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RBC Disorders Flashcards

1
Q

Describe why a patient with chronic anemia will present with subtle symptoms of anemia vs a patient with acute blood loss

A

1) Body has time to compensate

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2
Q

Two types of Hemolytic Anemia

A

1) Intravascular

2) Extravascular

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3
Q

General Presentation of Intravascular hemolytic anemia

A

1) Hemoglobinuria
2) Hemoglobinuria
3) Hemosiderinuria

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4
Q

General Presentation of Extravascular Hemolytic Anemia

A

1) Splenomegaly
2) Jaundice
3) Anemia

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5
Q

Howell Jolly Bodies in Hereditary Spherocytosis is due to

A

DNA Inclusions

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6
Q

How do you Treat Hereditary Spherocytosis. Does that get rid of HS?

A

Splenectomy

No red blood cells are still spherocytic w/ Howell Jolly Bodies

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7
Q

Pt with Hereditary Spherocytosis comes in and presents with severe Anemia. What microbe do you suspect that this pt has?

A

1) Parvovirus B19

- Infects precursor red blood cells

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8
Q

Triggers of glucose 6 Phosphate Dehydrogenase Def.

A

1) Fava Beans
2) Drug
3) Stress
4) Infection

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9
Q

Clinical Feature of G6PD Def.

A

1) Antimalarials
2) Sulfanamides
3) Nitrofurantiona

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10
Q

Heinz Bodies are characteristic of

A

G6PD Def.

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11
Q

Clinical Presentation of Sickle Cell Anemia

A

1) Acute Respiratory Distress and Acute Chest Syndrome
2) Autosplenectomy and infection (encapsulated bacteria)
3) Aseptic Bone Necrosis and Osteomyelitis

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12
Q

How does iron overload occur in B-Thalassemia Major

A

1) Ineffective erythropoiesis and hemolysis
2) Tissues become Hypoxic
3) Increase EPO release
4) Marrow expansion and skeletal deformities
Hepcidin Suppression –> Iron Overload

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13
Q

Diagnosis of B-Thalassemia

A

Hemoglobin Electrophoresis: Increased HbF and HbA2

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14
Q

X-ray show ___ in B-thalassemia

A

Crew cut appearance due to medullary and extramedullary hematopoiesis

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15
Q

What is a major cause of death in Paroxysmal Nocturnal Hemoglobinuria

A

Thromboembolism: Hepatic, Portal, and Cerebral Veins

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16
Q

Gene mutation in PNH

A

PIGA mutation

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17
Q

Is PNH intravascular or extravascular

A

Intravascular

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18
Q

Complications of PNH

A

1) Iron Def, Anemia due to hemoglobinuria

2) AML (develops in 10% pts)

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19
Q

Treatment of PNH

A

Eculizumab: Blocks C5–> C5a

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20
Q

Treatment Side Effect of PNH

A

1) Eculizumab

- Sus, to neisseria meningiditis

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21
Q

Methotrexate causes what def.

A

Folate Def.

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22
Q

Why does Methotrexate cause a Folate Def.

A

1) Inhibits DHF Reductase

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23
Q

Causes for a Lack of Folate

A

1) Decrease Intake
2) Impaired Absorption
3) Blocked Metabolism
4) Increased requirements

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24
Q

Clinical Presentations of Folate Def,

A

1) Neural Tube Defects
2) Mental retardation
3) Anemia

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25
Q

Diagnostic of Folate Def.

A

Increased levels of Homocysteine

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26
Q

Pathogenesis of Aplastic Anemia

A

genetic altered stem cells

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27
Q

What is the rare inherited form of Aplastic Anemia

A

Faconi Anemia

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28
Q

Causes of Pure Red Cell Aplasia

A

1) Autoimmune (Thyoma)

2) Parvovirus B19

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29
Q

Leukoeryhtroblastosis is characteristic of …..

A

Myelophthisic Anemia

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30
Q

What is Myelophthisic Anemia

A

Space-occupying in marrow replace normal hematopoietic cell

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31
Q

List the values that are tested for in Hemolysis labs

A

1) Lactate Dehydrogenase (LDH)
2) Indirect (unconjugated) Bilirubin
3) Haptoglobin: Plasma protein that binds to free hemoglobin

32
Q

Male Pts or postmenopausal females w/ iron def. anemia you should check for ___

A

GI bleed

-Colonoscopy

33
Q

How do you treat iron def. anemia

A

Underlying disease

34
Q

what are the symptoms of Hypovolemic Shock

A

1) Hypotensive
2) Tachycardia
3) Weak Pulses

35
Q

3 Consequences of Acute Blood Loss

A

1) Hypovolemia
2) Myocardial ischemia
3) renal Failure

36
Q

Is the blood loss compensated or uncompensated in acute blood loss

A

Uncompensated

  • Normocytic Anemia
  • Normal retic count
37
Q

Treatment for Hypovolemic Shock

A

1) Apply pressure/compression
2) Obtain IV access (two large bore IVs)
3) Rapid Infusion of 1L NS or LR
4) Blood

38
Q

Acute Blood Loss scenario will wither be ___ or ___

A

1) Hypovolemia

2) Heart Attack

39
Q

Know child has SCD. What is something you can do to prevent stroke from occurring

