RBC I, Anemias of diminished Erythropoiesis Putthoff Flashcards

(95 cards)

1
Q

what are anemias from underproduction caused by

A

nutritional deficiencies, renal failure and chronic inflammation

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2
Q

What causes megaloblastic anemia

A

impairment of DNA synthesis

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3
Q

Vit B12 and folic acid are needed for what

A

synthesis of thymidine

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4
Q

what do neutrophils look like in megaloblastic anemias

A

larger and with hypersegmentation

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5
Q

Pernicious anemia is caused by what

A

autoimmune gastritis that impairs production of IF

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6
Q

where is IF secreted

A

parietal cells of fundic mucosa

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7
Q

Vit B12 binds to what in stomach

A

haptocorrin

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8
Q

where does Vit B12 join IF

A

jejunum after pancreatic proteases released

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9
Q

Where is B12 absorbed

A

distal ileum

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10
Q

Absence of VitB12 leads to what

A

increased homocysteine and methyl-tetrahydrofolate

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11
Q

What is proximate cause of anemia in B12 deficiency

A

lack of folate

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12
Q

What Sx of B12 deficiency are not resolved by folic acid replacement

A

neurologic complications

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13
Q

What are the neurologic signs of B12 deficiency

A

spastic paraparesis, sensory ataxia, severe parathesias of lower limbs

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14
Q

what population is most affected by pernicious anemia

A

Scandinavian and other Caucasian populations
also blacks hispanics
60 y.o

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15
Q

What Ab are found in patients with pernicious anemia

A

type I Ab that blocks VitB12-IF binding
Type II Ab that prevent IF-B12 complex binding to ileal R
Type III Ab that recognize alpha and beta subunits of gastric H+ pump

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16
Q

Dx pernicious anemia is based on what criteria

A

moderate to severe megaloblastic anemia
leukopenia with hypersegmented granulocytes
low serum B12
elevated serum levels homocystein and methymalonic acid

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17
Q

increased homocystein is assoc with what other condition

A

accelerated athersclerosis

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18
Q

pernicious anemia is asso with what other conditions

A

autoimmune disorders

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19
Q

after parenteral administration of B12 when will Hct increase and reticulocytosis be notable

A

5 days

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20
Q

what are the Dx utility in pernicious anemia

A

autoantibodies

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21
Q

What is postulated to be the primary cause of gastric pathology in pernicious anemia

A

autoreactive T cell resoponse that inflicts gastric injury and triggers formation autoAb

