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Mercy CLS 6: Hematology > 9) Aplastic and hypoproliferative anemias > Flashcards

9) Aplastic and hypoproliferative anemias Flashcards

1
Q

morphologic and BM characteristics of hypoproliferative/aplastic anemias

A

normocytic normochromic
hypocellular marrow

aplasia —pancytopenia (precursor affected)

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2
Q

parts of aplastic anemia dx (4)

A
  • BM <25% cellularity
  • ANC <0.5
  • platelets <20
  • corrected retic <1% (no compensation)
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3
Q

2 age groups affected by aplastic anemia

A

young (<20) - usually idiopathic
older (>60) - usually secondary

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4
Q

4 general pathophysiologies of aplastic anemia

A
  • immune suppression (autoimmunity)
  • abnormal/deficient HSCs
  • bone marrow matrix defects
  • deficient GFs (not sole cause)
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5
Q

2 general categories of aplastic anemia and causes in both

A

Acquired
Idiopathic
Drugs
Chemicals
Radiation
Infectious agents
Metabolic errors
Paroxysmal nocturnal hemoglobinuria

Constitutional/congenital
Fanconi’s anemia
Familial aplastic anemia

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6
Q

chloramphenicol
phenobutazone

A

acquired aplastic anemia

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7
Q

benzene derivatives (inhibit DNA synth)

A

acquired aplastic anemia

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8
Q

this treatment does not work for radiation-related aplasic anemia

why?

A

BM transplant

the anemia is due to a matrix change in the BM

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9
Q

infectious agents that can cause aplastic anemia

A

TB
EBV
CMV
HIV
parvovirus (limited to RBCs)
hepatitis (not known viruses)

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10
Q

infection-related aplastic anemia that is often fatal

A

hepatitis

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11
Q

metabolic errors that lead to accumulation of ketones and glycine can cause…

A

aplastic anemia

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12
Q

explain PNH

A

membrane abnormality
lipid that protects cells from complement lysis is absent
cells lyse at night when pH in kidney drops

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13
Q

autosomal recessive, slowly progressive aplastic anemia

A

Fanconi’s anemia

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14
Q

Fanconi’s anemia s/s

A
  • bone dysplasia
  • renal abnormalities
  • organ malformations
  • mental retardation
  • dwarfism
  • microcephaly
  • hypogonadism
  • skin hyperpigmentation
  • thumb abnormalities
  • strabismus
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15
Q

Fanconi’s effects on blood cells

A
  • platelets fall first, and get critically low
  • decrease in granulocytes
  • neutrophils get large pink granules
  • macrocytosis occurs before anemia
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16
Q

Fanconi’s therapies

A
  • Androgen tx — reverse pancytopenia for several years
  • BM transplant
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17
Q

Fanconi’s median survival age

A

30

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18
Q

aplastic anemia
specific inheritance pattern uknown

A

familial aplastic anemia

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19
Q

clinical findings of aplastic anemia

A
  • insidious, gradual progression
  • bleeding, anemia, infection
  • usually no organomegaly
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20
Q

PB findings in aplastic anemia

A
  • pancytopenia
  • normocytic, normochromic
  • corrected retic <1%, no compensation
  • platelets decrease first, then neutrophils, then WBCs
  • relative lymphocytosis due to decreased neutros
  • neutrophil granules that are large and stain dark red
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21
Q

BM findings in aplastic anemia

A
  • hypocellular, >70% fat —dry tap
  • macrophages with Fe, but no siderocytes
22
Q

dry tap

A

aplastic anemia

23
Q

fetal characteristics of RBCs (Hgb F, i antigens)

