Anemia Flashcards
1
Q
Anemia is classified either by …
A
retic count or MCV of red blood cells
2
Q
Anemias caused by these… tend to have low retic count
A
defects in cell pathways and abnormal reproduction of RBCs
3
Q
T or F. Anemia due to loss have decreased retics
A
F! INCREASED reticulocytes because body is trying to compensate by increasing production and also has the necessary components to do so
4
Q
4 causes of anemia
A
- ineffective erythropoiesis
- insufficient erythropoiesis
- excessive blood loss
- hemolysis
5
Q
describe ineffective erythropoiesis
A
- defective erythroid precursor production
- cells die before maturing
- increased stimulation by EPO leading to high RBC production but RBC are defective
- lack of critical molecules
6
Q
anemia with ineffective erythropoiesis
A
- megaloblastic anemia
- thalassemia
- sideroblastic anemia
- IDA
lack of critical molecules
7
Q
describe insufficient erythropoiesis
A
- decrease in # of erythroid precursors in BM so low # of RBCs produced
8
Q
common causes of insufficient erythropoiesis
A
- IDA
- decreased EPO
- autoimmune diseases or infections
- replacement of normal hematopoietic cells with malignant cells
9
Q
stain used for iron studies
A
Prussian blue
10
Q
osmotic fragility test
A
- RBCs in 0.85 saline
- normal patients will reach certain normality before RBCs burst (hypotonic)
- spherocytes = increased fragility; don’t have excess membrane so hypotonic and burst FASTER
- targets = excess membrane = so take longer to lyse; decreased OF
11
Q
these factors can lead to IDA
A
- inadequate intake
- increased need (pregnancy, rapid growth periods, etc.)
- impaired absorption (decreased stomach acid, inflam. bowel disease, gastrectomies)
- chronic blood loss (menstrual bleeds, ulcers, etc.)
12
Q
iron studies in IDA
A
- decreased iron
- decreased ferritin
- increased TIBC
13
Q
platelets in IDA
A
often increased esp. if IDA due to blood loss or in severe anemia; WBC usually normal
14
Q
T or F. In IDA, normoblasts won’t be as blue
A
T! bc poorly hemoglobinized ; may also develop ragged cytoplasm
15
Q
anemia due to sideroblasts
A
anemia results from ineffective production of heme
- protoporphyrin ring = failure to use iron
- or impaired insertion of iron to ring centre
without iron = cannot carry O2
16
Q
cause of sideroblastic anemia
A
- deficiency in ALA or heme synthase
- decreased vit. B6 or interference by drugs
- deficiency in other enzymes necessary for synthesis of heme (congenital)
17
Q
iron studies in sideroblastic anemia
A
- increased serum iron (may have enough, just can’t incorporate it)
- increased ferritin
- incr Tsat
18
Q
PBS findings in sideroblastic anemia
A
- dimorphic
- basophillic (esp. w lead)
- normal indices, RDW increased
19
Q
how is sideroblastic anemia acquired?
A
- hereditary
or acquired - primary = myelodysplastic syndrome (chromosmal damage overtime)
- secondary = more common; due to drugs (which inhibit ALA synthase, etc.)
20
Q
BM findings for sideroblastic anemia
A
BM + Prussian Blue = siderocytes
21
Q
anemia of chronic infection mechanisms
A
cytokines:
- disturb iron metabolism = incr hepcidin and decrease iron absorption and release; increase lactoferrin and serum ferritin
- diminished erythropoiesis = blunted response to EPO
- decreased red cell lifespan = macs clear minimally damaged RBCs quicker bc they’re on high alert
22
Q
RBC indices for ACI
A
all decreased (MCV, MCHC, MCH)
23
Q
BM findings for ACI
A
- M:E ratio normal or slightly increased due to HYPOprolifeation of RBCs
(BM suppressed due to inflammation)
24
Q
iron studies for ACI
A
- serum iron = decreased
- serum ferritin = increased (sequestered in macs)
- TIBC = decreased
- Tsat = decreased
- BM iron = normal to increased due to sequestration
- elevated ESR and CRP bc of inflammation
25
If the anemia is caused by ineffective or insufficient erythropoiesis, there will be a __________in retics and the BM will show a _______ M:E ratio
decreased retics
decreased M:E ratio bc trying to ramp up RBC production
26
root cause of megaloblastic anemia
impaired DNA synthesis
27
without vitamin B12 or folate, we can't make this
thymine, one of the nucleotide bases in DNA
28
vitamin B12 role in NA synthesis
it is the coenzyme necessary for homocysteine to turn into methionine
29
PBS findings fr megaloblastic anemia
- oval macrocytes
- HJ bodies
-
30
BM findings for megaloblastic anemia
- hypersegs
- hypercellular; erythroid hyperplasia
- megakaryocytes decreased lobulation
- N:C asynchrony
- pancytopenia
- increased serum iron
- enlarged precursors
31
clinical presentation megaloblastic anemia
- jaundice
- glossitis
- gastritis
- nausea
- ailments related to GI
32
anemia caused by _________ causes increased homocysteine levels
folate
- thrombosis
- CV disease
33
pernicious anemia
- impaired absorption of B12 due to lack of IF
- autoimmune (parietal cells destroyed), gastric bypass, chronic gastric issues
34
non-megaloblastic anemia
- normal newborn blood
- postsplenectomy
- liver disease
- reticulocytosis
- chronic alcoholism
- bone marrow failure (MDS/AA)
35
aplastic anemia results from...
