Lecture 10 (labs) -Exam 5 Flashcards

Question 1

Q

Dysfunction of the immune system
* The immune network is tightly regulated by what?
* Derangement in this immune balance can result in what? (2)?

Answer

A

The immune network is tightly regulated by cells and cytokines

Derangement in this immune balance can result in:
* Autoimmune conditions: Immune response to self-antigens (failure of self-tolerance)
* Immunodeficiency syndromes: Failure to recognize pathogens and eliminate them

Question 2

Q

Autoimmune Conditions
* When a person has an autoimmune disease, what happens to the immune system?

Answer

A

When a person has an autoimmune disease, the immune system malfunctions and may produce large amounts of autoantibodies
* These antibodies may affect blood cells, skin, joints, kidneys, lungs, nervous system, and organ systems

Question 3

Q

Examples of autoimmune conditions, explain what they are:
* Systemic lupus erythematosus (SLE)
* Rheumatoid arthritis
* Scleroderma
* Sjogrens syndrome
* Amyloidosis

Answer

A

Systemic lupus erythematosus (SLE) – chronic disease that can affect the joints, skin, heart, lungs, blood vessels, kidneys, and brain
Rheumatoid arthritis – affects the joints causing pain and swelling
Scleroderma- rare disease that may affect the skin, blood vessels, and organs
Sjogrens syndrome- rare disease that affects the glands that make tears and saliva and other parts of the body
Amyloidosis - not autoimmune but closely related….

Question 4

Q

Antinuclear antibody test (ANA)
* What does it look for?
* Antinuclear antibody attacks what?
* Is it normal or not?
* What does antinuclear mean?

Answer

A

A test that looks for antinuclear antibodies in your blood
* Antinuclear antibody attacks your own healthy cells
* Its normal to have a few in your blood
* “antinuclear” means it targets the nucleus (center) of the cells

Question 5

Q

Antinuclear Antibody Test (ANA)
* How is done?
* Gets reported as what?

Answer

A

How is the test done?
* Indirect immunofluorescence – gives two results- a titer and a pattern

Gets reported as a titer- any titer over 1:160 is positive

Question 6

Q

positive ANA test:
* You might have what?
* The test does not do what?
* What does a negative test tell?
* Positive means you might have what? What is the exception?

Answer

A

You MIGHT have an autoimmune condition
This test does not diagnose a specific disease
Negative means you are less likely to have an autoimmune disorder but does not completely rule out the possibility
Positive means you might have an autoimmune disorder BUT some healthy people have higher levels in their blood

Question 7

Q

Positive ANA:
* When does it normally increase?
* How many healthy people have a positive ANA test result?

Answer

A

Levels increase as you age
As many as 1/3 of healthy adults over the age of 65 may have a positive ANA test result

Question 8

Q

what to do with a positive ANA
* What do you look at?
* What questions do you need to ask?
* What else can you order?

Answer

A

Look at the patient, not the numbers!
Are there symptoms? Are there physical exam findings? Are you concerned with a condition?
There are other tests that can be ordered to further differentiate the condition

Question 9

Q

Pt with positive ANA, There are other tests that can be ordered to further differentiate the condition. Give examples for SLE and Sjogren’s disease

Answer

A

Systemic lupus erythematosus—If a diagnosis of SLE is suspected, then additional tests, looking for autoantibodies directed against double-stranded DNA, Sm antigens. Because these antibodies are relatively specific for SLE, the results may provide important clues to facilitate the diagnosis of SLE.
Sjögren’s disease—If a diagnosis of Sjögren’s disease is suspected, test for autoantibodies directed against antigens known as SSA and SSB. The presence of these autoantibodies provides support for the diagnosis of Sjögren’s disease.

Question 10

Q

rheumatologic tests
* What is CBC?
* What is Creatinine?
* What is LFTs?
* WHat is ESR?

Answer

A

Complete Blood Count (CBC):includes white blood cell count, hematocrit, and platelets. Can be abnormal in certain rheumatologic conditions or because of medication toxicity
Creatinine (Cr):measures kidney function
Liver Function Tests (ALT/AST):measures liver function; can be elevated due to medication toxicity
Erythrocyte Sedimentation Rate (“sed rate” or ESR):measures how quickly red blood cells fall to the bottom of a test tube; elevated in inflammatory conditions such as infection or rheumatologic diseases

Question 11

Q

rheumatologic tests
* What is CRP?
* What is ANA?
* What is ANA panel?
* What is RF?
* What is CCP?

