Chapter 11- Hemolytic Anemias Flashcards Preview

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Flashcards in Chapter 11- Hemolytic Anemias Deck (32)
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1

Hereditary spherocytosis
Pathologic Cells

RBCs, destroyed via hemolysis

2

Hereditary spherocytosis
Patients

1:2,000 individuals from Northern European Ancestry

3

Hereditary spherocytosis
Unique Features

Autosomal dominant
mutations in RBC membrane proteins, weak RBCs are
removed by spleen, manifests with anemia & splenomegaly, jaundice, RBCs lack central pallor

4

Hereditary spherocytosis
Prognosis

Variable severity, most have minor anemia, possibly complicated by
parvovirus B19 infection (aplastic crisis), splenectomy may be Tx. for severely affected patients

5

Sickle cell anemia
Pathologic Cells

RBCs, destroyed by hemolysis

6

Sickle cell anemia
Patients

8% of African Americans are heterozygotes (trait), 1:600 are homozygotes (have anemia)

7

Sickle cell anemia
Unique Features

Autosomal recessive betaglobin
mutation in the RBC
hemoglobin, makes RBCs
prone to thrombosis,
exacerbated by: blood is
sluggish (spleen, marrow),
dehydration, inflammation

8

Sickle cell anemia
Prognosis

Heterozygotes are asymptomatic
and homozygotes express the
condition, 50% of homozygotes live
past their 50s, death is most
commonly from a stroke or acute
chest syndrome

9

α-thalassemia
Pathologic Cells

RBCs, destroyed
via hemolysis

10

α-thalassemia
Patients

Individuals from:
Mediterranean,
Africa, Southeast
Asia

11

α-thalassemia
Unique Features

Both cause early RBC
hemolysis and damage RBC
precursors in marrow.
α-thalassemia results from α-globin mutation and damages
RBCs due to unpaired β-globin

12

α-thalassemia
Prognosis

lethal in utero (4/4 mutations) or
asymptomatic carrier (1/4
mutations)

13

β-thalassemia (Minor/Major)
Pathologic Cells

RBCs, destroyed
via hemolysis

14

β-thalassemia (Minor/Major)
Patients

Individuals from:
Mediterranean,
Africa, Southeast
Asia

15

β-thalassemia (Minor/Major)
Unique Features

Both cause early RBC
hemolysis and damage RBC precursors in marrow. α-thalassemia results from α-globin mutation and damages
RBCs due to unpaired β-globin
(vice versa for β-thalassemia).

16

β-thalassemia
(Minor vs Major)
Prognosis

β-thal. minor: asymptomatic or
minor anemia, normal lifespan.

β-thal. major: severe, hair-on-end skull, growth restrictions, lethal
during 20s, hemochromatosis
(heart/liver failure) secondary to
repeated blood transfusion

17

Glucose-6-phosphate
dehydrogenase (G6PD)
deficiency
Pathologic Cells

RBCs, destroyed
via hemolysis

18

Glucose-6-phosphate
dehydrogenase (G6PD)
deficiency
Patients

Individuals from
Mediterranean or
the Middle East,
10% of U.S. blacks

19

Glucose-6-phosphate
dehydrogenase (G6PD)
deficiency
Unique Features

X-linked, RBCs are susceptible
to damage because
glutathione can’t be produced
without G6PD, hemolysis is
exacerbated by oxidants:
infections, aspirin, fava beans

20

Glucose-6-phosphate
dehydrogenase (G6PD)
deficiency
Prognosis

Many patients are asymptomatic or
have very mild anemia, many go undiagnosed due to the lack of obvious features, avoidance of precipitating oxidants is
recommended.

21

Paroxysmal nocturnal
hemoglobinuria (PNH)
Pathologic Cells

RBCs, destroyed
via hemolysis

22

Paroxysmal nocturnal
hemoglobinuria (PNH)
Patients

Individuals from
Southeast Asia or
the Far East,
frequently diagnoses
among middle-aged
adults

23

Paroxysmal nocturnal
hemoglobinuria (PNH)
Unique Features

X-linked, PIGA gene mutations in myeloid stem cells, RBCs are susceptible to damage from complement fixation, which is exacerbated at night a pH drops slightly

24

Paroxysmal nocturnal
hemoglobinuria (PNH)
Prognosis

This conditions is very rare, may be managed with antibodies that
inhibit the MAC of the complement system

25

Immunohemolytic anemias (Warm/Cold)
Pathologic Cells

RBCs, destroyed
via hemolysis

26

Immunohemolytic anemias (Warm/Cold)
Patients

Individuals with leukemia/lymphoma
and other or other
autoimmune disorders: SLE, RA, systemic sclerosis, or inflammatory bowel disease (Crohn, U.C.)

27

Immunohemolytic anemias (Warm vs Cold)
Unique Features

Opsonization of antibodies against one’s own RBCs, commonly results is removal of
RBCs in the spleen,
complement activation is also involved to a lesser degree
a) Warm: IgG, rarely IgA
b) Cold: IgM

28

Immunohemolytic anemias (Warm/Cold)
Prognosis

Mild-moderate anemia when the condition is isolated, may be associated with reduced lifespan in patients with serious comorbidities
(e.g. leukemia, SLE)

29

Malaria (tertian malaria)
Pathologic Cells

RBCs, destroyed
via hemolysis

30

Malaria (tertian malaria)
Patients

Anyone infected
with malaria:
Southeast Asia, Africa