Pathology Final Exam

Front 1

when was AIDS first recognized as a disease

Back 1

1981

Front 2

true or false AIDS related deaths per year have been decling since 1995

Back 2

TRUE
meaning that there is an enlarging pool of persons with HIV infection and AIDS

Front 3

true or false the infection rate for HIV is also declining

Back 3

FALSE the number of new HIV infections/yr remains stable

Front 4

true or false the number of new HIV infections/yr remains stable and the number of AIDS cases/yr decreases

Back 4

True!
due to treatment with antivirals

Front 5

rates of HIV infection are skewed in minorites and are escalating. what % do minority women and children with AIDS make up

Back 5

>80% if AIDS in women were minority and children

Front 6

what is the leading mode of transmission of AIDS worldwide

Back 6

heterosexual sex

Front 7

what is the risk of HIV infection from a needle stick without post exposure treatment

Back 7

0.3-0.4%

Front 8

what is the post exposure treatment after a needle stick to prevent contraction of HIV

Back 8

dual or triple agent therapy is recommended for 4 wks this is called "post exposure prophylaxis"

Front 9

what is the risk of transmission of HIV from infected mother to infant if mother is not being treated compared to mother that is being given AZT and with combination therapy

Back 9

no treatment 25-30%
with AZT alone 8%
with combination therapy 0-2%

Front 10

when does transmission occur from mother to child

Back 10

in utero (antepartum) 30%
at delivery (intrapartum, peripartum) 70% or via breast feeding (postpartum)

Front 11

true or false: there are NO known from urine, stool or tears

Back 11

true

Front 12

true or false persons 13-64 don't need to have HIV screening as part of their routine yearly medical care.

Back 12

false
it does not need to be yearly but should be part of their routine medical care

Front 13

what type of virus is the HIV

Back 13

RNA virus single stranded bilipid membrane

Front 14

what are the 3 structural genes of HIV

Back 14

gag gene: encodes for proteins like p24 (core protein) used as a marker for viral replication

Front 15

I am the "core protein" inner shelf of nucleocapsid, clinically used as a marker for viral replication

Back 15

p24

Front 16

what major envelope glycoprotein mediates attachment to CD4 receptora and coreceptors

Back 16

gp120

Front 17

what major envelope glycoprotein mediates helps mediate fusion of the virus to the host cell

Back 17

gp41

Front 18

what envelope gene encodes for enzymes

Back 18

pol gene

Front 19

what enzymes do the pol gene encode for

Back 19

reverse transcriptase, integrase, protease

Front 20

this test is a screening test, detects antibodies, is reported as reactive or non reactive

Back 20

ELISA
if test is neg. = no further testing if reactive ELISA is repeated

Front 21

this test for HIV is known as the confiramtory test and detects antibodies fro 3 key bands. what test is this and what do the key bands test for

Back 21

western blot : after repeat ELISA is still reactive
key bands: gp120/160 and gp41 and p24

Front 22

if the western blot test shows no key bands what does this mean

Back 22

negative

Front 23

western blot test shows 2 of 3 key bands

Back 23

positive

Front 24

what is an indeterminate Western blot test

Back 24

if 1 key band is detected and or accessory bands found

Front 25

what is the rapid HIV screening test

Back 25

1. uses ELISA technology
2. turnaround time is ~30 mins
3. although it is very sensitive and sp. it requires confirmation

Front 26

what is the window period

Back 26

early HIV infection will show up as 1 key band and or accessory band

Front 27

which HIV test is not routinely used for diagnosis of HIV

Back 27

HIV antigen test performed primarily to determine viral load also can be used to diagnose early infection (window period)

Front 28

what are the symptoms of acute HIV infection

Back 28

flu-like illness: fever, fatigue, nausea, pharyngitis, wt. loss, RASH (2/3 get)
-ELISA and western blot may be neg.
-diagnosis would need to be made by HIV antigen test

Front 29

what is the CDC definition of AIDS

Back 29

1. CD4 count < 200/ml with a positive HIV test
2. HIV pos. with an AIDS indicator condition, regardless of CD4

Front 30

on average what is the time span btw HIV infection and dev. of AIDS

Back 30

10 YRS

Front 31

What makes AIDS un-curable

Back 31

the latent infected CD4 cells

Front 32

what determines how sick you get with AIDS and how is it measured

Back 32

the viral load measured by PCR or bDNA
they measure HIV RNA in plasma
undetectable viral load < 50c/ml

Front 33

what is the effect of HAART on CD4 and viral load. what is HAART

Back 33

CD4 increases and viral load dec.
HAART: Highly Active Antiretroviral Therapy, combination therapy at least 3

Front 34

what is used to assessed if antiviral therapy should be initiated

Back 34

-low CD4 count (indicates immune def./ advanced dz or
- high viral loads means dz will advance rapidly

Front 35

what is the most common opportunistic infection in the HIV population

Back 35

tuberculosis
HIV accelerates TB and TB accelerates HIV
ALWAYS do PPD for TB testing in HIV patients.

