B3.047 Hypoproliferative Anemia Flashcards

1
Q

what is the best way to differentiate between inadequate and ineffective production from BM?

A

look at a bone marrow aspirate
full- ineffective
empty- inadequate

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2
Q

what are some potential causes of inadequate BM production?

A

bone marrow failure
bone marrow infiltration/ replacement (myelophthisis)
nutritional deficiency
anemia of chronic disease

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3
Q

what are some reasons for bone marrow failure? which is most common?

A

aplastic anemia
pure red cell aplasia
iatrogenic - most common, many immunosuppressive meds

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4
Q

what are some ways that ineffective production in the BM manifests?

A

cells collect in BM and don’t leave
OR
rapid destruction within the BM

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5
Q

what is aplastic anemia?

A

myeloid stem cell disorder

marrow hypoplasia and pancytopenia

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6
Q

aplastic anemia epidemiology

A

incidence - 2-5 cases / 1,000,000 per year

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7
Q

clinical features of aplastic anemia

A

anemia
leukopenia
thrombocytopenia
no splenomegaly

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8
Q

why is aplastic anemia a misnomer?

A

hypoplastic, not completely aplastic

all myeloid cell lines, not just RBCs

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9
Q

pathogenesis of aplastic anemia

A

primary stem cell abnormality
OR
immune mediated stem cell destruction

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10
Q

immunological factors causing aplastic anemia

A

idiopathic

viral infections

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11
Q

environmental factors causing aplastic anemia

A

drugs/chemicals
whole body radiation
viral infections

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12
Q

genetic factors causing aplastic anemia

A

Fanconi
telomerase defects
acquired stem cell defects
potential for acquisition of additional mutations and transformation into AML

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13
Q

aplastic anemia blood path

A

pancytopenia
normochromic, normocytic RBCs, no poikilocytes
reticulocytopenia

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14
Q

aplastic anemia BM path

A

hypocellular
decreases hematopoiesis
increased marrow fat

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15
Q

aplastic anemia treatment

A

supportive (transfusions)
immunosuppression (see if it works to differentiate between immunologic or clonal)
androgens
hematopoietic stem cell transplant

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16
Q

aplastic anemia prognosis

A

1 year without treatment
75% 5 year survival with transplant
50-70% response to immunosuppression

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17
Q

what is pure red cell aplasia

A

disorder or erythroid progenitor cell causing decreased erythropoiesis

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18
Q

epidemiology PRCA

A

very rare

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19
Q

clinical features of PRCA

A

anemia

20
Q

pathogenesis of PRCA

A

thymoma (paraneoplastic syndrome)
viral infection (parvovirus B19)
drugs
autoimmune disorders

21
Q

describe parovirus B19 and its relationship to PRCA

A

small minority of people affected get severe BM suppression
transient drop in Hgb that self recovers
people who have hemolytic anemia (need BM to always work well) can have severe aplastic crisis

22
Q

PRCA blood path

A

normochromic, normocytic anemia
no poikilocytes
reticulocytopenia

23
Q

PRCA bone marrow path

A

markedly decreased erythroid precurors

intranuclear inclusion in erythroblasts in parvovirus B19 infection

24
Q

treatment of PRCA

A

thymectomy
plasmapheresis
androgens
supportive (transfusion)

25
Q

prognosis of PRCA

A

median survival >10 y without treatment
50% spontaneous remission
50% cure rate with thymectomy

26
Q

what is myelophthisic anemia?

A

decreased erythropoiesis due to bone marrow infiltration or replacement

27
Q

clinical features of myelophthisic anemia

A

anemia

features of underlying disease

28
Q

myelophthisic anemia pathogenesis

A

metastatic cancer
fibrosis (primary myelofibrosis or following treatment)
hematopoietic neoplasms

29
Q

myelophthisic anemia blood path

A
normochromic, normocytic anemia
teardrop shaped RBCs
circulating nucleated RBCs
granulocytic left shift
reticulocytopenia
30
Q

myelophthisic anemia BM path

A

replacement of normal elements

31
Q

what is Plummer-Vinson syndrome (Paterson-Brown-Kelly syndrome)

A

iron def anemia
atrophic glossitis
esophageal webs

32
Q

what is anemia of chronic disease associated with?

A

systemic inflammation
chronic infection
autoimmune disorders
cancer

33
Q

pathogenesis of anemia of chronic disease

A

decreased proliferation of RBC precursors
impaired iron utilization
-increased hepcidin due to inflamm mediators like IL-6
-decreased transfer of iron to RBC precursors in BM

34
Q

pathology of anemia of chronic disease

A
microcytic or normocytic anemia
minimal anisocytosis
no increase in reticulocytes
decreased serum iron
normal or increased ferritin
decrease or normal transferrin and soluble transferrin receptor
increase storage iron in BM
35
Q

what is megaloblastic anemia?

A

macrocytic anemia due to def of vitamin B12 or folic acid
impaired DNA synthesis due to defective thymidine synthesis
ineffective hematopoiesis

36
Q

what is vitamin B12 used for?

A

regeneration of folate

37
Q

what is folate used for?

A

synthesis of DNA from thymidine

38
Q

run through the steps of vitamin B12 absorption

A
  1. B12 is bound to proteins in food and enters mouth
  2. gastric acid causes B12 to disassociate from binding protein in stomach
  3. B12 complexes with haptocorrin (made in salivary glands)
  4. proteases from the pancreas separate B12 from haptocorrin in the intestine
  5. B12 complexes with IF in ileum
  6. IF receptors allow B12 to be absorbed
  7. stored in liver
39
Q

why is supplementing B12 into a diet/orally not always effective?

A

there could be a problem with any of the steps in the absorption process
need to five a shot

40
Q

what are some reasons for vitamin B12 def

A
decreased intake- vegan diet
impaired absorption
-IF def
-malabsorption
-ileal resection
-bacterial overgrowth in blind loops
-fish tapeworm infection
41
Q

what are some reasons for folic acid def

A
decreased intake (impaired diet, alcoholism)
impaired absorption
-malabsorption
-intestinal disease
-anticonvulsants, oral contraceptives
increased loss
increased requirement
-pregnancy, infancy, disseminated cancer, markedly increased hematopoiesis
impaired utilization
-folic acid antagonists
42
Q

clinical features of megaloblastic anemia

A

anemia
neurological deficits - with vitamin B12 def only
-demyelinatin of dorsal and lateral spinal tracts
-spastic paraparesis, sensory ataxia, severe parasthesia

43
Q

pathology of megaloblastic anemia

A

macrocytic anemia- macroovalocytes
hypersegmented neutrophils
megaloblastic hematopietic precursors

44
Q

pathology of vitamin B12 def

A
brain and spinal cord demyelination
intrinsic factor antibodies in pernicious anemia
low serum vitamin B12
high serum methylmalonic acid*
high serum homocysteine*
*precursors usually acted on by B12
45
Q

pathology of folic acid def

A

low serum and RBC folate

high serum homocysteine

46
Q

what would be the problem with treating B12 def with folic acid?

A

exacerbate neurological deficits despite improving anemia