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Hematology and Oncology ERIN > Microcytic anemias > Flashcards

Microcytic anemias Flashcards

1
Q

What is microcytosis?

A

An extra division of RBC progenitor cells which occurs to maintain haemoglobin concentration

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2
Q

What is hemoglobin made of?

A

Heme and globin

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3
Q

What is heme made of?

A

iron and protoporphyrin

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4
Q

Causes of microcytic anemias?

A

Iron deficiency anemia
anemia of chronic disease
sideroblastic anemia
thalassemia

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5
Q

stages of iron deficiency

A

Storage depleted - decreased ferritin, increased TIBC
Serum iron depleted - decreased serum iron, decreased saturation
Normocytic anemia - bone marrow makes fewer but normal-sized RBCs
Microcytic, hypochromic anemia –bone marrow makes smaller and fewer RBCs

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6
Q

Clinical features of iron deficiency

A

Anemia, koilonychia, pica

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7
Q

Lab findings in iron deficiency anemia

A 
Decreased ferritin
Decreased serum iron
Decreased saturaiton
Increased TIBC
Increased free erythrocyte protoporphyrin
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8
Q

plummer-vinson triad

A

iron deficiency anemia, esophageal webs, atrophic glossitis

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9
Q

Pathophys in anemia of chronic disease

A

Chonric inflammation leads to production of hepcidin, which sequester iron in storage sites by limiting transfers of iron from macrophages to erythroid precursors and suppresses EPO
This is done to prevent bacteria from accessing iron

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10
Q

Lab findings in anemia of chronic disease

A

increased ferritin, decreased TIBC, decreased serum iron, decreased iron saturation
increased free erythrocyte protoporphyrin

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11
Q

protoporphyrin synthesis pathway

A

succinyl coa –> ALA via ALAS
ALA –> porphobilinogen via ALAD
porphobiliniogen ———> protoporphyrin
Protoporphyrin + Iron –> Heme via ferrochetalase

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12
Q

pathophys of sideroblastic anemia

A

Congenital defect involve ALAS or acquired. Results in protoporphyrin deficiency and buildup of Iron in mitochondria
Mitochondria form a ring around nucleus of erythroid precursors

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13
Q

What is the cause of sideroblastic anemia?

A

Deficiency of protoporphyrin

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14
Q

Causes of acquired sideroblastic anaemia

A

Alcoholism - mitochondrial poison
Lead poisoning - inhibits ALAD and ferrochetalase
Vitamin B6 def - needed for ALAS

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15
Q

Lab findings in sideroblastic anemia

A

Increased ferritin
decreased TIBC
increased serum iron
increased iron saturation

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16
Q

HbF

A

alpha 2 gamma 2

17
Q

HbA

A

alpha 2 beta 2

18
Q

HbA2

A

alpha 2 delta 2

19
Q

cause of alpha thalassemia

A

gene DELETION

20
Q

cause of beta-thalassemia

A

gene MUTATION

21
Q

What chromosome has the alpha genes?

A

16

22
Q

What chromosome has the beta genes?

A

11

23
Q

Presentation if one alpha gene is deleted?

A

asymptomatic

24
Q

Presentation if two alpha genes are deleted?

A

mild anemia with increased RBC count

25
Q

who more commonly has cis deletion of alpha gene?

A

asian persons

26
Q

who more commonly has trans delation of alpha gene?

A

african descent

27
Q

Presentation if three alpha genes are deleted?

A

severe anemia

Beta chains form tetramers (HbH) that damage RBCs

28
Q

Presentation if four alpha genes are deleted?

A

Hydrops fetalis in utero – gamma chains form tetramers that damage RBCs

29
Q

HbH

A

tetramers of beta chains

30
Q

Hb Barts

A

tetramers of gamma chains

31
Q

beta thalassemia minor presentation

A

mild disease usually asymptomatic with increased RBC count

32
Q

what will hemoglobin electrophoresis show in Beta thalassemia minor?

A

increased HbA2 and increased HbF

33
Q

explain the pathophys of Beta-thalassemia major

A

Most severe form of disease.
Unpaired alpha chains precipitate and damage RBC membranes causing ineffective erythropoiesis and intravascular hemolysis

34
Q

Presentation of beta-thalassemia major

A

Massive erythroid hyperplasia –> crewcut appearance from hematopoiesis in the skull, chipmunk facies, hepatosplenomegaly, risk of aplastic crisis from parvovirus

Hematology and Oncology ERIN (12 decks)

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