Front 36

this is the most opportunistic herpes family virus, and may cause blindness

Back 36

cytomegalovirus (CMV)

Front 37

this common AIDS related illness presents as multifocal vascular lesion classified as malignant neoplasm

Back 37

Kaposi Sarcoma may be caused by herpes virus type 8

Front 38

this common AIDS related illness presents as brain lesions in HIV patients

Back 38

Toxoplasmosis

Front 39

This common AIDS related illness is common in AIDS patients when CD4 counts drops below 50

Back 39

mycobacterium avium intracellulare complex

Front 40

this common AIDS related illness presents as headache and fever with minor neck stiffness and mental status changes

Back 40

cryptococcal meningitis

Front 41

what is the HALLMARK of immunodeficienc

Back 41

unusual and recurrent infections

Front 42

true or false: secondary immune deficiency disorders are rare

Back 42

false secondary immune def. are common
the deficiency is the result of an underlying dz or other factor

Front 43

I may be hereditary or acquired the deficiency is the cause of the disease

Back 43

primary immune deficiency disorders

Front 44

what characteristics differentiate X-linked infantile agammaglobulinemia

Back 44

ONLY in males, NO mature B-cells, defective B-cell tyrosine Kinase gene (btk gene, X-LA)
- dramatic dec. in all 5 antibody isotypes

Front 45

this primary immunodeficiency you will see very small tonsils, and you will see frequent infections after 6-9 mos (begin to appear when maternal IgG in infants falls below a protective level.) You will also see a high incidence of dental caries and early onset of periodontal disease

Back 45

X-linked infantile agammaglobulinemia (X-LA, Bruton disease)

Front 46

In this primary immuno deficiency there is a delay in the ablility of B cells to prod IgG
result: is low IgG and normal IgM and IgA levels
due to: lack of help from CD4 T-helper cells

Back 46

transient hypogammaglobulinemia

Front 47

In this primary immuno deficiency:
B-cells are present but they do NOT differentiate into mature B cells:
-do not divide and do NOT secrete antibody do divide but still do NOT secrete antibody
common variable: no antibody secretion
with this immunodeficiency there is a high freq. of autoimmune disease and malignancy

Back 47

COMMON VARIABLE hypogammaglobulinemia

Front 48

what is the most common selective immunoglobulin deficiency? and what is characteristic of this disease

Back 48

IgA deficiency = failure in terminal differentiation of IgA producing B cells
will commonly see sinopulmonary infections, chronic diarrhea, and aurtoimmune disease is common with this deficiency

Front 49

What selective immunoglobulin deficiency is very rare

Back 49

IgG subclass deficiency = failure in terminal differentiation of IgG sublass producing B cells

Front 50

What is occuring in the selective immunoglobulin deficiency with high IgM (HIGM)

Back 50

this selective immunoglobulin deficiency B-cells cannot switch from IgM to IgG, IgA or IgE production. Thus you will have high IgM and low or absent other antibody isotypes

Front 51

what caused the decrease in T cells in DiGeorge syndrome

Back 51

DiGeorge syndrome= congenital Thymic Aplasia
Aplasia = defective development or congenital absence
in this disease there is interference in the dev. of pharyngeal pouches 3 and 4th during the 1st trimester
= no thymus develops = no T-cells and no parathyroid gland
results: hypocalcemia, facial heart and blood vessel malformations

Front 52

In this immunodeficiency there is chronic infection of skin and mucus membranes with candida (especially C albicans)

Back 52

chronic mucocutaneous candidiasis : inability of T-cells to respond to candida antigens

Front 53

this immunodeficiency is due to developmental arest of precursor cells at A VERY EARLY stage. this effects B and T lymphocytes and granulocytes.
RESULT: rapidly fatal disease due to early intractable fungal infection of mucosal surfaces (diarrhea, pneumonias, viral infections)

