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[Board Exam] Pathology > [4] Anemia > Flashcards

[4] Anemia Flashcards

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1
Q

Definition of Anemia in Males and Females

A

Hb < 13.5 g/dL in Males

Hb < 12.g g/dL in Females

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2
Q

Based on MCV, anemia is classified as?

A

Microcytic: MCV < 80
Normocytic: MCV = 80 - 100
Macrocytic: MCV > 100

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3
Q

What is the progenitor RBC?

A

Erythroblast

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4
Q

Microcytic anemias are due to what defect?

A

Decreased production of Hb

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5
Q

Heme is composed of?

A

Iron and Protoporphyrin

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6
Q

Sideroblastic Anemia is due to?

A

Defective protoporphyrin synthesis

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7
Q

How do Thalassemias cause Microcytic Anemia?

A

Decreased production of globin chains

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8
Q

Most common type of anemia

A

Iron Deficiency Anemia

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9
Q

Absorption of heme occurs in what part of the GIT?

A

Duodenum

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10
Q

What is responsible for the uptake of iron in the gut?

A

Enterocytes

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11
Q

Iron is bound to this molecule in the blood

A

Transferrin

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12
Q

Storage sites for iron in the body

A

Liver and Bone Marrow

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13
Q

What does TIBC measure?

A

Amount of Transferrin in the blood

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14
Q

What does Serum Ferritin measure?

A

How much iron is in the storage sites

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15
Q

These parasites are common causes of iron deficiency

A

Necator and Ancylostoma (Hookworms)

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16
Q

How goes gastrectomy cause IDA?

A

Decreased acid would cause less iron to be in a reduced state (Fe 2+), which is better absorbed compared to Fe3+

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17
Q

RBCs in early stage Iron Deficiency Anemia present as?

A

Normocytic Anemia

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18
Q

Function: Hepcidin

A

Sequesters iron in storage sites

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19
Q

How does chronic disease cause iron deficiency anemia?

A

Release of acute phase reactants cause:

Hepcidin: Iron sequestration
Decrease of EPO production

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20
Q

What enzyme is the rate-limiting step for the production of Protoporphyrin?

A

Aminolavalunic Acid Synthase (ALAS)

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21
Q

What is the Co-Factor for the rate-limiting step for the production of Protoporphyrin?

A

Vitamin B6

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22
Q

Classic presentation of a RBC in Sideroblastic Anemia

A

Ring Sideroblast (Ring of Iron that is trapped inside the mitochondria)

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23
Q

Most common form of congenital Sideroblastic anemia is caused by?

