Hypoproliferative Anemias Flashcards
(99 cards)
1
Q
reticulocyte index for hypo proliferative anemias
A
- below 2%
2
Q
absolute retic count for hypoproliferative anemias
A
- below 75,000
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
4
Q
most common causes of megaloblastic anemias
A
- B12
- folate deficiency
5
Q
typical presenting feature of megaloblastic anemia
A
- anemia
6
Q
hematologic presentation of megaloblastic anemia
A
- elevated MCV and RDW
7
Q
peripheral smear of megaloblastic anemia shows
A
- hypersegmented PMNs (neutrophils)
8
Q
non-hematologic presentation of megaloblastic anemia
A
- beefy red smooth tongue
- neuro/psychiatric features
9
Q
beefy red smooth tongue seen with which deficiencies
A
- B12
- folate
10
Q
neuro/psychiatric features seen with which deficiencies
A
- B12 only
11
Q
if you are folate deficient, what precursor will build up?
A
- homocysteine
12
Q
if you are B12 deficient, what precursor will build up
A
- homocysteine
- methylmalonic acid
13
Q
homocysteine important for
A
- DNA synthesis
14
Q
methylmalonic acid important for
A
- myelin synthesis
15
Q
homocysteine path
A
- homocysteine
- methionine
- DNA synthesis
16
Q
methylmalonic acid path
A
- methylmalonic acid
- succinic acid
- myelin synthesis
17
Q
how does B12 get into the body
A
- diet
- animal products
18
Q
parietal cells in stomach secrete
A
- intrinsic factor
19
Q
what does B12 bind to
A
- salivary R protein in acidic environment
20
Q
role of pancreatic enzymes
A
- degrade R protein
- frees B12
21
Q
B12-IF complex taken up by cells in
A
- distal ileum
22
Q
B12 carried in blood by ____ to ____
A
- transcobalamin
- tissues
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
24
Q
inadequate dietary intake - B12 deficiency
A
- vegan diet
- no animal products
25
inadequate absorption - B12 deficiency
- lack of gastric acids
- destruction or removal of parietal cells
- gastric bypass
26
destruction/removal of parietal cells by
- pernicious anemia
| - gastrectomy
27
reduced B12 absorption in the ileum
- Crohn's disease
- Sprue
- Metformin
28
Sprue
- celiac
| - tropical
29
competition for B12
- fish tapeworm,
| - bacterial overgrowth
30
what inactivates cobalamin
- nitrous oxide
31
neurologic changes of B12 deficiency
- loss of vibration and position sense - subacute degeneration of the spinal cord
- dementia "megaloblastic mania"
32
laboratory findings of B12 deficiency
- macrocytosis
- hypersegements PMNs
- pancytopenia
33
lab findings with severe B12 deficiency
- elevated bilirubin and LDH
| - due to intramedullary hemolysis
34
diagnosing B12 deficiency
- low B12 level
- elevated homocysteine
- elevated methylmalonic acid
35
how long does B12 deficiency take to develop
- years
36
treat B12 deficiency
- treat underlying cause
| - IM injection of B12 lifelong
37
folic acid is absorbed where
- through the small intestine
38
folate is found in what food products
- uncooked leafy green vegetables
| - bread, cereal products
39
enterohepatic circulation important for
- folate absorption
40
biliary drainage causes
- profound folate deficiency that occurs within hours
41
extra folate should be given to these people
- patients with hemolytic anemias and psoriasis
- women contemplating pregnancy
- pregnant/lactating women
42
folate stores in liver for how long
- 2-5 months only
43
most common cause of folate deficiency
- malnutrition
44
why do we give folate to women contemplating pregnancy
- prevent neural tube defects
45
definition of aplastic anemia
- pancytopenia in peripheral blood w hypocellularity in marrow
46
pancytopenia
- decreased WBCs, RBCs, and platelets
47
bone marrow biopsy of aplastic anemia shows
- hypocellularity
| - deficiency of hematopoietic stem cells
48
aplastic anemia can also be considered
- an autoimmune condition
49
secondary causes of aplastic anemia
- ionizing radiation
- cytotoxic chemotherapy
- benzene exposure
- viruses
- PNH
50
viruses associated with aplastic anemia
- EBV
| - non Hep A, B, C, D, or E
51
anemia in aplastic anemia symptoms
- weakness
| - fatigue
52
low platelets in aplastic anemia symptoms
- bruising
| - oozing
53
low neutrophil counts in aplastic anemia symptoms
- infection fever
54
is there splenomegaly or adenopathy in aplastic anemia
- no!