bone marrow failure and subsequent decreased hematopoietic cells
36
causes of aplastic anemia
- acquired: idiopathic of secondary to toxic agents or viruses
- inherited: Fanconi's, Dyskeratosis congenita Shwachman-Diamond syndrome
37
Damage to bone marrow can cause (4)
- increased destruction of hem. stem cells (drugs, viruses, autoimmunity)
- decreased self-renewal of hematopoietic stem cells
- infiltration with abnormal cells
- inadequate or impaired microenvironment (decreased growth factors, etc.)
38
T or F. The majority of AA cases is inherited
F! acquired mostly - idiopathic
39
Fanconi's
- AA
- aut recessive
- defect in DNA repair
- increased fetal Hb (macros)
- renal probs, absent/hypoplastic thumb
40
Dyskeratosis Congenita
- X-linked or autosomal dominant
- abn skin, nail malformation, oral leukoplakia
- dyskerin protein lacking = inability to assemble ribosomes and maintain telomeres
41
Schwachman-Diamond syndrome
- aut recessive
- SBDS mutation = defect in ribosomal assembly
- affects BM - neuts predominantly so prone to infections
- pancreatic functions affected, skeletal abnormalities, etc.
42
Pure Red Cell Aplasia
- ACQUIRED through exposure to harmful substances
- no ribosomal assembly and function
- leads to Bm failure
43
AA CBC and PBS
- pancytopenia
- neuts and PLTs critically low
- low retics despite increased EPO
- normo/mormo or macrocytossis
- no poly or nRBCs
44
AA BM findings
- hypocellular
- trephine biopsy needed
- iron stores normal to increased
- Tx: BM transplants or continued transfusions
45
RBC indices in hemolytic anemia
usually N; can be increased due to reticulocytosis
46
T or F. Iron stores in hemolytic anemia is decreased
F! it is increased due to recycling!!
47
alpha thalassemia genetics
large deletion that removes one or more alpha genes; chr 16
48
Hemoglobin H
alpha thal; 3 gene deletion; alpha thal major
- does not precipitate right away but is unstable
- when leave the BM = Heinz bodies
- leads to decreased RBC lifespan and EVH
- still not as severe as B thal
49
Hb Barts
complete alpha deletion
excess gamma = incompatible with life
50
Hb S
- sickle cells
- Glu replaced with Val at 6th
- aut co-dom inheritance
- HbS less soluble than HbA
> polymerizes under deoxygenated conditions
> elongate/sickle
> RBCs less deformable
> vasculature occluded; sticky
> deformed RBCs hemolyzed; lifespan 10-20 days
51
Screens for sickle
HbS solubility test: patient sample with alkaline buffer = Hb precipitates out; HbS = insoluble = turbid
Sickling test: blood + alkaline solution to slide = real time sickling observed under mic
52
HbC
- Glu to lysine at 6th of B chain
- also polymerizes under low O2 = but less splenic sequestration and hemolysis, milder than HbS
- crystals (in/extracellular), spheros, targets
53
HbE
- Glu to Lys at 26th
- no clinical symptoms, mild anemia
- problem when co-dom with other Hbinopathies
54
HbM
- predisposition to form methemoglobin (Fe 3+)
- methemoglobin reductase CANNOT correct this oxidation
55
G6Pd deficiency tests
- negative DAT
- blister cells
- IVH indicators (incr LD, bili, hemoglobinuria, decrease hapto) or EVH
- decreased G6PD activity
56
what causes echinocytes in PK deficiency?
- less ATP formed
- Na/K pump fails
- K+ and water leak out
- cells rigid, shrink
57
RBC indices in HE
normal
58
T or F. In HE, the elliptos are made in the BM
F! they form like that overtime as they pass through vasculature
59
T or F. 80-95% of HE are asymptomatic
T!
60
three clinical findings of HS
- jaundice
- anemia
- splenomegaly
** symptoms often appear early in life**
61
screens vs confirmatory testing for HS
- EMA (binds to band 3; HS is decreased fluorescence) and osmotic fragility test
- molecular testing
62
hereditary acanthocytosis
- pts w liver disease may develop HA with acanthocytosis
- excess free cholesterol; mutation in MTP; decreases fluidity in cells
- spleen remodels cells into acanthocytes
- low Hb; normal indices
- EVH
- autosomal recessive
63
CBC for PNH
- low Hb
- decreased WBcs and PLTs
- elevated MCV due to retics
- loss of iron in urine => IDA?
64
PNH
red cells lack GPI-anchored proteins = susceptible to spontaneous lysis by complement; also found in WBCs and PLTs
65
T or F. PNH can lead to BM failure or AA
T
66
tests for PNH
- IVH tests
- flow cytometry for CD55, CD59
67
hemolysis markers
- increased LDH (intravascular)
- increased bilirubin (shows increased turnover of heme rings
- decreased haptoglobin
- decrease methemalbumin (occupied by Hb to transport to liver)
