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Impaired Production Anemias Flashcards

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1
Q

What is impaired production

A

anemias that are caused by impaired production of RBCs that results in a decreased production

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2
Q

Cause of Iron Deficiency Anemia

A

inadequate stores of Iron for Hgb

  1. decreased intake of iron - vegetarian, infancy, pregnancy
  2. increased need for iron - pregnancy, children
  3. increased loss of iron through blood loss - menstruation , chronic blood loss
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3
Q

Pathogenesis of Iron Deficiency Anemia

A 
develops slowly as various iron compartments become depleted
3 stages:
iron depletion
iron deficient erythroipoiesis
iron deficiency anemia
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4
Q

Stage 1 of IDA - Iron Depletion

A

body’s reserve is sufficient to maintain functions
iron stores are becoming depleted
decrease in serum ferritin
no anemia

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5
Q

Stage 2 of IDA - Iron Deficient Erythropoiesis

A 
exhaustion of the storage pool of iron
results in zinc complexes with protoporphyrin - ZPP
RBCs become slightly microcytic
Hgb will begin to drop
Ferritin is still low
serum iron is low
TIBC of transferrin will rise
% saturation will be low
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6
Q

Stage 3 of IDA - Iron Deficiency Anemia

A 
Developing RBCs are unable to develop normally due to depleted iron stores & diminished iron transport
show anemia symptoms
MICROCYTIC, HYPOCHROMIC
ferritin extremely low
serum iron low
TIBC of transferrin is high
% saturation is low
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7
Q

Symptoms of IDA

A

Pica syndrome - crushed ice
sore tongue (glossitis)
cracks at corner of mouth
spooning of the fingernails

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8
Q

Screening for IDA

A

in the PB : increased RDW & microcytosis
hypochromic/microcytic
Hgb decreases

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9
Q

Diagnostic tests for IDA

A 
ferritin decreased 
serum iron decreased
TIBC increased
% saturation decreased
panel test for iron
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10
Q

Specialized testing for IDA

A

ZPP will be increased

BM will display iron deficiency - reveal an absence of hemosiderin

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11
Q

Anemia of Chronic Disease Pathogenesis

A 
iron is abundant in the body
serum iron is low (sideropenia)
unable to recycle iron to BM normoblast
Hepcidin is released in inflammation
decreased EPO by cytokines
suppression of RBC production by cytokines
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12
Q

Hepcidin in ACD

A

causes sideropenia
produced in response to inflammatory cytokines
blocks iron uptake in the duodenum & blocks the release of iron from Macrophages

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13
Q

Lab Diagnosis of ACD

A 
mild anemia (Hgb 9-11)
Normochromic/ Normocytic can be hypochromic/microcytic in long standing cases
RDW -normal
Retics - normal or decreased
Ferritin - normal to increased
serum iron - decreased
TIBC -decreased
% saturation - decreased 
ZPP - increased
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14
Q

Sideroblastic Anemia

A

anemia in which heme synthesis is impaired
iron is abundant
results from mutation that affects enzymatic steps of heme synthesis

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15
Q

2 Groups of Sideroblastic Anemias

A

inherited & acquired

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16
Q

Hereditary Sideroblastic Anemia

A

X-linked sideroblastic anemia
females are carriers & males are affected
very uncommon anemia

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17
Q

Acquired Sideroblastic Anemia

A

unknown cause or secondary to an underlying disease
most common causes :
1. heavy metals (lead)
2. alcohol

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18
Q

Lead Poisoning & children

A

eating lead based paint chips

leads to hyperactivity, low IQ, concentration disorders, hearing loss, impaired grown & development

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19
Q

Lead Poisoning and Sideroblastic Anemia

A

once ingested lead passes through the blood to the BM & accumulates in the mitochondria of erythroblasts & inhibits cellular enzymes involved in heme synthesis

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20
Q

Lab Diagnosis of Siderblastic Anemia

A

Normochromic Normocytic
can be microcytic/hypochromic with chronic exposure
can have a duel population of hypo & normo cells
Basophilic stippling
Pappenheimer bodies or siderotic granules (iron deposits)
poikilocytosis
in BM - ringed sideroblasts (excess of iron in mitochondria)

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21
Q

Porphyrias Definition

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A

group of disorders that result in the build up of porphyrins
diseases characterized by impaired production of heme ( if any enzymatic step in heme synthesis is blocked)
RARE ANEMIAS

22
Q

Symptoms of Porphyria Anemias

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A

vary depending on type
abdominal pain
light sensitivity - rashes
problems with nervous system & muscles

23
Q

Types of Porphyrias

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A 
2 types:
Congenital erythropoietic porphyria (CEP)
Erythropoietic protophyria (EPP)
24
Q

Congenital Erythropoietic porphyria (CEP) Characteristics

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A

autosomal recessive disease
missing enzyme: uroporphyrinogen III cosynthetase
creates a build up of : Uroporphyrin I & coproporphyrin I
EXTREME photosensitivity
RBC lifespan : 18 days