55
making diagnosis of aplastic anemia
- rule out other causes of pancytopenia
- think about exposures to drugs, test for viruses
- test for PNH
- bone marrow biopsy
56
rule out other causes of pancytopenia
- B12/folate deficiency
- splenomegaly
- other marrow toxins such as alcohol
- autoimmune conditions such as lupus
57
what to look for in bone marrow biopsy
- degree of cellularity
- rule out cancer, infiltrative disorders
- rule out dysplasia
58
infiltrative disorders
- sarcoidosis
| - fibrosis
59
dysplasia disorders
MDS
60
treatment of aplastic anemia
- supportive care
| - definitive therapy
61
supportive care of aplastic anemia
- transfusions
- antibiotics
- growth factors
62
definitive care of aplastic anemia
- stem cell transplantation
| - T cell directed immunosuppression with antithymocyte globulin (ATG) and cyclosporine
63
stem cell transplantation mostly used in
- younger patients
64
T cell directed immunosuppression with antithymocyte globulin (ATG) and cyclosporine mostly used in
- older patients
| - those without donor match
65
paroxysmal nocturnal hemoglobinuria is what kind of disorder
- clonal hematologic disorder
66
abnormal clone for paroxysmal nocturnal hemoglobinuria is missing which gene
- PIG-A
67
purpose of PIG-A
- makes GPI
68
purpose of GPI
- anchors several proteins to cell surface
69
most important GPI proteins
- CD55
| - CD59
70
function of CD55 and CD59
- inhibit complement on surface of red cells
71
PNH clinical manifestations
- hemolysis at night
- thrombosis
- bone marrow failure
72
hemolysis at night in paroxysmal nocturnal hemoglobinuria leads to
- free hemoglobin lost in urine
| - leads to development of iron deficiency
73
types of thrombosis in paroxysmal nocturnal hemoglobinuria
- arterial and venous
74
think of paroxysmal nocturnal hemoglobinuria when you see
- Budd Chiari syndrome
75
Budd Chiari syndrome
- thrombosis of the hepatic veins
| - leads to hepatic failure
76
PNH leads to predisposition of other diseases such as
- MDS
| - AML
77
intravascular hemolysis leads to
- low nitric levels
| - defect in smooth muscle relaxation
78
defects in smooth muscle relaxation
- esophageal spasm
- erectile dysfunction
- pulmonary hypertension
79
how to treat hemolysis in PNH
- a drug!
| - eculizumab
80
MOA of eculizumab
- targets C5
| - blocks terminal complement activation
81
eculizumab will increase susceptibility to infection with
- Neisseria organisms
82
most common form of inherited aplastic anemia
- Fanconi's anemia
83
Fanconi's anemia genetic linkage
- autosomal recessive
| - X-linked
84
Fanconi's anemia due to mutation in
- 1 of 16 genes for DNA repair
85
clinical manifestations of Fanconi's
- pancytopenia with macrocytic anemia
| - other somatic abnormalities
86
increased incidence of ____ in Fanconi"s
- AML
| - solid tumors
87
Fanconi's usually presents in
- early childhood
88
characteristic congenital mutations of Fanconi's
- hypo-pigmented spots and cafe-au-lait macules
| - abnormalities of thumbs and radii
89
diagnosis of Fanconi's
- culture lymphocytes or fibroblasts with DEB (diepoxybutane)
- look for abnormal chromosomal breakage
90
treatment of Fanconi's
- allogenic transplant
| - not much chemotherapy and radiation
91
Dyskeratosis congenita inherited disorder of
- telomere shortening
| - inherited form of aplastic anemia
92
Dyskeratosis congenita clinical characteristics
- bone marrow failure
| - predisposition to AML and other solid tumors
93
classic triad of Dyskeratosis congenita
- mottled hyperpigmentation of skin involving arms, shoulders, neck, torso
- abnormal nails of fingers and toes
- mucosal leukoplakia - white lesions
94
additional findings in Dyskeratosis congenita
- pulmonary fibrosis
- prematurely gray hair
- cirrhosis of liver
95
treatment of Dyskeratosis congenita
- allogenic transplant with not much chemotherapy and radiation
96
differential diagnosis of hypoproliferative anemias
- acute blood loss or destruction
- nutritional anemia
- bone marrow depression
- defective RBC production
- destruction of erythroid precursors
- replacement of normal marrow
BAD DNR
97
nutrients necessary for normal erythropoesis
- iron
- B12
- folic acid
98
defective RBC production by
- kidney disease
| - myelodysplasia
99
destruction of marrow erythroid precursors by
- aplastic anemia
| - parvovirus infection