Answer

A

C Reactive Protein (CRP):a protein that alsocan be elevated in inflammation
Anti-nuclear Antibody (ANA):measures blood levels of antibodies that can be seen in patients with rheumatologic diseases including lupus, scleroderma, rheumatoid arthritis, dermatomyositis, mixed connective tissue disease, and Sjögren’s syndrome
ANA panel:further tests that may be performed for patients with a positive ANA that may help to narrow down the diagnosis. Includes anti-smith, dsDNA, SSA/SSB, RNP and centromere antibodies
Rheumatoid Factor (RF):antibody found in 70-80% of patients with rheumatoid arthritis
Cyclic Citrullinated Peptide (CCP):a more specific test for rheumatoid arthritis

Question 12

Q

rheumatologic tests
* What is Creatine Phosphokinase (CPK or CK)?
* What is uric acid or urate?
* What is complement?
* What is SPEP?

Answer

A

Creatine Phosphokinase (CPK or CK):muscle enzyme that can be elevated in autoimmune diseases that affect the muscles such as polymyositis or due to medication toxicity (such as from statins used to treat high cholesterol)
Uric Acid or Urate:increased levels can be seen in gout
Complement (e.g. C3, C4):measures a group of proteins important to the body’s response to infections; levels can be low in lupus
Serum Protein Electrophoresis (SPEP):this test separates proteins into albumin and globulins that form important components of the immune system. Can be abnormal in certain blood diseases such as multiple myeloma

Question 13

Q

rheumatologic tests
* What is HLA-B27
* What is Anticardiolipin Antibodies (ACL)
* What is Anti-Neutrophil Cytoplasmic Antibody (ANCA)?
* What is ACE?

Answer

A

HLA-B27:A genetic marker that can be seen in a group of rheumatic diseases called the “spondyloarthritides” such as ankylosing spondylitis
Anticardiolipin Antibodies (ACL), lupus anticoagulant (LAC), Beta-2-Glycoprotein-1 (B2GP1):tests for certain antibodies that can be seen in patients who have blood clots
Anti-Neutrophil Cytoplasmic Antibody (ANCA):antibodies that can be seen in rare rheumatic diseases such as vasculitis
Angiotensin Converting Enzyme (ACE):an enzyme found in lung and kidney cells. Can be helpful in following disease activity in patients with sarcoidosis

Question 14

Q

ESR- Erythrocyte Sedimentation Rate
* What does it measure?
* Helpful in what?
* Useful in what?
* This is NOT what? Can be evlvated when?

Question 15

Q

Rheumatoid factor
* What are they? What do they form?

Answer

A

Rheumatoid factors are autoantibodies directed against IgG or IgM
* They form antibody complexes with IgG and IgM molecules that cause an issue like joint swelling and deformity

Question 16

Q

Why is RF a misnomer?

Answer

A

Rheumatoid factor is a misnomer- it does not definitively diagnose RA, but may not point towards it
* RF is present in many people at low levels, but can be present in higher levels in 5-10% of the population
* Can also be seen in varying amounts in other autoimmune conditions like SLE and Sjogrens, infections/viruses like malaria, rubella, Hep C, and malignancies.

Question 17

Q

At diagnosis, only 60% of patients with what test positive for RF?

Answer

A

At diagnosis, only 60% of patients with rheumatoid arthritis test positive for rheumatoid factor, but 75-80% of patients are positive at some point in the course of their disease

Question 18

Q

Anti-CCP antibody
* What is it for?
* Has a sensitivity and specificity of what?
* What is the high risk of? What is there a low risk of?

Answer

A

Anti-cyclic citrullinated peptide (CCP) antibodies are particularly useful in the diagnosis of rheumatoid arthritis

Reportedly has a sensitivity of 47-75% and specificity of 90-96%
* Low sensitivity so the test may fail to detect a significant number of individuals who actually have the disease (higher risk of false negative result)
* High specificity means the test correctly identifies individuals who are truly disease free (low risk of false positive)

Question 19

Q

Anti-CCP antibody
* Why is it Even more helpful is the presence early in the disease process?
* more specific than what?

Answer

A

Even more helpful is the presence early in the disease process – identifies patients who are likely to have severe disease and irreversible damage
Anti-CCP antibody testing is more specific than RF for diagnosing rheumatic arthritis

Question 20

Q

Anti dsDNA
* Play an important role in what?

Answer

A

Play an important role in the diagnosis, classification, and management of systemic lupus erythematous (SLE)

Question 21

Q

Anti double-stranded DNA (anti dsDNA)
* SLE has a wide range of what?
* These antibodies have a high or low specific? BUT what is an issue?
* Would not do this test when?
* Anti dsDNA antibodies increases risk of what?