A

aplastic anemia
Diamond-Blackfan syndrome

24
Q

iron and EPO in aplastic anemia

A

EPO ↑
serum Fe ↑
% sat ↑

25
Q

cure for aplastic anemia

A

BM transplant

26
Q

survival rate for aplastic anemia

A

2 years for 60-90%

27
Q

3 other disorders important to differentially diagnose from aplastic anemia

A
  • myelophthisic anemia
  • myelodysplastic syndrome (MDS)
  • congenital dyserythropoietic anemia (CDA)
28
Q

characteristics of myelophthisic anemia

A
  • BM “crowded out” by fibrotic, granulomatous or neoplastic cells
  • tear drops
  • giant platelets
  • poik
  • nRBCs and left shift (leukoerythroblastosis)
29
Q

characteristics of MDS

A
  • “preleukemia”, tends to end in leukemia
  • cytopenia with ↓ retic
  • BM is normocellular or hypercellular, with dyshematopoiesis (weird BM cells) —cells lyse before release into PB
30
Q

characteristics of CDA

A
  • abnormal and ineffective erythropoiesis
  • rare, familial, refractory anemia
  • BM is normocellular or hypocellular
  • pancytopenia
  • myeloblasts and promyelocytes
  • RBC precursors multinucleated
  • macrocytic anemia
31
Q

3 types of pure red cell aplasia

A
  • acquired acute PRC aplasia
  • acquired chronic PRC aplasia
  • diamond-blackfan syndrome
32
Q

general s/s of pure red cell aplasia

A
  • ↓ RBC, Hct, Hgb
  • Corrected retic <1%
  • ↑M:E ratio in BM
  • ↑EPO
33
Q

parvovirus B19, Fifth disease
hepatitis (poor prognosis)
other infections

A

acquired acute pure red cell aplasia

34
Q

worst prognosis for acquired acute PRC aplasia

A

with hepatitis

35
Q

transient erythroblastopenia of childhood is a type of…

A

acquired acute PRC aplasia

36
Q

autoimmune disorders
thymoma
middle age

A

acquired chronic PRC aplasia

37
Q

rare, congenital, progressive RBC aplasia

A

diamond-blackfan syndrome

38
Q

characteristics of diamond-blackfan

A
  • very young children
  • severe anemia
  • ↑i Ag, ↑ Hgb F
  • 100% sat, ↑ serum Fe, ↑ ferritin
  • ↑EPO
  • minor abnormalities of head and arms
39
Q

distinguish Diamond-blackfan from TEC

A

↑i Ag, ↑ Hgb F found only in diamond-blackfan

40
Q

most common cause of non-aplastic, non-pure RBC hypoproliferative anemias

A

↓ EPO

41
Q

can result in normo/normo, megaloblastic, or micro/hypo anemia

how?

A

renal disease

↓ EPO —normo/normo
dialysis ↓ folate —megaloblastic
dialysis ↓ iron —micro/hypo IDA

42
Q

high BUN and creatinine often causes…

A

burr cells

43
Q

how can endocrine abnormalities cause a hypoproliferative normo/normo anemia?

A

hypothyroidism/hypopituitarism

↓ O2 demand in tissues
indirectly ↓ EPO

44
Q

anemia occuring in patients with chronic infection, inflammation, or neoplasms

A

anemia of chronic disease/inflammation

45
Q

iron in ACD patients

A

↓ serum Fe
normal or ↑ Fe stores
↑ ferritin
↓ TIBC

46
Q

second most common anemia

A

ACD

47
Q

3 mechanisms of ACD

A
  1. cytokines released by inflammatory response inhibit EPO, and cell response to EPO
  2. hepcidin blocks Fe mobilization, so macrophages are unable to give it up for use
  3. early phagocytosis of good RBCs leads to shortened lifespan (extracorpuscular factors)
48
Q

PB findings in ACD

A
  • mild anemia (Hgb ≥9)
  • normo/normo, but eventually micro/hypo
  • RPI <2
  • normal WBC, normal platelets
49
Q

BM findings in ACD

not necessary for dx

A
  • ↑ M:E ratio
  • ↑ hemosiderin in macrophages
50
Q

malignancy can cause a ————– anemia when it infiltrates the BM

A

myelophthisic

51
Q

2 main types of anemia of liver disease and characteristics

A
  • chronic liver disease (target cells, acanthocytes from altered lipid metabolism)
  • alcoholism (megaloblastic, hemolytic anemia)

Mercy CLS 6: Hematology (25 decks)

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