Back 53

Reticular dysgenesis

Front 54

this immunodeficiency is due to defective lymphoid stem cells = NO T or B cells
characterized by:
-defective gene that codes for gamma chain of IL2 receptor
-bare lymphocyte syndrom
-adenosine deaminase (ADA) enzyme deficiency
Clinically:
-born with a skin rash
-extreme susceptibility to all infectious agents

Back 54

severe combined immunodeficiency disease
(SCID)

Front 55

this immunodeficiency is X-linked and seen only in males
-initial T-lymphocyte count may be normal, but declines with time, protection is low
-IgM level is low due to a high catabolism rate
Clinically:
-thrombocytopenia, abnormal platelets, bleeding and eczema

Back 55

Wiskott-Aldrich syndrome
(WAS)

Front 56

this Immunodeficiency is a progressive disease that is due to defective DNA repair mechanisms
results in breaks in T cell antigen receptor genes and heavy chain genes of antibody
Clinically;
*IgA is often low
*high incidence of malignancies and susceptibility to radiation
-spider like vascular dilation
-severe cerebellar ataxia

Back 56

Ataxia-telangiectasia (AT)

Front 57

this is a deficiency in NADPH oxidase (needed to release chemicals from immune cells, crucial for phagocytosis)
Results: granulomatous abscesses in lymph nodes, lungs and liver and greatly increase susceptibilty to opportunistic bacterial infections

Back 57

Chronic granulomatous Disease

Front 58

what will result if you have a deficiency in early complement componenets (C1,C2, C4)

Back 58

high incidence of autoimmune disease

Front 59

what will result if you have a C3 deficiency

Back 59

increased incidence of autoimmune disease and inc. susceptibility to bacterial infections

Front 60

what will result if you have a deficiency in late complement components (C5, C6, C7, C8, C9)

Back 60

Infection with Nisseria are the most common
*** gram neg. bacteria are most common microbes when there is C3, C5-9

Front 61

in this abnormality of the complement system there is a deficiency of C1 esterase inhibitor
name this abnormality and describe what results from the C1 esterase inhibitor def

Back 61

this inhibitor down regulates C1qrs so a deficiency results in:
uncontrolled C1qrs and increased kinin related proteins = inc. vascular permeability = recurrent attacks of swelling especially skin and mucus membranes

Front 62

true or false primary immunodeficiencies are more common than secondary immunodeficiencies

Back 62

FALSE
secondary immunodeficiencies are more common
remember the deficiency is the result of an underlying dz or other factor

Front 63

name the underlying causes of secondary immunodeficiencies

Back 63

1. poor nutrition: most common cause in dev. countries
2. immunosuppressive druges: most common cause in the USA
3. protein loss: hypogammaglobulinemia
4. infections
5. other: diabetes, liver dz, alcohol abuse, uremia, anesthetic agents ,radiation, lymphoproliferative, splenectomy

Front 64

where are RBC's made

Back 64

bone marrow

Front 65

what is the function of a RBC

Back 65

O2 carrying capacity

Front 66

what is the diameter of a RBC and what is the typical shape

Back 66

7.5 um in diameter and biconcave disc shape

Front 67

what is the typical life span of a RBC

Back 67

120 days

Front 68

what is the difference btw medullary and extramedullary hematopoiesis

Back 68

medullay hematopoiesis: prod. of RBC's that takes place in bone marrow
extramedullary hematopoiesis: prod of RBC's outside of the bone marrow = liver and spleen

Front 69

I am a reduction in the normal total circulating red blood cell mass OR the reduction in the amount/quality of hemoglobin in a RBC's

Back 69

Anemia

Front 70

what are the causes of anemia

Back 70

1. blood loss
2. inc. RBC destruction
3. Dec in RBC prod.

Front 71

blood loss is a cause of anemia what is the diff. btw an acute blood loss and a chronic

Back 71

acute is due to trauma while chronic is loos of blood over prolonged period of time ex. due to ulcers

Front 72

increased RBC destruction is a cause of anemia. what are some causes of increased RBC destruction

Back 72

1. Infection of RBC: malaria, hemolysis, sickle cell
2. Sequestration of RBC's
3. Genetic defects in RBC prod
4. RBC membrane disorders: Hereditary spherocytosis, hereditary elliptocytosis
5. hemoglobinopathies: genetic defects that results in abnormal structure of 1 of the globin chains of hemoglobin = speen taking RBC's out of circulation
6. drugs

