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Flashcards in Disease Deck (18)
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1

Pathology:

Breakdown of hemoglobin in vessels.

Lab test:

↑free Hgb
↑LDH
↑SGOT
↑RetC-to compensate for hemolysis
↑RDW-fast output old and new cells
Hemoglobinuria/plasma – b/c cannot process all.
↑ Indir bili-overloaded system cannot keep up with demand so bili does not get conjugated to glucuronic acid
↑Metheme/methemoglobin-b/c broken free heme byproduct
↓Haptoglobin-taken out of serum when binds heme to the liver

Disease: Intravascular Hemolysis

Splenomagaly

2

Pathology:

Ingestion and clearance by macrophages in the Reticulo-endothelial system. Fe stored in ferritin and hemeàbili
Enterohepatic recirculation of urobilirubin after cleavage of glucuronic acid in gutà elevated levels of direct bili.

Lab tests:
↑LDH
↑SGOT
↑RetC
↑RDW
↑Direct bili (enterohepatic recirculation)

Extravascular Hemolysis

3

Pathology:

Protects against oxidative stressà ↓ G6PDà↓reduced glutathioneàlacks ability to help with oxidative stressà Hgb sticks to spectrin on RBC wallà abnml shapeàlysis by spleen
-sex-linked rec

Lab Test:
Protein in urine (?)
Perif Smear (sometimes see)à
Microspherocytes
Blister cellsà cytoskeleton defect
Bite cellsà same
Usually NO morphology Δ

Treatment: -Folate
-Avoid oxidative foods (fava beans)
-supportive care

Glucose-6-Phosphate Dehydrogenase

Clinical SS:

-Intermittent acute hemolytic anemia
-hyperbilirubinemia

4

Clinical SS:

-Anemia mild-mod
-Jaundice
-Splenomegaly
-Hyperbili in 1/3rd of neonates
-Aplastic crisis
-Bilirubin -> high stone -> cholecystectomy

Pathology:
Spectrin/ankyrin/
band 3 mutations -> weak cytoskeleton, spherical shape _>splenic entrapment and easy to lyse

Lab Test:Hct-variable
Hgb-variable
↑RetCàaccount for hemolysis
↑indir biliàtoo much heme to process
↓MCVàmaking too fast so microcytic
Osmotic fragility testàabnml/low
Spherocytes on smear

Treatment:
splenectomy

Hereditary Spherocytosis

5

Clinical SS:

Variable chronic anemia
Extravascular hemolysis
Splenomegaly
Gallstones
Aplastic crisis

Pathology:

Problemàpyruvate problemà ↓ATP productionà ↑2,3-BPG and mis-shapen RBCsàsplenic destruction

Lab Test:
↑RetCàaccount for hemolysis
NO morphology Δ

Treatment:
-Folate
-Spenectomy-s.times
-transfusions in severe cases

PK-Pyruvate Kinase Deficiency

6

Clinical SS:
Dark Urine
Acute/Chronic anemia
Pallor/Jaundice
Splenectomy s.times

SAME FOR EXTRA


Pathology:
IgG and IgMàactivates C5-9 complementàRBC damage by complement

Lab Test:

↑RetC
↑Bili
Hemoglobinuria
Spherocytes
Bite cells
Direct Coombs (test for IgG, C3D, C4D-bound to RBC)à + Complement (C3D, C4D)
Indirect Coombs
(test for IgG and Complement-free in plasma)à
+ complement
Treatment:

Autoimmune Hemolytic Disorders
COLD/INTRAvascular

7

Clinical SS:
Dark Urine
Acute/Chronic anemia
Pallor/Jaundice
Splenectomy s.times

Pathology:
IgG only
Opsonizing
Macrophages and spleen affected so that the lysis is in the spleen and outside the blood system

Lab Test:
↑RetC
↑Bili
Hemoglobinuria
Spherocytes
Bite cells
Direct Coombs (test for IgG, C3D, C4D-bound to RBC)à + IgG (sometimes weak complement)
Indirect Coombs
(test for IgG and Complement-free in plasma)à
+ IgG

Treatment:

Autoimmune Hemolytic Disorders
WARM/EXTRAvasc.

8

Clinical SS:
Fatigue
Pallor
Pain with exercise
SOB
Tachycardia
Muscle problems
Neurological problems

Pathology:
Dietary
Absorption problems
Inflammation/Infectionà
Cytokine production

Lab Test:
Microcytic and Hypochromic
↓Hgb-no iron to bind heme
↓Hct-less and smaller RBC’s bc no iron
↓ferritin-iron stores used up
↓RetC-because no iron to make RBC’s
↓serum iron
↑TIBC-bc capacity is increased due to no binding
↑RDW
Perif Smear
Spherocytes, fragmented cells, target cells

Treatment:
Oral iron salts (150-200 mg tid)
IV/IM iron. 1st see serum Fe respond àRetC nmlizes, Hgb nml after 7- 10 days. Continue treatment until ferritin stores returned and MCV and RDW are nml (which will take 100 days or so until abnml RBC’s have been removed)

Iron Deficiency

9

Clinical SS:
-Cyanosis without accompanying ↓ in arterial PP
-Brown-blood due to Fe+3 without change when exposed to O2
-In severe casesàbrady, respire depression, convulsions, acidosis.

Pathology:
Acquired
-free radicals (NO, H2O2)
-Drugs (benzocaine)
Hereditary
-homozygous def of cytochrome-b-5-reductase (prevents reduction of Fe+3àFe+2)
-Hemoglobin M (mutation of chains that inhibits reduction of Fe+3)

Lab Test:

Treatment:

Acquired
Remove drug or chemical causing disease
Hereditary
Methylene Blue

Methemoglobinemia

10

Clinical SS:
-Variable anemia
-Underlying inflammatory disease
-Renal insufficiency
-Thyroid disorder
-Adrenal insufficiency
-fever
-arthritis
-swelling (infections)

Pathology:
Inability to use iron stores and decrease EPO production result from inflammatory cytokines:
-TNF/IL-1à↓iron mobilization/ EPO production
-INFγ/βàinhibits erythropoisis
-Hepcidin (induced by cytokines) à↑ Fe storage, ↓duod abs, block Fe release from macrophages

Lab Test:
-Hgb 8-12
-Usually Normocytic/ normochromic à microcytic /mild hypochroma
↓RetC –reticulocytopenia b/c cannot get iron since sequestered due to presence of hepcidin.
↑Ferritin-due to increased/sequestration of iron(DIFF from Iron defic anemia)
↓TIBC (DIFF from IDA)
↓Serum Iron (like IDA)
↓Transferrin saturation (as with IDA)
↓EPO for degree of anemia (espec with renal disease. But usually not seen until <40% renal function)

Treatment:
-Treat underlying condition to à ↓cytokines and
inflammation
-Give iron
-Give EPO (especially with renal disease)
-Hormone replacement of endocrine disease

Anemia of Chronic Disease/Inflammation

11

Clinical SS:
-Personality changes
-irritability
-weakness
-wt loss
-abdominal pain and vomiting

Pathology:-Pb inhibits synthesis of protoporphyrin ringà↓heme

Lab Test:
↓MCV =
Microcytic/hypochromic because Pb is preventing production of porphyrin ring
↓RetC-because no heme to make iron so cannot make RBC’s
↑Lead levels
Basophilic stippling-aggregates of RNA(also seen with macrocytic anemia of B12 and Folate)

Treatment:
1.. REMOVE source of lead
2. Chelation

Lead Based Anemia

12

Clinical SS:
Variable anemia


Pathology:
Impaired production of protoporphyrin ring or incorporation of Fe into hemeà accumulation of iron in mitochondriaà Ring sideroblasts

Lab Test:
↓MCV =
Microcytic/hypochromic
Pappenheimer bodies (ppt iron in mitochondriaàring around nucleus)

Treatment:
B6-s.times works
Transfusions
Tx of other underlying cause (Cu deficiency, EtOHism, drugs)

Sideroblastic Anemia

13

Clinical SS:
Slow (months) onset
CNS changes-cognitive dysfunction and emotional changes
Numbness, tingling, loss of fine sensations
Proprioception
Ataxia
CNS changes may be permanent.

Pathology:
Required for synthesis of methionine from homocysteineàprecursors for DNA synthesisàeffect erythropoisis in BM.
So essentially it is a disease of ↓DNA synthesis
Causes
-Vegan Diet-rare cause
-IF deficient = Pernicious Anemiaà (mal-abs of B12)
-autoimmune destruction of parietal cells that secrete IF
-Loss of ilial receptors-surgery, IBD
TcII deficiency

Lab Test:↑MVC =
Macrocytic/Normochromic (because increase in size as waiting for DNA to be made-arrest in S phase
↓RBC
↓RetC-because no B12 to make cells
Hypersegmented neutrophils-classical sign of B12 or folate deficiency.
↑RDWàanisocytosis
-Nuclear-Cytoplasmic Asynchrony
M:E ratioàE predominance
↑Homocysteine levels
↑methylmalonic acid
↑ to nml serum folate*
↓B-12 levels (s.times predictive)
Shillings Test: If abnml then add IF and see if corrects. If normal then not B-12 abs problem


Treatment:
B-12 injections
Oral replacement
↑dosesàabs via mass action
Watch for other immune diseases
-Rapid response to treatment and slowly resolving CNS indicates accurate dx.

B-12 Deficiency

14

Clinical SS:
Quick Onset (weeks)

Pathology:
Required for synthesis of methionine from homocysteineàprecursors for DNA synthesisàeffect erythropoisis in BM.
So essentially it is a disease of ↓DNA synthesis
Causes
Insufficient dietary intake*1⁰
Mal-absàparasitic infection (disrupts enterohepatic recirc)
EtOH consumption excess


Lab Test:
↑MVC =
Macrocytic/Normochromic (because
↓RBC
↓RetC-because no B12 to make cells
Hypersegmented neutrophils
↑RDWàanisocytosis
-Nuclear-Cytoplasmic Asynchrony
M:E ratioàE predominance
↑Homocysteine levels
↓serum folate
↓methylmalonic acid

Treatment:
Folate 1mg/d PO/IV/IM
Rapid recovery of CBC.

Folate Deficiency

15

Clinical SS:
Chronic Hemolytic Anemia
Aplastic crisis - ↓RetC
Pain crisis – Acute chest pain, multi-organ failure, priapism, bone infaction.
Pathology:
Sickling cells are actually “sticky” which leads to vessel damage, endothelial remodeling and vessel narrowing. This microvessel damage affects all organs (lungs, retina, kidneys, spleen, CNS)

Lab Test:
↑RetC-to compensate for hemolysis (except during aplastic crisis)
↑WBC/platelets due to BM response
↑RDW-increase production and constantly changing shape of the sickled cell
↓MCV – Microcytosis (Sβ⁰/Sβ+)
Heme C crystals = red rods in RBC’s (SC disease)
↑Hb Fetal (2-30%)
↑LDH, AST (released from lysed RBC)
↑indirect bilirubin-due to hemolysis
↑Total bilirubin
Perif Smear
Schistocytes
Polychromasia-blue colored cells
Anisocytosis (RDW effect)
Howell Jolly bodies-occur in pt w/out functional spleen due to injury/occlusion from sickled cellsàsplenic sequestration

Treatment:
1. BM transplant w/ HLA matched sibling is 90%
2. Hydroxyureaà ↑Hb F
3. Transfusions – decrease pain crises extend life.

Sickle Cell Anemia

16

Clinical SS:
Mod-severe anemia
Infant Barts bodies = γ-4
H-bodies in adult

Pathology:
--/-α àlimited binding to gamma chain during in-utero development and then β-agglutination during life.

Lab Test:
↓MCV – bc low α chains and agglutination of β-chains whichàBarts
↓MCHC-because low levels of heme since less produced

Treatment:
Transfusions

Hb H (Barts)

17

Clinical SS:
Mild Anemia
Mild splenomegaly
Extramedullary Hematopoiesisàfrontal bossing, hair on ends, enlarged mandible etc

Pathology:
Hb E is an unstable globin chainà ↓amount for heme production

Lab Test:
↓MCV =
Microcytic
↓MCHC-because low levels of heme
Target cells = Mexican hat = Excess of RBC plasma membrane due to ↓in heme content to RBC itself (RBC is not filled up so takes on this shape)

Treatment:
No transfusions

Hb EE

18

Clinical SS:
Severe anemia
Extramedullary Hematopoiesis
2/3rds have abnml endocrine problems (hypothyroid, gonad problems etc)

Pathology:
2 severely abnml β-globin chains
↑ Levels of Hb F (10-90%)

Lab Test:
↓MCV =
Microcytic
↓MCHC-because low levels of heme since less produced
Target cells = Mexican hat = Excess of RBC plasma membrane

Treatment:
ALWAYS transfusions
Always chelation tx
Hydroxyurea=↑HbF (via increasing gamma chains that can bind with the excess α-chains.

Cooley’s β-thal Major