Anemia Flashcards
0
Q
Iron Deficiency anemia (Pathogenesis, labs, morphology)
A
- Most common form in the world,
- Microcytic, Decreased hemoglobin synthesis
- Decrease in serum ferratin, and decreased % saturation, Increase in TIBC, increased FEP
- central pallor much lighter, wider range of cell size
- iron absorbed in gut
1
Q
Classifications of anemias (3)
A
- microcytic (100): folate, B12 deficiency
2
Q
Causes of iron deficiency anemia
A
- infants: Breast Feeding
- children: Nutrition
- Adults: peptic ulcer disease/Menorrhagia
- Elderly: colon polyps/carcinoma in the west, Undeveloped countries include Hookworm (ancyclostoma and necator)
3
Q
Plummer-Vinson syndrome
A
- Esophageal web
- Atrophic glossitis
- Dysphagia
- Beefy red tongue
- Anemia
4
Q
Anemia of chronic disease
A
- Hepcidin Sequester’s iron And suppresses EPO to inhibit bacterial development
- ## Lab findings include increased ferratin, decreased TIBC, decreased serum iron, and decreased % saturation, Increased FEP
5
Q
Sideroblastic anemia
A
- Defective protoporphyrin synthesis
- iron (and associated proteins) increase, Hb decrease
- ALAS -> ALAD -> / (mitos) -> ferrochetalase
- most commonly congenital lack of ALAS
~ alcoholism, Pb (ALAD) and B6 deficiency - ringed sideroblasts: iron builds up around the nucleus of primitive RBCs
6
Q
Ringed sideroblasts
A
- iron builds up around the nucleus of primitive RBCs
- sideroblastic anemia
- iron overloaded state
7
Q
Thalassemia
A
- mutating gene ends up producing inappropriately matched dimers
- less protein + intact DNA replication = microcytic anemia (extra division)
8
Q
Alpha thalassemia
A
- 4 alpha genes on ch 16
- number of deletions corresponds to severity
- double cis more dangerous
- HbH: beta tetramers
9
Q
Beta thalassemia
A
- B/B+ mildest
- Bo/Bo most severe
- crew cut/ chipmunk cheeks
- a-tetramers cause ineffective erythropoisis and extra vascular hemolysis
- target cells
10
Q
Target cells
A
- results from microcytic cells having excess cytoplasm due to decreased protein content
11
Q
Parvovirus B19
A
- initial viremic symptoms, followed by slapped rash
- increase RBC precursors: any condition that results in
12
Q
Macrocytic anemia
A
- MCV: > 100
- decreased DNA synthesis -> less division
- folate, B12 deficiency, drugs (5-FU, et al)
- also shows hyper segmented nucleus
- hyper segmented neutrophils
13
Q
Folate deficiency (etiology (3), morphology, lab findings)
A
- poor diet (EtOH), increased demand (preggers), drugs (methotrexate)
- macrocytic RBCs, hyper seg nuetrophils, glossitis, increased serum homocysteine, normal methylmalonic acid (indicates B12 in normal)
14
Q
B12 deficiency
A
- Less common than folate deficiency because of larger B12 stores
- Pernicious anemia most common cause
- pancreatic insufficiency, and inflammatory causes
- dietary is rare
- macrocytic RBCs, glossitis, central demyelination
- B12 deficiency -> increase in methylmalonic acid
- will also show increase in serum homocysteine
15
Q
Normocytic anemia
A
- MCV ~ 80-10
- Reticulocytes (RC) can increase to 3%
- correct with Hb x (Hct/45)
- 3+% -> good marrow response = increased destruction/loss
- 3-% -> poor marrow response = decreased production
- spherocytosis, HbS/C, PNH, G6PDHD, autoimmune, parvovirus, aplastic anemia,
16
Q
Peripheral hemolysis (mechanism, effect)
A
- globin, Iron and protoporphyrin are released (iron is scavanged, globin is broken into AAs, proph is conjugated as biliruben)
- jaundice
- hemoglobinurea
- hemoglobinemia
- hemosiderinurea
- decreased haptoglobin
17
Q
Heriditery spherocytosis (pathenogenesis, indicators, diagnosis, trt)
A
- congenital mutation of actin anchoring system
- loss of central pallor
- increase in RWD and MCHC (mean corpuscular [Hb])
- splenomegaly (killing all the unusual blood cells)