Answer

A

SLE has a wide range of autoantibodies, but in 2019, there was criteria reinforcing the importance of autoantibodies in SLE diagnosis, assigning the highest score (6 points) to anti-ds DNA antibodies/anti-SM
These antibodies are relatively specific (95%) for SLE making them useful for diagnosis, especially if paired with a positive ANA test and clinical suspicion
BUT these antibodies only occur in up to 60% of patients with SLE
Would not do this test if patient has a negative ANA
Anti dsDNA antibodies increase the cardiovascular risk of SLE by altering key molecular processes that drive a distinctive and coordinated immune and vascular activation

Question 22

Q

What is Smith (Sm) antigen/anti-SM?

Answer

A

Highly specific antibody for SLE

Question 23

Q

What is Sjogren syndrome?
* What are the antigens related to this disease? what are they made up of?

Answer

A

Sjogren syndrome is an autoimmune rheumatic disorder characterized by immunologic responses to SS-A and SS-B antigens
The SS-A and SS-B antigen are made up of three different proteins and 4 small RNA particles

Question 24

Q

What antigen can be present in Sjogren’s but can also be in SLE?
* What antigen is found primarily in sjogrens?

Answer

A

SS-A is present in Sjogren’s but can also be present in SLE, sclerosis, inflammatory myopathies
SS-B are found primarily in patients with Sjogrens

Question 25

Q

RNP
* What is this?
* When does antibodies to RNP occur?

Answer

A

A small nuclear protein
Antibodies to RNP occur in approximately 50% of patients with Lupus and in patients with connective tissues diseases- specifically mixed connective tissue disease

Question 26

Q

What is mixed connective tissue disease characterized by?
How it diagnosed?

Answer

A

Mixed connective tissue disease is characterized by high levels of RNP antibodies without detectable SM or ds DNA antibodies

What is a mixed connective tissue disease?
* Diagnosed with positive RNP and there are features of at least two connective tissue disease, including systemic lupus erythematosus, systemic sclerosis, polymyositis, dermatomyositis, and RA

Question 27

Q

Serum protein electrophoresis (SPEP)
* Used to identify patients with what?
* Electrophoresis separates proteins based on what?
* The serum is placed on what?
* The pattern of serum protein electrophoresis results depends on what?

Answer

A

Use to identify patient with multiple myeloma and other serum protein disorders
Electrophoresis separates proteins based on their physical properties and the subsets of these proteins are used in interpreting the results
The serum is placed on a specific medium and a charge is applied
The pattern of serum protein electrophoresis results depends on the fraction of two major types of protein- albumin and globulins

Question 28

Q

HLA-B27
* What is HLA?
* Where are they located?
* Corrlation between HLA B27 and what? Can also cause what?
* The allele strongly contributes to the susceptibility of development of what?

Question 29

Q

Immune Deficiency
* Laboratory tests that measure different parts of what?
* What is the basic panel of immune function tests?

Answer

A

Laboratory tests that measure different parts of the immune system are important for diagnosis someone with primary immunodeficiency and determining which of the over 450 different conditions they may have
Basic panel of immune function tests: CRP, IgM/IgG, IgA, ANA, total protein

Question 30

Q

Most common laboratory tests used to evaluate immune disorders are looking to identify what? (4)

Answer

A

Antibody deficiencies
Cellular (T cell) defects
Neutrophil disorders
Complement deficiencies

Question 31

Q

What do they do to test specific antibody production?
It is worth noting that during the maturation of the immune system, what happens to the response to carbohydrate antigen vaccines?

Answer

A

To test specific antibody production, the person is immunized with common vaccines, and blood samples are obtained immediately prior to and approximately four weeks after the immunization to evaluate how well the individual forms specific antibodies.
It is worth noting that during the maturation of the immune system, the response to carbohydrate antigen vaccines lags behind the response to protein antigen vaccines. This is the reason for having a childhood version of the pneumococcal vaccine, the PCV13, which makes it easier for infants to respond toStreptococcus pneumoniae.

Question 32

Q

Vaccines provide protection by inducing what?
What induces long lasting protection against encapsulated bacteria even amongst persons in high risk groups?

Answer

A

Vaccines provide protection by inducing humoral and/or cellular immunity to the pathogens causing disease. With respect to humoral immunity, the dense surface distribution of often unique glycan structures on diverse pathogens and on malignant cells makes carbohydrates attractive vaccine targets
It is now well known that immunization with neoglycoconjugates composed of capsular polysaccharide-derived glycans covalently coupled to an immunogenic protein carrier(conjugate vaccines)induces long lasting protection against encapsulated bacteria even amongst persons in high risk groups

Question 33

Q

Titer:
* What are antibody serology tests? Can also be used for what?
* Can be elevated from what?
* Typically used to evaluate immunity against what?
* Recently have been used to evaluate what?
* They are not used to diagnose disease, but can show what?

