that leads to liver failure

91
Q

Budd-Chiari Syndrome is associated with what condition?

A

PNH

92
Q

In PNH, patients will have ___ ____ failure as well as will be at a predisposition for MDS and ___

A

Bone marrow; AML (leukemia)

93
Q

The intravascular hemolyis in PNH leads to low ___ ___ levels that lead to a defect in smooth muscle relaxation/contraction

A

Nitric oxide; relaxation

94
Q

Patients with PNH may present with constriction of smooth muscle that leads to esophageal spasms, ____ dysfunction, and pulmonary hypertension

A
  • Erectile
95
Q

Eculizumab may be given to patients with _________

A

PMH

96
Q

What is the MOA of eculizumab?

A
  • Targets C5 and blocks activation of the terminal complement (MAC complex)
97
Q

Someone on eculizumab is more suscpetible to what type of infections?

A

Neisseria

98
Q

What are the treatments for PNH?

A
  • Iron pills
  • Anticoagulation
  • ATG and cyclosporine (immunosuppression)
  • Eculizumab
99
Q

Fanconi’s Anemia is the most common form of inherited ____

A

AA

100
Q

What is the inheritance pattern of Fanconi’s anemia?

A

Autosomal recessive or X-linked

101
Q

Someone with fanconi’s anemia may present with ________ and then the disease progresses to pancytopenia

A

Thrombocytopenia

102
Q

PNH can develop into AA or ___

A

AML

103
Q

Clinical manifestations of fanconi’s anemia

A
  • Pancytopenia
  • Macrocytic
  • May start with thrombopenia and then progress
  • Somatic abnormalities: (short stature, cafe-au-lait macules, weird thumbs, micro- or hydrocephaly, hypogonadism, developmental delay)
  • Increased incidence of acute leukemia and tumors
  • Usually presents in childhood
104
Q

What are some characteristic congential mutations of fancon’s anemia?

A
  • short stature
  • cafe-au-lait macules
  • weird thumbs
  • micro- or hydrocephaly
  • hypogonadism
  • developmental delay
105
Q

To diagnose for fanconi’s you should take the lymphocytes or fibroblasts and then add ___ and look for abnormal ______ breakage

A

Diepoxybutane (DEB); chromosomal

106
Q

You should suspect ___ in any child or young adult with bone marrow failure

A

FA

107
Q

Suspect ___ in patients who have cancer at a young age or who have familial cancer

A

FA

108
Q

To treat fanconi’s anemia, you should perform an _______ transplant with reduced intensity pre-transplant conditioning

A

Allogenic

109
Q

People with Fanconi’s anemia have increased toxicity from _____ and chemotherapy

A

Radiation

110
Q

What causes fanconi’s anemia?

A

A mutation in one of the 16 genes responsible for DNA repair –> several of these genes are tumor suppressors

111
Q

Dyskeratosis congenita is an inherited disorder of _____ shortening and is also an inherited form of __

A

-telomeres; AA

112
Q

What are some clinical characterisics of dyskeratosis congenita?

A
  • Bone marrow failure
  • Predisposition to acute leukemia and other solid tumors (typically squamous cancers)
  • Classic triad
    1. Mottled hyperpigmentation of skin that involves the arms, shoulders, neck, and torso
    2. Abnormal nails of fingers and toes
    3. Mucosal leukoplakia
  • Pulmonary fibrosis
  • Prematurely gray hair
  • Cirrhosis of liver
113
Q

Classic triad of what disease

A

Dyskeratosis congenita (DKC)

114
Q

Excessive sweating (hyperhydrosis) is seen in what condition?

A

DKC

115
Q

if have a negative genetic test for FA causing mutation, should you rule out FA?

A

No

116
Q

What is the only curative treatment for DKC?

A

Allogenic stem cell transplant with reduced intensity pre-transplant

117
Q

Patients with DKC have increased sensitivity of radiation and chemo in the ____ while the toxicity is in the ____ ____ in fanconi’s anemia

A

lungs; bone marrow

118
Q

The FLAER test is used in ___ to test GPI-anchored proteins

A

PNH

Hematology Exam 1 (16 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions