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Year 2 Heme lymph > RBC I, Anemias of diminished Erythropoiesis Putthoff > Flashcards

RBC I, Anemias of diminished Erythropoiesis Putthoff Flashcards

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
Q

Ileal resection leads to what

A

megaloblastic anemia form def B12

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26
Q

patient has Hx of eating raw fish and is presenting with megaloblastic anemia

A

tapeworm competes for B12

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27
Q

What are some metabolic states that have increased demand B12

A

pregnancy, hyperthyroidism, disseminated cancer, chronic infection

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28
Q

patient has shiny beefy tongue, sign of what

A

megaloblastic anemia

atrophic glossitis

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29
Q

what are the CNS morphological changes in pernicious anemia

A

demyelination of dorsal and lateral spinal tracts

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30
Q

pernicious anemia patients are at increased risk in developing what

A

gastric carcinoma

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31
Q

what is folic acid needed for

A

purien synthesis, conversion of homocystein to methionine and dTMP synthesis (needed for DNA synthesis)

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32
Q

Folic-acid rich foods

A

green vegetables

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33
Q

What are the 3 major causes of folic acid deficiency

A

decreased intake
icnreased requirements
impaired utilization

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34
Q

In the US what population is more prone to Fe deficiency

A

adolescent girls, women of child bearing age and toddlers

alcoholics

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35
Q

sprue can lead to what

A

Fe deficiency microcytic anemia

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36
Q

what drugs can lead to folic acid deficiency

A

anticonvulsant phenytoin and oral contraceptives that affect absorption

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37
Q

what are folic acid antagonists

A

methotrexate

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38
Q

how can you differentiate B12 def from folic acid

A

look at folate levels

also methymalonate will be normal in folic acid.

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39
Q

where is Fe from in normal diet

A

heme from animal products and inorganic iron in vegetables

40
Q

80% functional Fe found where

A

in hemoglobin and myoglobin

41
Q

Major sites of Fe storage

A

liver and mononuclear phagocytes

42
Q

how do you measure storage pool of Fe

A

plasma ferritin levels

43
Q

Iron absorption is regulated how

A

hepcidin made in liver

44
Q

where does absorption of Fe occur

A

duodenum proximally

45
Q

What disorders have increased hepcidin levels

A

anemia of chronic disease

familial diseases with inability to absorb iron

46
Q

when do you see low hepcidin levels

A

primary and secondary hemochromatosis

47
Q

most common cause of Fe deficiency in the western worl

A

chronic blood loss

48
Q

patient has microcytic anemia and mutation in TMPRSS6

will they respond to Fe therapy

A

no because have high levels hepcidin

49
Q

how come primary and secondary hemochromatosis ahve low levels hepcidin

A

because ineffective erythropoeisis somehow suppresses hepcidin production

50
Q

melena in anemic patient is indicative of what

A

GI bleed somwhere

chronic anemia

51
Q

what is morphology of Fe deficiency anemia

A

microcytic hypochromic anemia with poikilocytosis

52
Q

Causes of Fe deficiency

A

dietary lack, impaired absorption, increased requirement or chronic blood loss

53
Q

what increases absorption of inorganic iron

A

ascorbic acid, citric acid, amino acids and sugars in diet

and inhibited by tannates (found in tea)

54
Q

criteria for Dx Fe deficiency anemia

A

Hct and Hb decreased
low serum Fe and ferritin
High TIBC
low serum hepcidin

55
Q

how can gastrectomy lead to Fe deficiency

A

impairs acidity in proximal duodenum

56
Q

good way to Dx Fe deficiency based on morphology

A

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
Q

what type of physical changes do you get with Fe deficiency

A

kiolonychia, alopecia, atrophic changes in tongue, gastric mcosa and intestinal malabsorption
PICA (eating dirt)

58
Q

Triad plummer vinson syndrome

A

esophageal webs with microcytic hypochromic anemia and atrophic glossitis

59
Q

Most common type anemia in hospitalized patients in the US

A

anemia of chronic disease

impaired RBC production assoc with chronic disease

60
Q

3 major categories of diseases that cause chronic anemia

A

chronic infection
chronic autoimmune disorders
Neoplasms: bronchogenic carcinoma, hodgkin lymphoma

61
Q

what happens in anemia of chronic disease

A

persistent inflammation and cytokines and medioatros suppress erythropoiesis
stimulate hepcidin
low WPO levels

62
Q

Dx anemia of chronic disease based on CBC

A

serum ferritin is high

TIBC low!!

