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Heme boards > Anemia > Flashcards

Anemia Flashcards

1
Q

Soluble transferrin receptor level in IDA

A

HIGH

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2
Q

IV iron recommended for pregnancy

A

Ferric gluconate (ferrlecit)
Iron sucrose

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3
Q

AICD lab profile

A

Low serum iron
low transferrin
low TSAT (<20% in 80% of cases, normal range 20-45% (**can be pseudo-normal if very low transferrin concentration (<200))
ferritin normal or increased

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4
Q

AICD mechanism

A
  • ## increase in hepcidin (and other cytokines) causes iron to be retained within cells of the reticuloendothelial system (inhibits release of iron from macropa
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5
Q

Mechanism of anemia of pregnancy

A
  • increase in plasma volume (dilutional)
  • can be folate and iron and b12 deficiency
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6
Q

What is megaloblastic anemia?

A
  • form of macrocytic anemia in which nucleic acid metabolism is impaired, leading to reduced efficiency of cell division and nuclear-cytoplasmic dyssynchrony (eg B12, folate deficiency)
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7
Q

Drugs associated with macrocytic anemia

A
  • MTX
  • azathioprine
  • hydrea
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8
Q

Smear findings with B12 deficiency

A
  • hypersegmented neutrophils (see photo online, will prob show photo)
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9
Q

clinical features of copper deficiency 2) clinical context in which it is seen

A

macrocytic anemia + older people with gastric surgeries (bypass) OR denture cream leading to zinc excess OR excessive zinc use
*bone marrow biopsy looking like MDS

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10
Q

Max hgb goal for anemia of CKD

A

12

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11
Q

Site of folate absorption

A

Duodenum or proximal jejenum

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12
Q

HS inheritance

A

autosomal dominant

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13
Q

HS 1) pathophys 2) most common mutation

A

1) affected proteins involved in VERTICAL ASSOCIATIONS that link the membrane cytoskeleton to the lipid bilayer
2) ankyrin (anchoring)

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14
Q

HS diagnosis

A
  • now done with genetic testing
  • previously by osmotic fragility test
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15
Q

Spherocyte differential *common board question

A
  • HS
  • immune mediated hemolytic anemia
  • CAD
  • extensive thermal burns
  • snake bites
  • scorpion bites
  • brown recluse spider bites
  • clostridium sepsis
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16
Q

elliptocytosis/ovalocytosis 1) inheritance 2) phenotype 3) pathophys 4) mutation

A
  • autosomal dominant
  • mild, minimal hemolysis
  • affected proteins involved in horizontal associations that link the membrane cytoskeleton to the lipid bilayer
  • spectrin
17
Q

hereditary pyropoikilocytosis 1) mutation 2) presentation 3) smear
*always on boards, love to show smear

A
  • spectrin (also
  • variable hemolysis (worse in early childhood), many asymptomatica
  • rod-shaped elliptocytes (see photo)
18
Q

G6PD 1) clinical features 2) ethnicity

A
  • acute hemolytic episodes after exposure to medication or infection
  • early/prolonged neonatal jaundice
  • Possible family history
  • Mediterranean, middle east, africa, SE asia
19
Q

G6PD diagnosis - how to determine during acute hemolysis

A
  • blister cells on peripheral smear at time of hemolytic event (see photo).
    *G6PD level is falsely elevated during acute hemolysis so always need to screen after crisis has resolved
20
Q

G6PD management

A

supportive, transfusions as needed during hemolytic events

21
Q

Pyruvate kinase deficiency 1)clinical features 2) gene 3) population

A
  • moderate hemolytic anemia at baseline + episodes of worsening hemolysis
  • PKLR
  • Amish
22
Q

Pyruvate kinase deficiency treatment

A
  • mitapivat (PK activator)
23
Q

Pyruvate kinase deficiency response to splenectomy

A
  • mild hgb response
  • BUT retic count markedly increases due to preferential sequestering of reticulocytes in spleen with PK deficiency
24
Q

Buzz words for marrow findings with megaloblastic anemia (anemia from b12 or folate deficiency)

A

Impaired nuclear maturation but abundant cytoplasm due to
large marrow progenitors.
Key term is -
*“nuclear-cytoplasmic dyssynchrony” (large immature appearing nucleus with open chromatin pattern and mature appearing cytoplasm)

25
Q

How does folate supplementation affect B12 deficiecy?

A
  • may correct hematologic findings but will NOT treat neurologic findings
26
Q

Homocysteine level with B12 deficiency

A
  • HIGH
27
Q
A
28
Q

Imerslund-Grasbeck syndrome

A
  • rare autosomal recessive disorder due to B12 malabsorption due to IF/B12 inability to bind to terminal ileum
29
Q

Folate deficiency causes

A

Decreased intake →
Impaired absorption (small bowel disease – crohns, celiac, amyloidosis) →
alcoholism (impairs enzyme used to absorb folate) –>
Increased utilization (pregnancy, hemolysis (SLE), psoriasis)

30
Q

homocysteine and MMA in folate deficiency

A
  • homocysteine high, MMA normal
31
Q

copper deficiency presentation

A
  • cytopenias
  • looks like MDS
  • neuro deficits
32
Q

copper deficiency primary cause

A

history of GI surgery

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