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22
Q

What is acholorhydria and how does it lead to B12 deficiency

A

loss pepsin secretion, so B12 not readily released from foods

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23
Q

how does gastrectomy lead to B12 deficiency

A

IF not available

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24
Q

if pancreas is affected what could happen to B12 levels

A

B12 cannot be released from haptocorrin B12 complex

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25
Ileal resection leads to what
megaloblastic anemia form def B12
26
patient has Hx of eating raw fish and is presenting with megaloblastic anemia
tapeworm competes for B12
27
What are some metabolic states that have increased demand B12
pregnancy, hyperthyroidism, disseminated cancer, chronic infection
28
patient has shiny beefy tongue, sign of what
megaloblastic anemia | atrophic glossitis
29
what are the CNS morphological changes in pernicious anemia
demyelination of dorsal and lateral spinal tracts
30
pernicious anemia patients are at increased risk in developing what
gastric carcinoma
31
what is folic acid needed for
purien synthesis, conversion of homocystein to methionine and dTMP synthesis (needed for DNA synthesis)
32
Folic-acid rich foods
green vegetables
33
What are the 3 major causes of folic acid deficiency
decreased intake icnreased requirements impaired utilization
34
In the US what population is more prone to Fe deficiency
adolescent girls, women of child bearing age and toddlers | alcoholics
35
sprue can lead to what
Fe deficiency microcytic anemia
36
what drugs can lead to folic acid deficiency
anticonvulsant phenytoin and oral contraceptives that affect absorption
37
what are folic acid antagonists
methotrexate
38
how can you differentiate B12 def from folic acid
look at folate levels | also methymalonate will be normal in folic acid.
39
where is Fe from in normal diet
heme from animal products and inorganic iron in vegetables
40
80% functional Fe found where
in hemoglobin and myoglobin
41
Major sites of Fe storage
liver and mononuclear phagocytes
42
how do you measure storage pool of Fe
plasma ferritin levels
43
Iron absorption is regulated how
hepcidin made in liver
44
where does absorption of Fe occur
duodenum proximally
45
What disorders have increased hepcidin levels
anemia of chronic disease | familial diseases with inability to absorb iron
46
when do you see low hepcidin levels
primary and secondary hemochromatosis
47
most common cause of Fe deficiency in the western worl
chronic blood loss
48
patient has microcytic anemia and mutation in TMPRSS6 | will they respond to Fe therapy
no because have high levels hepcidin
49
how come primary and secondary hemochromatosis ahve low levels hepcidin
because ineffective erythropoeisis somehow suppresses hepcidin production
50
melena in anemic patient is indicative of what
GI bleed somwhere | chronic anemia
51
what is morphology of Fe deficiency anemia
microcytic hypochromic anemia with poikilocytosis
52
Causes of Fe deficiency
dietary lack, impaired absorption, increased requirement or chronic blood loss
53
what increases absorption of inorganic iron
ascorbic acid, citric acid, amino acids and sugars in diet | and inhibited by tannates (found in tea)
54
criteria for Dx Fe deficiency anemia
Hct and Hb decreased low serum Fe and ferritin High TIBC low serum hepcidin
55
how can gastrectomy lead to Fe deficiency
impairs acidity in proximal duodenum
56
good way to Dx Fe deficiency based on morphology
no stainable Fe in macrophages in BM with Prussian blue stains increased zone of pallor in center of RBC elongated RBC called pencil cells are characteristic
57
what type of physical changes do you get with Fe deficiency
kiolonychia, alopecia, atrophic changes in tongue, gastric mcosa and intestinal malabsorption PICA (eating dirt)
58
Triad plummer vinson syndrome
esophageal webs with microcytic hypochromic anemia and atrophic glossitis
59
Most common type anemia in hospitalized patients in the US
anemia of chronic disease | impaired RBC production assoc with chronic disease
60
3 major categories of diseases that cause chronic anemia
chronic infection chronic autoimmune disorders Neoplasms: bronchogenic carcinoma, hodgkin lymphoma
61
what happens in anemia of chronic disease
persistent inflammation and cytokines and medioatros suppress erythropoiesis stimulate hepcidin low WPO levels
62
Dx anemia of chronic disease based on CBC
serum ferritin is high | TIBC low!!
63
Which inflammatory mediator is most assoc with anemia of chronic disease because it stimulates hepcidin
IL-6
64
Tx anemia of chronic disease
Tx underlying disease
65
Aplastic anemia is what
syndrome of chronic primary hematopoietic failure and pancytopenia
66
what are major causes of aplastic anemia
acquired, chemical agents, physical agents, inherited
67
persistent marrow aplasia can appear after what viral infections
non-A non-B non-C non-G type viral hepatitis
68
What is Fanconi anemia
rare autosomal recessive defects in complex needed for DNA repair accompanied by congenital anomalies (hypoplasia)
69
Adult onset inherited aplastic anemia is assoc with what
defects in telomerases or just abnormally short telomeres
70
What are types of acquired aplastic anemias
idiopathic, stem cell defects, immune mediated
71
what type of chemical agents have dose related causes of aplastic anemia
``` alkyating agents antimetabolites benzene chloramphenicol Inorganic arsenicals ```
72
What chemical agents are idiosyncratic in causing aplastic anemia
``` chloramphenicol phenylbutazone organic arsenicals methylphenylethylhydantoin carbamazepine penicillamine gold salts ```
73
Majority aplastic anemia are considered what cause
idiopathic
74
how to Dx aplastic anemia
BM biopsy
75
Wat physical agents can cause aplastic anemia
irradiation viral infections CMV, EBC Herpes Zoster
76
what 2 major mech are thought to be involved with aplastic anemia
intrinsic stem cell abnormality | extrinsic immune mediated suppression of marrow precursors
77
what can aplastic anemia transofrm into
myeloid neoplasia like myelodysplasia or AML
78
what does BM aspirate look like in aplastic anemia
devoid of hematopoietic cells and lots of fat cells and fibrous stroma scattered lymphocytes and plasma cells
79
Sx aplastic anemia
panctyopenia cause weakness, pallor, dyspneam petichae, ecchymosis, minor infections that are persistent chills, fever and prostration NOT splenomegaly!!!
80
what do TBC look like in aplastic anemia
macrocytic and normochromic | reticulocytopenia!
81
Tx choice for aplastic anemia
bone marrow transplantation
82
What is the primary marrow disorder that only afects erythroid progenitors
pure red cell aplasia
83
what can cause pure red cell aplasia
thymoma, large granular lymphocytic leukemia certain drugs, autoimmune disorders parvovirus B19 infection (immunocompromised)
84
Tx of pure red cell aplasia with patient who has thymoma
resection = improvement
85
What are myelophthisic anemias
space occupying lesions like metastatic breast lung prostate carcinomas that replace normal marrow
86
myelophthisic anemia is a feature of what disorder
myeloproliferative disorders | tear drop cells
87
hwo does chronic renal failure lead to anemia
diminished syntehsis of EPO by dmaged kidneys
88
How does hepatocellualr liver failure lead to anemia
decreased marrow function
89
hypothyroidism is assoc with what type anemia
mild n/n anemia
90
What is polycythemia
abnormal high RBC and increase Hb
91
what can cause relative polycythemia
dehydration, prolonged vomiting diarrhea or diuretics | or stress polycythemia- obese HTN patients
92
what can cause absolute primary polycythemia
intrinsic abnormalitiy of hematopoietic precursors
93
what can cause secondary absolute polycythemia
RBC progenitors respond to increased EPO
94
most common cause primary polycythemia
Polycythemia Vera | myeloproliferative disorder with mutations with EPO independent growth
95
What can cause secondary polycythemia
EPO secreting tumors | inherited defects that stabilize HIF-1alpha that stimulates transcription EPO gene