63
Q

Which inflammatory mediator is most assoc with anemia of chronic disease because it stimulates hepcidin

A

IL-6

64
Q

Tx anemia of chronic disease

A

Tx underlying disease

65
Q

Aplastic anemia is what

A

syndrome of chronic primary hematopoietic failure and pancytopenia

66
Q

what are major causes of aplastic anemia

A

acquired, chemical agents, physical agents, inherited

67
Q

persistent marrow aplasia can appear after what viral infections

A

non-A non-B non-C non-G type viral hepatitis

68
Q

What is Fanconi anemia

A

rare autosomal recessive
defects in complex needed for DNA repair
accompanied by congenital anomalies (hypoplasia)

69
Q

Adult onset inherited aplastic anemia is assoc with what

A

defects in telomerases or just abnormally short telomeres

70
Q

What are types of acquired aplastic anemias

A

idiopathic, stem cell defects, immune mediated

71
Q

what type of chemical agents have dose related causes of aplastic anemia

A 
alkyating agents
antimetabolites
benzene
chloramphenicol
Inorganic arsenicals
72
Q

What chemical agents are idiosyncratic in causing aplastic anemia

A 
chloramphenicol
phenylbutazone
organic arsenicals
methylphenylethylhydantoin
carbamazepine
penicillamine
gold salts
73
Q

Majority aplastic anemia are considered what cause

A

idiopathic

74
Q

how to Dx aplastic anemia

A

BM biopsy

75
Q

Wat physical agents can cause aplastic anemia

A

irradiation
viral infections
CMV, EBC Herpes Zoster

76
Q

what 2 major mech are thought to be involved with aplastic anemia

A

intrinsic stem cell abnormality

extrinsic immune mediated suppression of marrow precursors

77
Q

what can aplastic anemia transofrm into

A

myeloid neoplasia like myelodysplasia or AML

78
Q

what does BM aspirate look like in aplastic anemia

A

devoid of hematopoietic cells and lots of fat cells and fibrous stroma
scattered lymphocytes and plasma cells

79
Q

Sx aplastic anemia

A

panctyopenia cause weakness, pallor, dyspneam petichae, ecchymosis, minor infections that are persistent
chills, fever and prostration
NOT splenomegaly!!!

80
Q

what do TBC look like in aplastic anemia

A

macrocytic and normochromic

reticulocytopenia!

81
Q

Tx choice for aplastic anemia

A

bone marrow transplantation

82
Q

What is the primary marrow disorder that only afects erythroid progenitors

A

pure red cell aplasia

83
Q

what can cause pure red cell aplasia

A

thymoma, large granular lymphocytic leukemia
certain drugs, autoimmune disorders
parvovirus B19 infection (immunocompromised)

84
Q

Tx of pure red cell aplasia with patient who has thymoma

A

resection = improvement

85
Q

What are myelophthisic anemias

A

space occupying lesions like metastatic breast lung prostate carcinomas that replace normal marrow

86
Q

myelophthisic anemia is a feature of what disorder

A

myeloproliferative disorders

tear drop cells

87
Q

hwo does chronic renal failure lead to anemia

A

diminished syntehsis of EPO by dmaged kidneys

88
Q

How does hepatocellualr liver failure lead to anemia

A

decreased marrow function

89
Q

hypothyroidism is assoc with what type anemia

A

mild n/n anemia

90
Q

What is polycythemia

A

abnormal high RBC and increase Hb

91
Q

what can cause relative polycythemia

A

dehydration, prolonged vomiting diarrhea or diuretics

or stress polycythemia- obese HTN patients

92
Q

what can cause absolute primary polycythemia

A

intrinsic abnormalitiy of hematopoietic precursors

93
Q

what can cause secondary absolute polycythemia

A

RBC progenitors respond to increased EPO

94
Q

most common cause primary polycythemia

A

Polycythemia Vera

myeloproliferative disorder with mutations with EPO independent growth

95
Q

What can cause secondary polycythemia

A

EPO secreting tumors

inherited defects that stabilize HIF-1alpha that stimulates transcription EPO gene

Year 2 Heme lymph (28 decks)

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