Front 73

dec. RBC prod is a cause of anemia. what may cause a dec. in RBC prod

Back 73

1. nutritional def: Fe and B12
2. erythropoietin def
3. chemical defect
4. immune med injury
5. neoplasm

Front 74

how would you test for anemia

Back 74

1. measure hematocrit: ratio of packed RBC's to total blood vol.
2. hemoglobin: concentration of Hb in circulating RBC's
3. peripheral blood smears: aid in the diagnosis of anemia of one kind or another

Front 75

what microscopic characteristics of anemia

Back 75

1. normochromic normocytic: acute/chronic blood loss
2. hypochromic microcytic: ex. Fe def anemia
3. hyperchromic macrocytic: ex. B12 anemia

Front 76

what are the normal lab values of hemoglobin and hematocrit from men and women. why do men gen. have a higher value than women

Back 76

Men:
hemoblobin (g/dl): 13.6-17.2
heatocrit (%): 39-49

Women:
hemoglobin: 12-15
hematocrit: 33-43
usually hematocrit is 3X's hemoglobin
men's is higher b/c they have more muscle and don't menstruate

Front 77

what are the signs and symptoms associated with anemia

Back 77

symptoms (what the patient feels): fatigue, weakness, rapid heart rate, SOB (shortness of breath), feeling light headed
signs: Rapid heart rate, pallor, chest pain, dark tarry stools, heart attack, tachypnea, cold skin, hypotension, jaundice, SPLENOMEGALY

Front 78

where can greater than 80% of Fe stores can be found where and where is the remainder found

Back 78

80% of Fe stores can be found in hemoglobin, myoglobin and other iron containing enzymes and proteins (metalloproteins, NADH dehydrogenase, iron sulfur proteins)
remainder: is found in form of ferritin and hemosiderin

Front 79

what are the causes of Fe deficiency anemia

Back 79

1. CHRONIC BLOOD LOSS
2. dietary lack
3. hormonal deficiency: EPO = responsible for RBC prod
4. inability to absorb Fe
5. pregnancy
6. growth spurts: body is playing catch up

5.

Front 80

what is the morphology of iron deficiency anemia

Back 80

1. hypochromic microcytic anemia (less color, sm. size, central area of pallor)
2. low serum iron

Front 81

what are the clinical features of Fe deficiency anemia

Back 81

Plummer vinson syndrome:
-atrophic glossitis (red, smooth beefy tongue)
-hypochromic microcytic anemia
-esophageal webs

Front 82

what would a lab test show in Fe deficiency anemia

Back 82

DECREASE IN:
-MCV (mean cell size
-MCH (mean cell hemoglobin), MCHC (mean cell hematocrit)
Increase:
RBC count = reticulocyte %if RBC's are not carrying around O2 the body compensates by prod. more RBC's

Front 83

how would you treat Fe deficiency anemia

Back 83

duh!
-Fe supplementation
- another first line of treatment = blood transfusion

Front 84

a group of nutritional disease of vitamin B12 or folate def. that result in abnormally LARGE RBC precursors and thus LARGE mature RBC's

Back 84

Megaloblastic anemias

Front 85

I am only found in bacteria and animal sources, while I am only found in leafy vegetables, but we result in the same anemia. who are we and what anemia occurs if we are absent

Back 85

vitamin B12, and folate resulting in megaloblastic anemia

Front 86

what important role do vit B12 and folate play

Back 86

both are important as coenzymes in the formation of thymidine and are necessary in DNA synthesis

Front 87

what is the morphology that we will see in megaloblastic anemia

Back 87

-LARGER than normal RBCs and white cells
-hyperchromic macrocytic anemia
-bone marrow hyperplasia
***Hallmark = hypersegmented anemia***

Front 88

pernicious anemia is a type of B12 def. anemia that is caused by autoimmunity dz. what are the autoantibodies to

Back 88

1. intrinsic factor: protein responsible for shuttling B12 across ileum into the cells
2. parietal cells: intrinsic factor is secreted by parietal cells in the gastric fundus
* therefore B12 is present it cannot be absorbed = megaloblastic anemia dev.