25
Erythropoietic protophyria(EPP) Characteristics
``` autosomal dominant disease missing enzyme: ferrocheletase build up of : protoporphyrin less severe photosensitivity no hemolytic anemia ```
26
Causes of Megalobastic Anemia
1. deficiency of vitamin B12 (cobalamin) | 2. deficiency of folic acid (folate)
27
Pathogenesis of Megaloblastic Anemia
v B12 & folic acid are necessary as coenzymes for nucleic acid synthesis = DNA synthesis will be impaired results in the demyelinization of nerves
28
Causes of Vitamin B12 Deficiency
1. dietary deficiency - vegans etc 2. increased need during pregnancy/lactation & growth 3. impaired absorption of v B12
29
Vitamin B12 Absorption
V. B12 is bound to haptocorrin & is released from haptocorrin by trypsin in the small intestine V. B12 then binds to Intrinsic factor (IF) produced by gastric cells
30
Ways of Impaired absorption of Vitamin B12
1. failure to separate VB12 from haptocorrin 2. Lack of IF - most common cause 3. malabsorption caused by conditions (inflammatory bowel disease) 4. competition for available VB12- tapeworm etc
31
Symptoms of Vitamin B12 deficiency
``` memory loss numbness loss of balance 'megaloblastic madness' all symptoms derived from demylination of the nerves ```
32
Causes of Folic Acid Deficiency
1. dietary deficiency 2. increased need w/ pregnancy & growth 3. impaired absorption -usually due to intestinal disease 4. impaired utilization due to drugs - anti-epileptic drugs 5. excessive loss w/ renal dialysis
33
Symptoms of Folic Acid Deficiency
depression/ psychosis fetus can be effected - spina bifida all neurological symptoms in addition to generic anemia symptoms
34
Wintrobe Classification of Megaloblastic Anemia
``` Normochromic, Macrocytic anemia MCV > 120 fL pancytopenia elevated RDW oval macrocytes HYPERSEGMENTED NEUTROPHILS poikilocytosis low retic count ```
35
Bone Marrow Diagnosis for Megaloblastic Anemias
confirm megaloblastic appearance of cells nuclear-cytoplasm asynchrony = nucleus appears younger than expected in relation to the cytoplasm WBCs appear larger/giant
36
Chemistry tests for Megalobastic Anemias
Vitamin B12 levels serum folate or red cell folate intermediate tests: methylmalonic acid (MMA) - normal in folate def. & increased in vB12 def. homocysteine levels - elevated in both vB12 & folate def.
37
Pernicious Anemia (PA)
form of megaloblastic anemia - most common form due to vitamin B12 deficiency- autoimmune destruction of IF secreting gastric cells produce antibodies to IF that can be assayed
38
Determining the Cause of Megaloblastic Anemia
1. assay the amount of folate or vitamin B12 in the body | 2. if vitamin B12 is decreased, determine whether if it is due to IF deficiency (schilling test/ assays)
39
Schilling Test Principle
once absorbed, a portion of any oral dose of v. B12 hat is not used by the body will be excreted in the urine if vB12 is nto detected in the urine then malabsorption is confirmed 2 phase test
40
Why is the Schilling Test not used any longer
because of the use of radio-labeled vitamin B12
41
Macrocytic Anemia w/o Megaloblastosis
macrocytes (not as pronounced) no hyper-segmented neutrophils WBC & Plts are normal other symptoms of megaloblastosis are not present
42
Two most common causes of Macrocytic Anemia w/o Megaloblastosis
Alcoholism - usually no alcohol | Liver Disease- anemia is present
43
Causes of Aplastic Anemia
acquired : Fanconi's Anemia & Dyskeratosis Congenita | inherited - ~ 70% of cases have unknown causes secondary to toxic chemicals (benzene), radiation, drugs, etc
44
Pathogenesis of Aplastic Anemia
Bone marrow failure due to depletion of BM & replacement of BM with fat results in pancytopenia
45
Lab Diagnosis of Aplastic Anemia
normochromic, normocytic anemia panctyopenia normal RDW
46
Causes of Myelophthisic Anemia
``` Tumors & malignant diseases (prostate, breast, stomach cancer) nonmalignant diseases (TB) ```
47
Pathogensis of Myelophthisic Anemia
normal BM is replaced with or infiltrated by fibrotic or neoplastic (cancerous) cells and leads to BM failure
48
Lab Diagnosis of Myelophthisic Anemia
normochromic, normocytic anemia pancytopenia moderate-marked poikilocytosis - often decryocytes
49
Anemia Associated with Renal Disease
caused by renal disease Hgb < 11 g/dL deficiency of EPO production by the kidneys
50
Lab Diagnosis of Anemia Associated with Renal Disease
normochromic, normocytic anemia normal RDW poikilocytosis retics - normal or decreased

Hematology (14 decks)

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