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Heme Midterm 1 > Hypoproliferative Anemias > Flashcards

Hypoproliferative Anemias Flashcards

1
Q

Definition and common causes of megaloblastic anemias

A

defect in DNA synthesis leading to characteristic morphology of bone marrow cells

b12 and folate cause

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2
Q

hematologic smear features of megaloblastic anemia

A

MCV elevated as is RDW, have hypersegmented PMNs

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3
Q

What reaction does folate help with in DNA synthesis?

A

homocysteine to methionine

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4
Q

what reactions does B12 help with?

A

homocysteine to methionine
AND
methylmalonic acid to succinic acid

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5
Q

What is important about succinic acid and B12 deficiency?

A

b12 helps make succinic acid which helps myelin synthesis…why B12 deficiency has neurological issues

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6
Q

salivary R protein role in B12 absorption

A

made in salivary glands and binds B12, travels through stomach attached to B12

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7
Q

pancreatic enzymes role in B12 absorption

A

these clip off the salivary R from the B12

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8
Q

parietal cells role in B12 absorption

A

produce intrinsic factor

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9
Q

intrinsic factor role in B12 absorption

A

binds with B12 and this complex is absorbed by cells

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10
Q

where is B12 actually absorbed?

A

in the distal/terminal ileum

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11
Q

How does pernicious anemia cause B12 deficiency?

A

autoimmune response against parietal cells so no IF to attach and help absorb B12

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12
Q

How does gastric bypass cause B12 deficiency?

A

bypassing the parietal cells so not getting IF on the B12

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13
Q

what drug decreased B12 absorption in the ileum?

A

metformin

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14
Q

What two GI disorders can inhibit B12 absorption?

A

celiac and crohns

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15
Q

3 other ways leading to B12 deficiency?

A

pancreatic insufficiency, competition for B12 by tapeworms or bacteria, inactivation of cobalamin by nitrous oxide

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16
Q

What are the neurological changes seen in B12 deficiency?

A

loss of proprioception (falling at night), and loss of vibrational sense

can lead to dementia

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17
Q

in addition to hypersegmented PMNs and macrocytosis, what else can you have in lab findings for B12 deficiency?

A

elevated LDH and bilirubin because of intramedullary hemolysis

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18
Q

what are the 3 core lab values that signal B12 deficiency?

A

low B12
high homocysteine
high methylmalonic acid

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19
Q

how long does it take B12 deficiency to develop?

A

5-10 years…liver has big time stores

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20
Q

how to treat pernicious anemia?

A

intramuscular injection of B12 for life

21
Q

where is folate absorbed? and where do we get folate from?

A

it is absorbed throughout the small intestine and is found in leafy veggies and bread and cereal products

22
Q

what happens if you have a biliary drain?

A

enterohepatic circulation is important for folate…leads to folate deficiency within minutes

23
Q

what is the most common form of folate deficiency?

A

malnutrition

24
Q

when to give extra folate?

A

pregnancy, hemolytic anemia patients

25
Q

Definition of aplastic anemia

A

pancytopenia caused by hypocellularity in bone marrow due to deficiency in hematopoietic stem cells

26
Q

5 common secondary causes of Aplastic Anemia?

A 
ionizing radiation
chemotherapy
benzene exposure (dry cleaners)
viruses (parvovirus, EBV, hepatitis)
paroxysmal nocturnal hemoglobinuria
27
Q

clinical presentation of aplastic anemia

A

anemia leads to weakness and fatigue
low platelets cause bruising and oozing
low neutrophils cause infections

NO SPLENOMEGALY or ADENOPATHY

28
Q

to make a diagnosis of aplastic anemia what 4 things must you rule out?

A

B12folate deficiency
splenomegaly
other marrow toxins
autoimmune conditions like lupus

29
Q

what test do you run for aplastic anemia?

A

test for PNH, viruses and drug exposures

BONE MARROW BIOPSY

30
Q

what is included in supportive care of aplastic anemia?

A

transfusions, antibiotics and growth factors

31
Q

what is the definitive therapy for aplastic anemia?

A

stem cell transplant…

32
Q

if you cannot give stem cell therapy for aplastic anemia, what is the other drug therapy you should give to patients?

A

T cell directed therapy with cyclosporine and antithymocyte globulin

33
Q

paroxysmal nocturnal hemoglobinemia information

A

clonal disorder of hematopoietic stem cells that are acquired

34
Q

what is the missing gene for in PNH?

A

PIG-A enzyme for making glycosyl phosphophatidyl inositol

35
Q

PIG-A makes GPI and GPI is lacking in PNH..what is GPI important for??

A

anchoring proteins that inhibit complement on RBCs

36
Q

what is the most common clinical manifestation of PNH?

A

hemolysis of blood cells at night leading to iron loss and hemoglobin lost in urine

37
Q

what is a vascular risk of PNH?

A

thrombosis in arterial and venous circulation

38
Q

what is the common type of thrombosis in PNH called and what does it affect?

A

Budd Chiari sydrome leading to thrombosis of hepatic veins and liver failure

39
Q

how to treat PNH?

A

replenish iron and treat thrombosis

treat hemolysis with eculizumab

40
Q

eculizumab treatment for PNH

A

targets C5 and blocks complement…need to be careful with neisseria infection

41
Q

Fanconi anemia cause and inheritance pattern

A

mutation in one of many genes dealing with DNA repair

it is X linked or autosomal recessive

42
Q

what are clinical signs of fanconi anemia?

A 
pancytopenia
short stature
cafe au lait spots
hypo pigmented spots
thumb abnormalities 
microcephaly
hypogonadism
DD
43
Q

what test to use to diagnose fanconi anemia?

A

the Diepoxybutane (DEB) test that looks for abnormal chromosome breakage in lymphocytes

44
Q

treatment for fanconi anemia?

A

allogeneic transplant with reduced intensity conditioning

45
Q

dyskeratosis congenita classic triad and other findings

A

hyperpigmentation of neck, arms shoulders
abnormal nails and fingers/toes
mucosal leukoplakia

it is inherited, and causes bone marrow failure

premature grey hair, liver cirrhosis, pulmonary fibrosis

46
Q

dyskeratosis congenita predispositions

A

acute leukemia and solid tumors

47
Q

dyskeratosis congenita cause

A

telomere shortening involving one of 9 genes

48
Q

treatment for dyskeratosis congenita

A

allogeneic transplant with reduced intensity conditioning since have sensitive chromosomes

Heme Midterm 1 (11 decks)

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