Question 34

Q

Why do you need to have a titer drawn? (4)

Answer

A

To find out if you have had a recent or past infection
Check out vaccination status – If your medical records are incomplete and you don’t know your vaccination status
To find out if a vaccine is effective - the test shows if your previous vaccine is providing enough protection
Requirements for many jobs and or school admission – to show proof of vaccination

Question 35

Q

Antibody Titer test
* For the test, a patient’s blood sample is what?
* A higher number of dilutions are needed for what?
* The result of an antibody titer is generally presented what? Give an example?

Answer

A

For the test, a patient’s blood sample is diluted serially and incubated with known antigens to determine the presence of antibodies against these antigens. The number of dilutions to be prepared depends on the concentration of antibodies in the blood.
A higher number of dilutions are needed for a sample wherein the antibody concentration is very high. The test measures the highest dilution of the blood at which antibodies cannot be detected anymore
The result of an antibody titer is generally presented as a ratio. For example, a ratio of 1:200 indicates that diluting one part of the blood sample to 200 parts of the diluent solution (saline) finally leads to an undetectable antibody level in the blood sample

Question 36

Q

Antibody Titer test
* A successful antibody titer result depends on what? What is an example?

Answer

A

A successful antibody titer result depends on the type of antibody being detected.
* immunoglobulin M (IgM) appears in the blood between 2 – 4 weeks post-infection,
* whereas immunoglobulin G (IgG) takes around 4 – 6 weeks

Question 37

Q

IgM antibodies are what?
* What do do they provide?
* Where are they found?

Answer

A

IgM antibodies are the first immunoglobulins your body makes after you’re exposed to germs. They provide short-term protection while your body makes other antibodies. IgM antibodies are in your blood and lymph fluid

Question 38

Q

What are IgG? What do they provide?

Answer

A

IgGantibodies are very important for fighting infections from bacteria and viruses. Most of the immunoglobulins in your blood are IgG. You also have some IgG antibodies in all your body fluids. Your body keeps a “blueprint” of all the IgG antibodies you have made. That way, if you’re exposed to the same germs again, your immune system can quickly make more antibodies.

Question 39

Q

What do IgA antibodies protect?

Answer

A

IgAantibodies protect your respiratory tract and your digestive system from infections. You have IgA antibodies in your blood, saliva, and gastric juices.

Question 40

Q

What measures the amounts of IgM, IgG, and IgA in your blood to help diagnose different types of diseases ?

Answer

A

An immunoglobulins blood test

Question 41

Q

What are the levels of IgM and IGG during infection?

Question 42

Q

What are the IgM/IgG primary vs secondary response curves?

Question 43

Q

Antibody Deficiency Tests
* The standard screening test for humoral immune function starts with measuring what?
* There are also tests to measure the different types of what?
* B cells may be absent in certain what?

Answer

A

The standard screening test for humoral immune function starts with measuring immunoglobulin levels in the blood. These consist of IgG, IgA, and IgM….sometimes IgE
There are also tests to measure the different types of lymphocytes in the blood, specifically B cells which produce antibodies
* B cells may be absent in certain antibody deficiencies such as X-linked agammaglobulinemia (XLA)

Question 44

Q

Cellular T cell immunity- determines what?
* First time this is tested when?
* Can be checked with that?
* Can also be looked at within what?

Answer

A

Cellular T cell immunity- determines the numbers and different types of T cells
* First time this is tested is at birth as part of the newborn screening tests
* Can be checked with flow cytometry
* Can also be looked at within the CBC with diff as the absolute lymphocyte count (75% of circulating lymphocytes are T cells)

Question 45

Q

IgA deficiency
* What is it? What is it caused by?

Answer

A

Most common primary immunodeficiency characterized by undetectable serum IgA
* Have no common mendelian pattern, can also be caused by medications, certain viruses

Question 46

Q

IgA deficiency
* When does this occurs?
* Sxs?
* What do recent studies show?
* Others are at risk for developing what? Blood should be given with

Question 47

Q

IgG deficiency
* This class of antibodies is composed of what?
* What does each subclass present in? How much is in blood (each subtype and age?