- hypotonic fragility
- splenectomy if anemia gets bad
18
Q
HbS (mutation, complications, trt)
A
- Glu-> Val: hypoxia conditions = polymerization to crisis
- homozygous mutant - 90% HbS
- HbF is protective (hydroxyurea)
- expansion of hematopoiesis (crew cut, chipmunk)
- increased risk of Parvovirus due to increased RCs
19
Q
HbS sickling presentation and risks
A
- autosplenectomy
- dactylitis
- increased risk of encapsulated bacteria (most common cause of death in kids)
- increased risk of salmonella osteomyelitis
- evidenced by Howell-Jolly bodies
20
Q
Acute chest syndrome
A
- typically follows pneumonia -> crisis in pulmonary microcirculation
- most common cause of death in adult patients
21
Q
Renal considerations in HbS
A
- hypertonicity and hypoxic conditions of medulla lead to microinfarcts and eventual decreased ability to concentrate urine
22
Q
HbC
A
- LyCine Crystals
- mild anemia
- HbC crystals are seen on smear
23
Q
Paroxysmal nocturnal hemoglobinurea (etiology, pathenogenesis, complication, diagnosis)
A
- GPI anchor deficit (CD55, CD59, C8)
- nighttime acidotic condition (or exercise) -> compliment activation not avoided by DAF and MIRL (gpi anchored CD55)
- main cause of death is thrombosis
- diagnosed by ID lack of CD55
24
G6PDH deficiency (etiology, types, indicator)
- reduced ability to respond to oxidative stress
- drugs (sulfa et al)
- African: longer 1/2life
- Mediterranean: markedly decreased 1/2Life
- Heinz bodies
25
Heinz bodies
- G6PDH deficiency
26
Autoimmune hemolytic anemia (Abs, etiology, pathenogenesis)
- IgG or IgM
- macs pick off Igs -> spherocytosis
- SLE, ALL and drugs (penicillin, quinidine, aldomet, cephalosporins, procainamide)
- IgM: cold agglutination (assoc with reyneauds)
~ IgM assoc with Mycoplasma and Mononucleosis
- direct (most important for IHA) and indirect Coombs test
27
Microangiopathic hemolytic anemia
- typically caused by TTP, HUS, DIC
| - production of schistocytes
28
Anemia due to underproduction
```
- Parvovirus B19: Infects progenitor cells and halts erythropoiesis
~ supportive trt
- aplastic anemia:
~ damage to HPSC: pancytopenia
~ biopsy reveals empty fatty marrow
~ cessation of drugs, CFU-GM/G
```
29
Iron overload in anemia
- extra vascular only
30
DIC in anemia
- intravascular only
31
Haptoglobin
- Hemoglobin scavanger
- decreases in anemia due to release of Hb
- Hb Eq of ferritin
32
Pyruvate kinase deficiency
- anemia
| - decreased ATP -> accumulates 2,3 BPG -> causes right shift thus tolerates greater of oxygen dissociation
33
Warm autoimmune anemia
- 37c, IgG
- No complement activation
- Predominantly extravascular
- associated with SLE
34
Cold autoimmune anemia (2)
- 32c, IgM
| - Cold agglutinin syndrome and Paroxysmal cold hemoglobinuria
35
Cold agglutinin syndrome and associated conditions/infxns
- IgM, extravascular
- associated with raynauds ph.
- associated with mycoplasma and mono/HIV, influenza infxns
36
Paroxysmal cold hemoglobinuria
- very rare
- intermittent massive hemolysis following exposure to cold
- compliment fixed by IgG-> rewarding leads to intravascular hemolysis
- Donath-Landsteiner Ab (+)
- idiopathic, can follow syphillis infxn
37
Aplastic anemia
- hypo proliferation at HPSC (anemia, neutropenia, thrombocytopenia)
- etiologies:
~ loss of stem cells
~ suppression of stem cells by macs and T lymphs
~ Ab mediated inhibition
~ Unfavorable microenvironment
- drugs: chloramphenicol, benzene et al
- hepatitis C D and E
38
Blackfan-Diamond syndrome
Pure red cell aplasia
| - congenital
39
Lack of CD 55
PNH diagnosis
40
Ribonucleotide reductase inhibitor
Hydroxyurea:cml
41
Donath-Landsteiner Abs and post syphilitic infxn
- associated with paroxysmal cold anemia
42
HbA2
Present in thalassemia minor
43
Duodenum, jejunum, Ilium,
Iron, folate, B12