Front 89

what are the clinical signs and symptoms of pernicious anemia

Back 89

Normal microscopic characteristics: fatigue, weakness, rapid HR, SOB, light headed, tacypenea, cold skin, jaundice, splenomegaly
Except:
-gastric atrophy:stomach shrinkage
-achlorhydria: state where the prod. of gastic acid in stomach is absent or low
-atrophic glossitis: red, smooth, beefy tongue

Front 90

what test would you do to diagnose and treat pernicious anemia

Back 90

diagnosis: schillings test "pernicious percy is seaky when trying to steal schillings"
has 2 parts
1st use radiolabeled B12
2nd: use radiolabeled B12 and IF
treatment: IV B12

Front 91

this anemia stems from increase breakdown of RBC's

Back 91

hemolytic anemias due to:
-drugs, autoimmunity,and inherited defects

Front 92

what 3 major characteristics are seen in hemolytic anemias

Back 92

1. ALL associated with inc. in RBC destruction and shortened life span of RBC'S
2. there is elevated EPO: prod in kidney responsible for RBC's prod. it is increased to compensate for loss of RBC's
3. accumulation of hemoglobin degradation prods.

Front 93

I am a autosomal DOMINANT defect that causes RBC's to be less deformable. Due to alteration in spectrin and ankyrin proteins

Back 93

Hereditary spherocytosis

Front 94

what proteins are altered in hereditary spherocytosis that makes RBC's less defomable

Back 94

alteration in spectrin and ankyrin proteins
they become spherical in shape with no central area of pallor (hyperchromic)! without their characteristic shape they will not be able to roll = degrade faster = taken out of circulation by the spleen = life span of RBC dec to 10-20 days and splenomegaly

Front 95

what are the signs/symptoms of hereditary spherocytosis, how would you diagnosis it and what treatment would you do?

Back 95

S/S: mild jaundice due to Hb degredation prod. and splenomegaly due to inc. in the spleen taking RBC's out of circulation
treatment: spleenectomy: will allow the RBC (that still fxn) to stay around for longer. you would also take spleen out so that it will not compromise other structures that it presses on. in children wait till age 5 to take out spleen b/c this is site of RBC prod

Front 96

I am an acquired stem cell (somatic cell) Dz that occurs b/c of a defective/missing pig-A gene that results in breakdown of RBC's and alteration in fxn of all hematopoietic cells.

Back 96

paroxysmal Nocturnal hemoglobinuria

Front 97

what are some defining characteristics of paroxysmal nocturnal hemoglobinuria

Back 97

-acquired mutation in somatic cell prod a mutation on proteins that are responsible for anchorage of certain proteins on RBC's surface that block complement mediated lysis
-with these proteins absent you will get complement med. lysis = HEMOGLOBINURIA (blood in urine)
- can happen throughout the day but urine is the most concentrated in the morning
other s/s:
-abdominal pain, easy bruising and blood clots along with norm. s/s of anemia

Front 98

how do you diagnose paroxysmal nocturnal hemoglobinuria (PNH)

Back 98

diagnosis:
1. based on CD55 and CD59 missing on the RBC = diagnostic
2. low free haptoglobin: haptoglobin binds to hemoglobin but due to inc in RBC lysis = free and the haptoglobin will bind to it
3. bone marrow smear

Front 99

what is the treatment for paroxysmal nocturnal hemoglobinuria (PNH)

Back 99

Soliris: drug that prevents breakdown of RBC's
-blood transfusion
-blood thinners: to prevent clots
-bone marrow transplant
- vaccination for certain DZ's: spleen could be taken out if too enlarged

Front 100

I am an X-linked hereditary condition in which RBC's are broken down prematurely due to stress of an infectious process or certain drugs

Back 100

G6PD defieciency: enzyme required in the metabolism of RBC (process that maintains structures)

Front 101

what drugs trigger G6PD deficiency to break down RBC's prematurely

Back 101

-antimalaria drugs
-anti TB drugs
-sulfonamides
-asprin
-avoid fava beans, moth balls and methylene blue
If you avoid these triggers then then you should be able to avoid premature breakdown of RBC's

Front 102

In this anemia destruction of RBC's is due to the involvement of the immune system. and how do you treat it

Back 102

immune hemolytic anemia
2 types:
warm type (most common)
cold type
treated with steroids: b/c they are immunosuppressive

Front 103

when autoantibodies formed in immune hemolytic anemia

Back 103

1. pregnancy: hemolytic Disease of the Newborn (HDN)
2. past transfusions
3. certain drug therapy can alter peitopes on RBC's
4. infections: ex. staph and strep infections mimic antigens on RBC's causing their lysis