Answer

A

This class of antibodies is composed of four different subclasses: IgG1, IgG2, IgG3, and IgG4.
* Each subclass is present in different concentrations in the blood and those concentrations vary with age.
* The IgG in the bloodstream is 60-70% IgG1, 20-30% IgG2, 5-8% IgG3, and 1-3% IgG4. IgG1 and IgG3 reach normal adult levels by 5-7 years of age, while IgG2 and IgG4 levels rise more slowly, reaching adult levels at about ten years of age.

Question 48

Q

IgG deficiency
* Each subclass serves predominantly (but not exclusively) a slightly different function in what? What is an example?
* In contrast, IgG2 predominantly protects against what?

Answer

A

Each subclass serves predominantly (but not exclusively) a slightly different function in protecting the body against infection. For example, IgG1 and IgG3 subclasses protect against toxins from bacteria such as diphtheria and tetanus, and against viral infections.
In contrast, IgG2 predominantly protects against the polysaccharide (complex sugar) capsule of certain disease-causing bacteria such asStreptococcus pneumoniaeandHaemophilus influenzae.

Question 49

Q

Individuals with IgG subclass deficiency are defined as having what?
* What subclass is mc? what does it result in?

Answer

A

Individuals with IgG subclass deficiency are defined as having recurrent infections, persistently low levels of one or more IgG subclasses, and normal concentrations of total IgG, IgM, and IgA.
* IgG2 or IgG3 deficiencies are the most common IgG subclass deficiencies. Since IgG1 comprises 60% of the total IgG level, having a deficiency of IgG1 usually drops the total IgG level below the normal range, resulting in hypogammaglobulinemia.
* IgG2 or IgG3 deficiencies would show a normal total IgG since IgG1 is the most common in blood

Question 50

Q

IgG deficiency
* What should be measured?
* An abnormal level should be confirmed how?
* What is txt?
* What happens when a child is dx with IgG subclass deficiency?

Question 51

Q

IgM deficiency
* IgM is what? What does is contain? What does it serve as?
* It is predominantly present in what?
* IgM is the first immunoglobulin secreted during what?

Answer

A

IgM is the largest in size of all immunoglobulins - it contains five antibody molecules held together by the J chain. IgM antibodies serve as a first line of defense against bacteria, viruses, and fungi and in protection against autoimmune diseases and inflammation.
It is predominantly present in the circulating blood versus IgG, which is present both in the circulation and in the tissues, and IgA, which is predominantly in secretions.
IgM is the first immunoglobulin secreted during an initial exposure to infectious organisms or a vaccine. IgM is also different from other immunoglobulins in that

Question 52

Q

IgM deficiency
* Although described more than 50 years ago, it is only recently that selective IgM deficiency has been included in what?
* It is characterized by what?
* What is more common?

Answer

A

Although described more than 50 years ago, it is only recently that selective IgM deficiency has been included in the list of forms of PI.
It is characterized by low (partial) to absent IgM (complete) in the blood but normal IgG and IgA levels. It occurs equally in children and adults and in both men and women.
Partial selective IgM deficiency is more common than previously realized, whereas complete selective IgM deficiency is rare.

Question 53

Q

IgM deficiency
* Individuals with selective IgM deficiency may be what?
* Among infections, the most common are what?
* Almost 40% of individuals with selective IgM deficiency have what?

Answer

A

Individuals with selective IgM deficiency may be asymptomatic or symptomatic. Of those who are symptomatic, approximately 80% present with predominant bacterial infections.
* Among infections, the most common are chronic sinusitis, upper respiratory tract infections, bronchitis, and pneumonia.

Almost 40% of individuals with selective IgM deficiency have allergic diseases, including hay fever and asthma.

Question 54

Q

IgM deficiency:
* Autoimmune diseases are observed in about how many people?
* Autoimmune diseases are more common in who? What is an example?

Answer

A

Almost 40% of individuals with selective IgM deficiency have allergic diseases, including hay fever and asthma.

Autoimmune diseases are observed in about one-third of all individuals with selective IgM deficiency. Autoimmune diseases are more common in adults than children with selective IgM deficiency.
* Example: SLE, RA, and autoimmune thrombocytopenia, Hashimoto’s thyroiditis and Addison’s disease

Question 55

Q

The diagnosis of partial selective IgM deficiency is made (3)?

Answer

A

if the serum IgM level is below two standard deviations of the mean for age-matched controls.
in children, should only be established after months of follow-up.
To confirm a diagnosis of primary selective IgM deficiency, other possible causes of IgM deficiency must be excluded, such as leukemia, lymphoma, and immune suppressive medications, such as Clozapine.