A

Loss of ALAS

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24
Q

Common causes of Acquired Sideroblastic Anemia

A

Alcoholism: Mitochondrial poison damages the production of protoporphyrin

Lead Poisoning: Denatures ALAD and Ferrochelatase

Vitamin B6 Deficiency: Co-factor for the first step of Protoporphyrin production

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25
Laboratory Findings in Patients with Sideroblastic Anemia
Ferritin: + TIBC: - Serum Iron: + % Saturation Iron: +
26
Laboratory Findings in Patients with Iron Deficiency Anemia
Ferritin: - TIBC: + Serum Iron: - % Saturation Iron: -
27
How do Thalassemias cause anemia?
Decreased synthesis of globin chains
28
Usual cause of alpha-thalassemias
Gene Deletion Normally there are 4 Alpha Alleles are present on Chromosome 16
29
In an alpha-thalassemia, is a cis deletion or trans deletion worse?
Cis deletion (Same gene knockout) Associated with increased risk of severe thalassemia in offspring Usually seen in Asians
30
What is the pathophysiology that occurs in a 3 gene deletion alpha-thalassemia?
B chains may form tetramers that cause RBC damage Patients present with severe anemia
31
What is the pathophysiology that occurs in a 4 gene deletion alpha-thalassemia?
y chains form tetramers (Hb Barts) that damage RBCs Fatal in utero (Hydrops fetalis)
32
Beta-Thalassemia is caused by?
Gene Mutation (Not deletions like Alpha-thalassemias) Two B genes are present on Chromosome 11, mutations result in absent (B0) or diminished (B+) production of B-globin chains
33
What is the mildest form of Beta-Thalassemia?
B/B+ (Beta-Thalassemia Minor) Usually asymptomatic with increased RBC count
34
What is the most severe form of Beta-Thalassemia? When does this disease present in the patient? Why?
B0/B0 (Beta-Thalassemia Major) Presents with severe anemia a few months after birth because HbF (A2Y2) at birth is temporarily protective
35
Treatment for patients with Beta-Thalassemia? What is a complication of this treatment?
Chronic Transfusion May cause secondary hemochromatosis (Remember that the body does not really have a way to remove iron)
36
Presentation of Beta-Thalassemic RBCs on Blood Smear
Microcytic, hypochromic target cells with nucleated red blood cells
37
Electrophoresis presentation of Beta-Thalassemia Major
HbA: - or 0 HbA2: + HbF: +
38
Most common cause of Macrocytic Anemia
Folate or Vitamin B12 Deficiency (Megaloblastic Anemia)
39
How does folate or vitamin B12 deficiency cause anemia?
Impairs synthesis of DNA precursors
40
What drug commonly causes macrocytic anemia?
5-FU
41
Where is folate obtained from and where is it absorbed in the GIT?
Folate is obtained from green, leafy vegetables Jejunum
42
Blood smear finding of Macrocytic Anemia
``` Macrocytic RBCs Hypersegmented Neutrophils (>5 lobes) ```
43
How does methylmalonic acid help differentiate a folate and vitamin B12 deficiency?
Normal serum levels of methylmalonic acid points to a folate deficiency as Vitamin B12 is necessary for its conversion to succinyl CoA
44
In what part of the GIT is Vitamin B12 absorbed?
Ileum
45
Why is Vitamin B12 deficiency less common than folate deficiency?
There is a large hepatic store of Vitamin B12
46
Most common cause of Vitamin B12 deficiency
Pernicious Anemia | Autoimmune destruction of parietal cells leading to IF deficiency: Vitamin B12 binds to IF to be absorbed in the ileum
47
How does pancreatic insufficiency cause B12 deficiency?
Lack of proteases to cleave the B12-R Factor unit, which prevents IF from binding to B12 for absorption
48
What are reticulocytes?
Young RBCs released from bone marrow
49
RBC Lifespan
120 Days
50
How do you correct reticulocyte count?
Reticulocyte x Hct/45
51
Define: Hereditary Spherocytosis
Inherited defect of RBC cytoskeleton membrane tethering proteins Ankyrin, spectrin and band 3:1
52
How does having a spherocyte shape of RBC cause anemia?
Unable to effectively pass through splenic sinusoids causing excessive destruction
53
Diagnostic test for Hereditary Spherocytosis
Osmotic Fragility Test | Increased fragility in hypotonic solution
54
Treatment for Hereditary Spherocytosis
Splenectomy
55
What is a Howell-Jolly Body?
Histopathologic finding of remnant DNA in Erythrocytes usually seen in patients with severe splenic dysfunction
56
What is the cause of Sickle Cell Anemia?
AR Mutation in B Chain of Hemoglobin | Glutamic Acid replaced by Valine
57
What is the predominant Hb of Sickle Cell Anemia? What is the problem with this type of Hb?
HbS These Hb polymerize when under oxidative stress causing the classic sickle pattern
58
Treatment for Sickle Cell Anemia
Hydroxyurea Causes increase of HbF, which is protective against sickling
59
Most common cause of death for children with sickle cell anemia
Infection by encapsulated organisms (H. influenza, Strep pneumo) Results from autophagy of the spleen
60
Most common cause of death for adults with sickle cell anemia
Acute chest syndrome Caused by vasoocclusion of pulmonary vasculature, usually preceded by an infection
61
Cause of Hemoglobin C
AR defect of Hemoglobin B chain | Glutamic Acid is replaced by Ly-C-ine
62
Pathophysiology of Paroxysmal Nocturnal Hemoglobinuria
Acquired defect in myeloid stem cells resulting in absent GPI Renders cells susceptible to complement (occurs at night due to mild respiratory acidosis that occurs during sleep)
63
Screening Test for Paroxysmal Nocturnal Hemoglobinuria
Sucrose Test
64
Confirmatory Test for Paroxysmal Nocturnal Hemoglobinuria
Acidified Serum Test | Flow Cytometry to detect lack of CD55 (DAF)
65
In patients with G6PD Deficiency oxidative stress causes Hb to precipitate as?
Heinz Bodies
66
What are some common causes of oxidative stress in patients with G6PD?
Infections Drugs (Primaquine, sulfa drugs, dapsone) Fava beans
67
Presentation of patients with G6PD
Hemoglobinuria and Back Pain
68
Pathophysiology of Autoimmune Hemolytic Anemia
IgG or IgM mediated destruction of RBC
69
Treatment of Autoimmune Hemolytic Anemia
Cessation of offending drug Steroids IVIG Splenectomy
70
Test used to diagnose Immune Hemolytic Anemia
Coombs Test
71
Which of the Coombs Tests are more important in the diagnosis of Autoimmune Hemolytic Anemia?
Direct Coombs: Anti-IgG is added to patients RBCs. If they are already coated with IgG, then they will agglutinate. [Indirect Coombs: Tests whether the patients serum has the antibody]
72
Hallmark of Microangiopathic Hemolytic Anemia
Schistoscytes
73
Define: Myelophthisic Process
Pathologic process that replaces bone marrow resulting in pancytopenia

[Board Exam] Pathology (5 decks)

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