Front 104

what is the most common familial anemia

Back 104

Sickle cell anemia = hereditary hemoglobinopathy characterized by inc. in RBc destruction that can lead to death. characteristic: abnormal Hb = HbS

Front 105

what point mutation results in the prod. of sickle cell anemia

Back 105

point mutation that exchanges valine for glutamic acid on position 6 on the Beta chain

Front 106

what is normal HbA made up of and what are the normal variants of hemoglobin HbA2 and HbF made up of

Back 106

HbA = 2 alpha globin and 2 beta globin chains that form a tetramer
HbA2 = 2 alpha and 2 delta globin chains
HbF = fetal hemoglobin = 2 alpha and 2 gamma
2 genes code for an alpha globin = 4 alleles
1 gene codes for a beta = 2 alleles

Front 107

in sickle cell anemia what causes a cell to sickle and lyse

Back 107

when O2 tension is low
ex. high altitudes, vigorous exercise or areas of micorvasculature of the body

Front 108

when O2 tension is low and RBC's begin to sickle

Back 108

1. microvascular occlusion
2. chronic hemolysis
3. tissue damage
4. painful vasoocclusive crisis: sickle cells do not flow well through vessels = occlusion = hypoxia = pain and angina

Front 109

how does sickeling cells lead to more sickling

Back 109

Hb is released as a result of lysis, which leads to inactivation of NO = if NO is INACTIVATED = dec. vasodilation = inc. vasoconstriction = dec. perfusion = dec. O2 = sickeling

Front 110

A defining morphological characteristic in sickle cell anemia is hyperplastic bone marrow expansion and secondary bone formation that results in prominent cheek bones and changes in the skull that resembles "spiky hair" what is this due to

Back 110

this is due to the inc. in RBC lysis, this inc. loss of cells causes body to need to replace lost RBC = inc. in RBC prod. and inc bone marrow produced everywhere to inc. RBC prod.

Front 111

if a baby has sickle cell Dz why is it not often manifested until 5-6 and sometimes 9 months. How does this trait explain why a heterozygotes not experience symptoms

Back 111

HbF is still present at this time, so just like a heterozygote HbAS you have one other variant of normal hemoglobin and thus no expression of the disease

Front 112

what is the prognosis for someone with sickle cell and what is the treatment

Back 112

90% of patients survive to age 20 treatment: hydroxyurea

Front 113

these are a group of disorders that result from altered synthesis (usually dec) in adult hemoglobin globin chains.

Back 113

thalassemia syndromes
these like sickle cell and hereditary spherocytosis are inherited dz's.

Front 114

true or false: defects in globin synthesis can also affect RBC prod

Back 114

TRUE!

Front 115

these 2 diseases are related to trotection against malaria

Back 115

thalassemia syndromes and sickle cell anemia

Front 116

in this syndrome there is altered synthesis in adult hemoglobin beta chains of globin protein

Back 116

b-thalassemia: 1 gene is responsible for making the 2 beta globin proteins = 2 alleles

Front 117

In this syndrome there is altered synthesis in adult hemoglobin alpha chains of globin protein

Back 117

alpha thalassemia: 2 genes are responsible for making the 2 alpha globin protein = 4 alleles

Front 118

what determines the severity of b-thalassemia

Back 118

severity is based on the types of mutations and the amount of altered beta chain synthesis. Over 100 types of mutations. Mutations cause splicing variation in mRNA resulting in non-production or reduced beta globin production
B0= no beta globin B+= reduced beta globin

Front 119

how does b-thalassemia (a mutation causing resulting in no, or reduced beta-globin) result in hemolytic anemia (inc. red cell destruction)

Back 119

due no or reduced beta globin results in an excess ratio of alpha:beta chains. this excess alpha chains causes them to precipitate in the RBC and hence the RBC i removed from circulation.
* most RBC's die in the BM before reaching circulation

Front 120

in beta thalassemia: no or reduced beta globin results in an excess ratio of alpha:beta chains. this excess alpha chains causes them to precipitate in the RBC and hence the RBC i removed from circulation. what happens as a result of this increase death of RBC's

Back 120

excess osseous lesions that lead to skeletal abnormalities (spiky hair like), and marrow hyperplasia
- splenomegaly: causes enlarged bloated belly
-jaundice: due to hb breakdown prod.
-fatigue

Front 121

B+/B+, B+/B0, B0/B0
What does these genotypes code for

Back 121

thalassemia major,
these are homozygous
B+= reduce
B0 = no b-globin
associated with severe hemolyticanemia

Front 122

What does these genotypes code for:
B/B+, B/B0

Back 122

Thalassemia minor = heterozygous = mild/asymptomatic

Front 123

what would you expect the Hb/Hct level to look like in b-thalassemia disease states

Back 123

remember that defects in globin syntehsis can also affect RBC prod. :
DECREASE in Hct, Hb, and RBC
this is diff. from Fe def anemia: dec in Hb and Hct and an inc. in reticulocyte %

Front 124

would a defect in alpha chains to make beta-thalassemia better or worse?