Question 56

Q

Neutrophils
* What do neutrophil play an essential role in what?
* What is normal?
* Failure to carry out that role leads to what?

Answer

A

Neutrophils play an essential role in immune defenses because they ingest, kill, and digest invading microorganisms – first line of cells recruited at the site of infection
* Normal WBC count 4000-11,000 cells/microL, out of which 60-70% are mature neutrophils circulating in the blood
* Failure to carry out that role leads to immunodeficiency characterized by the presence of recurrent infections

Question 57

Q

Neutrophils
* Defects can be what?
* Can obtain what?
* Much look at what to r/o certain diseases? Sometimes requires what? Assess patients with what?

Answer

A

Defects can be quantitative- neutropenia or qualitative- neutrophil dysfunction
Can obtain a CBC with differential that shows the absolute neutrophil count
Must look at a blood smear to rule out certain diseases that are associated with abnormalities in the structure of neutrophils
* Sometimes requires a bone marrow biopsy to see if neutrophils are being made properly
* Assess in patients with lymphoma undergoing chemotherapy

Question 58

Q

Neutropenia is serious life threatening disorder with what?

Answer

A

with high morbidity and mortality

Question 59

Q

How do diagnose severity of neutropenia?

Answer

A

Just know that we can calcuate it

Question 60

Q

Neutrophilia
* What is this?
* Absolute neutrophil count (ANC) typically ranges between what?
* Neutrophilia is defined as an absolute neutrophil count greater than what?

Answer

A

Neutrophilia is defined as a higher neutrophil count in the blood than the normal reference range of absolute neutrophil count
* Absolute neutrophil count (ANC) typically ranges between 2500 to 7000 neutrophils/microL
* Neutrophilia is defined as an absolute neutrophil count greater than 7000 neutrophils/microL

Question 61

Q

Neutrophilia can be seen in what?

Answer

A

infections, inflammation, and/or neoplastic processe

Question 62

Q

Neutrophilia / ”Left Shift” (bandemia)
* Normally, there is a balance between different types of what?
* What happens in left shift?

Answer

A

Normally, there is a balance between different types of white blood cells, including neutrophils, lymphocytes, monocytes, eosinophils, and basophils. Neutrophils are the most abundant type of white blood cell and play a crucial role in the body’s defense against infections.
In a left shift, there is an increase in the number of immature or “band” neutrophils circulating in the bloodstream. These immature neutrophils are released prematurely from the bone marrow in response to an increased demand for white blood cells, typically due to an acute infection or inflammation.

The term “left shift” originates from the graphical representation of the CBC results, where the different types of white blood cells are arranged from left to right based on their maturity.

Question 63

Q

What does bandemia show?

Answer

A

Acute and serious infections
* start antibiotics

Question 64

Q

Complement deficiencies
* The standard screening test for deficiencies in the classical complement pathway is the what? ⭐️
* Specialized complement laboratories can provide what?

Answer

A

The standard screening test for deficiencies in the classical complement pathway is the total hemolytic complement assay or CH50
Specialized complement laboratories can provide additional testing that will identify the specific complement component that is defective

Answer 58

A

Decreased levels of complement arise from immune-complex disorders such as SLE and other selected forms of vasculitis
NOT useful for screening of SLE, but it often used to monitor disease activity in patients with SLE

Answer 59

A

the bone marrow of the skull, ribs, sternum, vertebral column, pelvis, and femurs

Answer 60

A

Lymphocytes represent around 20-40% of WBC’s
Relative lymphocytosis is an increase in WBC’s of more than 40% in the presence of a normal absolute white cell count

Answer 61

A

Low white blood cell count
* Primary Lymphopenia: is rare and often results from genetic or developmental abnormalities that affect the production, function, or survival of lymphocytes. Examples include severe combined immunodeficiency (SCID), DiGeorge syndrome, and other primary immunodeficiency disorders.
* Secondary Lymphopenia: Secondary lymphopenia is more common and can be caused by a variety of factors

Answer 62

A

Infections: Viral infections (e.g., HIV, Epstein-Barr virus, cytomegalovirus) can directly affect lymphocyte production and function.
Medications: Certain medications, such as corticosteroids, chemotherapy drugs, immunosuppressants, and antiretroviral therapy, can suppress lymphocyte production or induce cell death.
Autoimmune Disorders: Autoimmune diseases, such as systemic lupus erythematosus (SLE) and rheumatoid arthritis, can lead to lymphocyte destruction or dysfunction.
Hematologic Disorders: Conditions affecting the bone marrow, such as aplastic anemia, myelodysplastic syndromes (MDS), and leukemia, can impair lymphocyte production.
Radiation Therapy: Exposure to ionizing radiation, either as medical treatment or environmental exposure, can damage lymphocytes and suppress immune function.