A

1) Blood Transfusions during childhood

40
Q

Sx of Acute Chest Syndrome

A

1) Chest Pain
2) Dyspnea
3) Cough
4) Hypoxemia and Fever

41
Q

CXR will show what when a pt has Acute Chest SSyndrome

A

Pulmonary infiltrate

42
Q

Pathogenesis of avascular Necrosis

A

Microvascular occlusions

- causes painful crises

43
Q

Fever + Hip pain is ____ until proven otherwise

A

Osteomyelitis

44
Q

How to prevent painful attacks w/o meds in SCD

A

1) Avoid High Altitudes

2) Avoid Strenuous Exercise

45
Q

Pt who has anemia and High MCV but Normal RDW is indicative of

A

1) Chemotherapy
2) Antivirals
3) Alcohols
4) Aplastic Anemia

46
Q

Treatment of Aplastic Anemia

A

1) Transfusion Support

2) Growth Factor Support

47
Q

What are the ways that iron can be delivered for pts with iron def. anemia

A

1) Oral Iron:

2) Parentraral (Colloidal) Iron

48
Q

Dosage iron to a iron def. pt.

A

1) 200-400 mg FERROUS iron/day in a SINGLE dose

49
Q

Oral Iron cannot be ___ or ___

A

1) Enteric Coated

2) Sustained-release

50
Q

Oral iron cannot be taken with

A

1) Water

2) Juice

51
Q

Indications for Iron given via IV

A

1) Iron Malabsorption
2) Intolerance of oral therapy
3) Noncompliance

52
Q

What is the least expensive drug that is give for iron via IV

A

LMW Dextran

- Others are: Sodium Ferric gluconate complex, iron sucrose, iron isomaltoside

53
Q

Side Effects of Iron supplementation

A

1) Nausea
2) Constipation
3) Anonexia
4) Heart Burn
5) V/D
6) Dark Stools

54
Q

Most common population involved with iron poisoning

A

1) Young Children

55
Q

Sx of Iron Poisonings

A

1) Necrotizing Gastroenteritis w/ vomiting

2) Abdominal Pain and bloody diarrhea –> Shock

56
Q

Iron Poising can eventually lead to __, __, and __ in young children

A

1) shock
2) lethergy
3) Dyspnea

57
Q

Treatment for Iron Poisonings

A

Deferoxamine (Iron chelating Compound)

58
Q

When would you treat a Vit B12 def with Cyanocobalamin via IV

A

1) Neurological Symptoms Present

59
Q

Dosage for Oral Vit B12

A

1) 1-2mg/day for 2 wks

2) then 1mg daily

60
Q

Treatment/Dosage for Folate Def.

A

1) Oral Folate

2) 1mg/day for 4 months

61
Q

Treatment/Dosage for Folate Def. w/ malabsorption present

A

1) 1-5mg/day

62
Q

The physiologic lowest point for hemoglobin occurs at ___ of age and = to 10.1-14.5

A

2mo

63
Q

How does anemia affect hemoglobin-oxygen-dissociation curve

A

1) 2,3 DPG increase due to hypoxia and anemia

2) Shifts Curve to the Right

64
Q

Signs of Anemia in a Child

A

1) Tired
2) Irritable
3) Decrease Exercise Tolerance
4) Pale
5) blood in stool/urine
6) Increased LAD
7) SOB
8) Fever

65
Q

Main causes of anemia

A

1) Decreased Production
2) Increased Destruction
3) Blood Loss: Acute or Chronic

66
Q

Diamond- Blackfan Syndrome is ….

A

Red Cell Aplasia

67
Q

Pathophysiology of Diamond-Blackfan Syndrome

A

1) Increased Apoptosis of erythroid Precursors

68
Q

What type of anemia is Diamond-Blackfan Syndrome (MCV)

A

1) Macrocytic Anemia w/ low to normal retic count

69
Q

What is the most common inhereted Aplastic anemai

A

1) Fanconi anemia

70
Q

Pathophysiology of Fanconi Anemia

A

Increased Apoptosis in bone marrow

71
Q

When does Fanconi Anemia present

A

Up to 10yrs

72
Q

MDS is a precursor to ____

A

AML

**Esp if MDS is due to prior cytoxic therapy/radiation (t-MDS)

73
Q

___ is the clonal disorder w/ morphologic manifestations of many cell lines

A

Myelodysplastic Disorder

74
Q

How would you distinguish a MDS?

A

1) Dysmorphic Features in one or more lineages
2) Chromosomal Analysis
3) Blast Count

75
Q

Describe the types of MDS in terms of blast count.

A

1) RAEB-1: 5-9%

2) RAEB 2: 10-19% (ANEMIA will probabl be present at this point)

76
Q

List the Histomorphologic Features of MDS

A

1) Dyserythropiesis
2) Ring Sideroblasts
3) Pseudo Pelger-Huet Cells
4) Dysmegakaryopoiesis

77
Q

List the diver mutations in MDS

A

1) Epigenetic Mutations
2) RNA splicing factor mutations
3) Transcription factor mutations

SBL (8 decks)

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