Back 124

no change or better b/c when you have a deficiency in beta chains causes a inc in alpha chains which cause RBC to precpitate more readily = RBC lysis
But.... if there is also a defect in alpha chains you will get less precipitation and = less damage

Front 125

This hemoglobin defect is commonly caused by a gene deletion. what characterizes this defect

Back 125

alpha thalassemia!
characterized by excessive gamma chains (previously had HbF) or excessive beta chains (previously had HbA)

Front 126

1 gene deletion, no noticeable dz, slight microcytosis, but cells are pretty well hemoglobinized
(mild/asymptomatic) microcytosis IS present are characteristic of this type of alpha thalassemia

Back 126

silent carrier = 1 gene

Front 127

type of a-thalassemia with 3 gene deletions, most common in asian population, high O2 affinity, hence poor delivery to tissue that can lead to hypoxia, and resembles beta-thalassemia intermedia

Back 127

HbH disease

Front 128

type of a-thalassemia with 2 gene deletions, similar to b-thalassemia minor (mild/asymptomatic) microcytosis IS present

Back 128

alpha thalassemia trait

Front 129

how many gene deletions are seen in hydrops fetalis type of alpha thalassemia. what characteristics define this alpha thalassemia

Back 129

4 gene deletions
high O2 affinity in fetus, fetus often requires an intrauterine transfusion, fetus also may often have hepatosplenomegaly

Front 130

what are some treatments of HbH disease and hydrops fetalis (3 gene deletion, and 4 gene deletion of alpha thalassemia)

Back 130

give baby a transfusion in utero
best short term after birth = blood transfusion
long term = bone marrow transplant

Front 131

what is normal level of WBC in an adult

Back 131

4.8-10.8 x 10^3/mm3

Front 132

this is the rarest white cell

Back 132

basophils
0-2% don't normally see in blood

Front 133

what white cell makes up the largest % in blood

Back 133

segmented neutrophils
29-60%
normal is toward the higher end

Front 134

what are the 2 most abundant white cells

Back 134

neutrophils- 29-60%
and lymphocytes: 25-31%

Front 135

what white cell is the third most abundant out of the 5

Back 135

monocytes = 2-6%

Front 136

what white cells are least abundant

Back 136

eosinophils: 0-5%
basophils: 0-2% rarest normally don't see in blood

Front 137

a reduction in the number of white blood cells

Back 137

leukopenia

Front 138

what is the difference between neutropenia and lymphopenia if they are both a dec. in WBC

Back 138

neutropenia dec due to reduced numbers of circulating neutrophils
lymphopenia dec. due to reduced numbers of circulating lymphocytes

Front 139

total white count is 1000 WBC/mm3 or less, with severe neutropenia

Back 139

Agranulocytosis

Front 140

what is reactive leukocytosis and give an example of this disease

Back 140

an increased number of white blood cells example is infectious mononucleosis: caused by infection by B lymphotropic epstein barr virus

Front 141

this WBC disorder affects adolescents and young adults typically you will see fever, sore throat and lymphadenitis. Most prominent features may be malaise, FATIGUE, and lymphadenopathy, and palatal petechiae

Back 141

Infectious mononucleosis

Front 142

how do you diagnose infectious mononucleosis

Back 142

1.lymphocytosis with atypical lymphocytes on peripheral blood smears
2. positive heterophile reaction is diagnostic referred to as a "monospot"
3. antibodies for EBV

Front 143

this disease is caused by a pleomorphic gram neg. bacillus, called Bartonella henselae. this bacterium is visible in tissue sections.
this disease often affects children and adolescents du to trauma and red pustule/papule forms at site.