Answer 63

A

High white count
Need to distinguish between reactive and malignant
Is defined as an absolute lymphocyte count over 4000 cells/microL
Lymphocytosis is detected incidentally in the majority of the patients.
The evaluationstarts with an extensivehistory and physical and review of peripheral blood smear review, which may direct for the need for further testing (flow cytometry, karyotype, and FISH).

Answer 64

A

Clues to the presence of clonal lymphoproliferative disorders include a highly elevated ALC, presence of abnormal lymphocyte forms on PBS, and the presence of other cytopenias.

Answer 65

A

EBV, CMV, Acute HIV infection (chronic HIV causes lymphopenia), influenza, measles and mumps, adenovirus

Answer 66

A

Bartonella henselae – leads to cat scratch disease – lymphocytes are large and atypical
Bordetella Pertussis – increased lymphocytes that are small with a deeply cleaved nucleus
Brucellosis, syphilis, malaria

Answer 67

A

Toxoplasma Gondii- lymphocytosis with atypical lymphocytes is a hematologic hallmark of the disease

Answer 68

A

Mycobacterial tuberculosis

Answer 69

A

Know the written part

Answer 70

A

know the written

Answer 71

A

Chronic lymphatic leukemia
Chronic myeloid leukemia
Acute lymphatic leukemia
Acute myeloid leukemia

Answer 72

A

Blood cells start as stem cells and then develop into specific types of white blood cells called lymphocytes
* Lymphocytes are made in the bone marrow
* become B cells (make antibodies) and T cells (attack and destroy bacteria, viruses, pathogens)
* Can also be found in blood and lymph tissue

Answer 73

A

Lymph nodes are composed of lymphocytes
* Found in clusters around the body
* Become swollen or tender if you have an infection or malignancy

Answer 74

A

Bood cancers occur when healthy blood cells start dividing, growing or spreading abnormally

Answer 75

A

Leukemia
* typically develops in lymphocytes but can also develop in myeloid cells
* Leukemia cells can spread throughout the body but primarily affect the bone marrow and blood
* Some are acute (grow quickly), others are chronic (grow slowly)

Lymphoma
* Develops in lymphocytes, however lymphoma usually affects lymph nodes and other organs instead of bone marrow
* Separated into Hodgkin vs non-Hodgkin

Answer 76

A

CLL is the most common leukemia in adult patients in the USA
Most often in people over age 55
Lymphocytes are over 5000 cells/microL and are typically small mature looking with dense nuclei and compact chromatin, also called “soccer ball” cells
Numerous smudge cells on peripheral blood smear

several genetic and chromosomal lesions paly a role in the malignant development of CLL B lymphocytes, in addition to antigens that could play a role in malignant cell selection.

Answer 77

A

It is characterized by the uncontrolled proliferation of myeloid cells, particularly granulocytes (neutrophils, eosinophils, and basophils), in the bone marrow
The majority of cases of CML are associated with a specific genetic abnormality known as the Philadelphia chromosome. This abnormality results from a translocation (exchange of genetic material) between chromosomes 9 and 22, leading to the formation of a fusion gene called BCR-ABL1. The BCR-ABL1 fusion gene produces a mutant protein with abnormal tyrosine kinase activity, which plays a central role in the development and progression of CML.

Answer 78

A

The diagnosis of CML is typically based on a combination of clinical evaluation, blood tests (complete blood count with differential), bone marrow biopsy, and genetic testing to detect the presence of the Philadelphia chromosome or BCR-ABL1 fusion gene. The presence of the Philadelphia chromosome or BCR-ABL1 fusion gene is considered diagnostic of CML.
Mainly affects adults

Answer 79

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Associated with increased lymphoblasts rather than more mature lymphocytes

Acute lymphoblastic leukemia is classified based on the type of lymphocytes affected and the stage of maturation of the leukemic cells. The two main subtypes of ALL are:
* B-cell ALL: The majority of cases of ALL (about 85%) involve abnormal proliferation of immature B-cell lymphoblasts.
* T-cell ALL: In this subtype, the leukemic cells are derived from abnormal proliferation of immature T-cell lymphoblasts.