Back 143

Cat scratch disease a form of reactive lymphadenitis weeks
after the initial trauma and red pustule regional lymph nodes become large, painful, tense and redden. Lymphadenopathy is out of proportion with size of the orig. wound

Front 144

name the histologic growth patterns of non-hodgkin lymphoma

Back 144

-nodular pattern: shows follicles, better prognosis not as aggressive
- diffuse pattern: no follicles, more biologically aggressive = worse prognosis

Front 145

name the most common and rarest type of non- hodgkin lymphoma

Back 145

common diffuse large cell lymphomas
rarest burkitt lymphomas

Front 146

If you are told by your doctor that you have non-hodgkin lymphomas which ones do you hope that you have

Back 146

small lymphocytic lymphoma = indolent course with prolonged survival
Not good = mantle cell lymphoma which is aggressive and difficult to cure

Front 147

this is the rarest non-hodgkin lymphoma in the US but is endemic in Africa. It predominantly affects children. name this non-hodgkin lymphoma what immunophenotype it has and its prognosis

Back 147

Burkitt lymphoma
immunophenotypes: matures CD 10+ B cells expressing surface Ig

Front 148

name the histologic growth patterns of non-hodgkin lymphoma

Back 148

-nodular pattern: shows follicles, better prognosis not as aggressive
- diffuse pattern: no follicles, more biologically aggressive = worse prognosis

Front 149

name the types of hodgkin lymphoma and list them from most to least common

Back 149

1. nodular-sclerosis hodgkin lymphoma: most common and most unique: has lacunar variants of Reed sternberg ceells and is the only HD that is more common in women
2. mixed cellularity HL: prognosis is not as good
3. lymphocyte-predominance: HL, excellent prognosis
4. lymphocyte depletion: older patient, aggressive form bad prognosis

Front 150

name the most common and rarest type of non- hodgkin lymphoma

Back 150

common diffuse large cell lymphomas
rarest burkitt lymphomas

Front 151

If you are told by your doctor that you have non-hodgkin lymphomas which ones do you hope that you have

Back 151

small lymphocytic lymphoma = indolent course with prolonged survival
Not good = mantle cell lymphoma which is aggressive and difficult to cure

Front 152

this is the rarest non-hodgkin lymphoma in the US but is endemic in Africa. It predominantly affects children. name this non-hodgkin lymphoma what immunophenotype it has and its prognosis

Back 152

Burkitt lymphoma
immunophenotypes: matures CD 10+ B cells expressing surface Ig

Front 153

name the types of hodgkin lymphoma and list them from most to least common

Back 153

1. nodular-sclerosis hodgkin lymphoma: most common and most unique: has lacunar variants of Reed sternberg ceells and is the only HD that is more common in women
2. mixed cellularity HL: prognosis is not as good
3. lymphocyte-predominance: HL, excellent prognosis
4. lymphocyte depletion: older patient, aggressive form bad prognosis

Front 154

name the histologic growth patterns of non-hodgkin lymphoma

Back 154

-nodular pattern: shows follicles, better prognosis not as aggressive
- diffuse pattern: no follicles, more biologically aggressive = worse prognosis

Front 155

name the most common and rarest type of non- hodgkin lymphoma

Back 155

common diffuse large cell lymphomas
rarest burkitt lymphomas

Front 156

If you are told by your doctor that you have non-hodgkin lymphomas which ones do you hope that you have

Back 156

small lymphocytic lymphoma = indolent course with prolonged survival
Not good = mantle cell lymphoma which is aggressive and difficult to cure

Front 157

this is the rarest non-hodgkin lymphoma in the US but is endemic in Africa. It predominantly affects children. name this non-hodgkin lymphoma what immunophenotype it has and its prognosis

Back 157

Burkitt lymphoma
immunophenotypes: matures CD 10+ B cells expressing surface Ig

Front 158

name the types of hodgkin lymphoma and list them from most to least common

Back 158

1. nodular-sclerosis hodgkin lymphoma: most common and most unique: has lacunar variants of Reed sternberg ceells and is the only HD that is more common in women
2. mixed cellularity HL: prognosis is not as good
3. lymphocyte-predominance: HL, excellent prognosis
4. lymphocyte depletion: older patient, aggressive form bad prognosis

Front 159

True or false
Stage I-A and II-A have close to 100% survival
Stages Iv-A and IV-B have up to 50%

Back 159

true

Front 160

what is the difference between leukemias and lymphomas

Back 160

leukemias are malignant neoplasms of the hematopoietic stem cells characterized by diffuse replacement of bone marrow by neoplastic cells
lymphomas: cancers of lymphoid tissues characterized by the proliferation or accumulation of cells native to lymphoid tissue