Answer 80

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Acute lymphoblastic leukemia is the most common type of leukemia in children, accounting for approximately 75-80% of pediatric leukemia cases. It is less common in adults but can still occur. The peak incidence of ALL occurs in children between 2 and 5 years of age

Answer 81

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Diagnosis: CBC with differential, bone marrow biopsy, genetic testing for gene mutations associated with ALL, flow cytometry (analyze the immunophenotype of the leukemic cells to distinguish between B-cell and T-cell ALL)

Answer 82

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Affects blood cells called myeloid stem cells (myeloid can become one of 3 types of mature blood cells- RBC, WBC, platelets)
Rapid abnormal proliferation and differentiation of these immature myeloid cells, called blasts, happens in the bone marrow
In AML, the proliferation of immature myeloid blasts disrupts the normal production of these blood cell types so healthy cells can’t do their job

Answer 83

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The incidence of AML increases with age, with the highest rates observed in individuals over 65 years old
* BUT is second most common childhood leukemia after ALL

Auer rod – a distinctive cytoplasmic inclusion found in AML
* Abnormal structures formed from components of the cells cytoplasmic granules
* Can also be seen in acute promyelocytic leukemia (that’s too far in detail!)

Answer 84

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Several different types of non-Hodgkin lymphoma
* Some start in B cells and others start in T cells
* Is indolent or slow growing and slow spreading OR it can be aggressive

Answer 85

A

Small mature looking lymphocytes and “smudge cells” in CLL and MBL
Atypical large lymphocytes are visible in EBV and other viral infections such as CMV or early HIV
Lymphocytes that are cleaved angulated, or have indented nuclei can be associated with pertussis or malignancies such as follicular lymphoma.

Answer 86

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“Hairy cells” with regular cytoplasmic projections are seen in hairy cell leukemia
Sezary Cells have cribriform nuclei with compact chromatin
“Villous” lymphocytes are seen in MZL
Large lymphocytes with multiple azurophilic granules are present in T-LGL
Lymphoblasts in ALL

Answer 87

A

Flow cytometry
Fluorescence in situ hybridization (FISH)

Answer 88

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Powerful laboratory technique used to analyze and quantify various characteristics of cells, including their size, complexity, and protein expression, at the single-cell level
Cells of interest are collected from a biological sample, such as blood, bone marrow, tissue, or cell culture. These cells may be labeled with fluorescent dyes or antibodies targeting specific cell surface markers or intracellular proteins of interest

Answer 89

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The prepared cell sample is introduced into a flow cytometer, a sophisticated instrument equipped with lasers, optics, detectors, and fluidics systems. The flow cytometer can analyze thousands of cells per second as they pass through a narrow, focused stream of fluid, called the sheath fluid, within the instrument.
Laser Excitation: As each cell passes through the flow cytometer’s laser beam, it is illuminated by one or more lasers of specific wavelengths. The laser light causes the fluorescent labels on the cells to emit light at characteristic wavelengths, which is then detected by photomultiplier tubes or other detectors.

Answer 90

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Flow cytometry software enables researchers to identify different cell populations based on their unique characteristics, quantify the expression levels of specific proteins or markers, and perform complex analyses, such as cell cycle analysis or apoptosis assays.

Answer 91

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Is a cytogenetic technique used to detect and analyze specific DNA sequences or chromosomal abnormalities in cells. Can visualize and quantify genetic alterations at the single-cell level.

Answer 92

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fluorescently labeled DNA or RNA probes are designed to specifically hybridize (bind) to complementary target sequences within the cell’s genome. These probes can be designed to target specific genes, chromosomal regions, or DNA sequences of interest.
The sample containing cells of interest, typically obtained from blood, bone marrow, tissue biopsy, or cytological specimens, is first fixed onto a glass slide to preserve cellular morphology. The cells are then treated with chemicals or heat to denature (unwind) the DNA, allowing the probes to bind to their complementary target sequences
Excess unbound probes are washed away, leaving only the fluorescently labeled probes bound to their target sequences within the cells. The slide is then examined under a fluorescence microscope equipped with filters that can detect the fluorescence emitted by the bound probes.
The fluorescence signals emitted by the bound probes are visualized and captured using imaging software. The number, location, and intensity of the fluorescence signals provide information about the presence and characteristics of specific genetic abnormalities or chromosomal rearrangements within the cells
*

Answer 93

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FISH can detect numerical abnormalities (aneuploidy) and structural abnormalities (translocations, deletions, duplications) in chromosomes associated with various genetic disorders, cancer, and developmental abnormalities.
Prenatal Testing: FISH can be used for prenatal diagnosis of chromosomal abnormalities, such as trisomy 21 (Down syndrome), trisomy 18 (Edwards syndrome), and trisomy 13 (Patau syndrome), by analyzing fetal cells obtained from amniotic fluid or chorionic villus sampling

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