BL Unit 2

Question 1

6 types of T cells and functions

Answer 1

5 helper T cells with surface CD4, 1 killer T cell

Th0- undecided precursor; differentiate after dendritic cell presents antigen

Th1- hypersensitivity T cell
secretes INTERFERON GAMMA- pro-inflammatory; M1; chemotactic for monocytes/macrophages

Th17- makes IL-17; resembles Th1 (inflammation); particularly resistant to pathogens

Th2- make IL-4 and IL-3; macrophages are ALTERNATIVELY ACTIVATED or M2; more involved in healing; IL-4 also chemotactic for eosinophils (kill parasites/worms)

Thf- migrate to cortex follicles; help B cells activate into antibody-secreting plasma cells; switch B cells from IgM to IgG/A/E, depending on organ

Treg- suppress all other Th cells; produce TGFbeta and IL-10; very potent

CTL- kill infected cells; lethal hit- signals target to apoptosis

Question 2

surface markers on T and B cells, and helper vs cytotoxic T cells

Answer 2

B cells: CD20

All T cells: CD3
All Th: CD4
CTL: CD8

Question 3

cytokines, lymphokines, and chemokines

Answer 3

cytokines- short range mediators made by any cell; affect behavior of same or other cell
IL-1, IL-12

lymphokines- short range mediators made by lymphocytes; subset of cytokine
IFNgamma IL-2,4,5,10

chemokines- small short range mediators made by ay cell; primarily cause inflammation
IL-8, eotaxin

Question 4

lymphokines made by Th and Treg cells

Answer 4

Th1- IFNgamma and IL-2; pro-inflammatory; attract/activate MI macrophages

Th17- IL-17; attract/activate MIs

Th2- IL-4; pro-inflammatory; attract/activate M2 macrophages

Treg- TGFbeta and IL-10; anti-inflammatory cytokine

Question 5

describe how Thf and B cells get activated by antigen and switch immunoglobin class

Answer 5

B cell binds its specific epitope and enodcytoses it; the fragments bind to MHC class 2 molecs and move to surface

B cell displays new antigen and Class 2 MHC complex on surface

correct Thf binds and focuses surface interactions and helper lymphokines on B cell

Question 6

define mitogen and uses for T and B cell mitogens in lab

Answer 6

a. Mitogen: protein that stimulates T cell division

examples of mitogens:
PHA: binds CD3, ConA to stimulate T cell division
PWM: nonspecifically stimulates B and T cell division

Question 7

effects of mitogen vs antigen when added to normal blood lymphocyte

Answer 7

antigens are specific
mitogens are nonspecific

mitogen- binds CD3 domain to always keep signal on

antigen- binds to antigen-binding site on T cell

Question 8

antigen receptors on T and B cells

Answer 8

B cells: bind antigen directly with surface antibodies; interact with free antigens

T cells: focus on cell surfaces; only see complexed antigens presented on surface of an identical cell

Question 9

Antigen Presenting Cells APC

Answer 9

dendritic cells
chop up and display antigens on surface as MHC-antigen complex for recognition by another T cell

Class 2 MHC molecs- when antigens are endocytosed and presented
T cell helpers recognize Class 2

Class 1 MHC molecs- when proteins are synthesized within the cell (not endocytosed);
CTL recognize Class 1

Question 10

role of T cells in ridding body of viral infection

Answer 10

CTL sees foreign cell (because MHC Class 1 will have it bound); activate target cell to commit suicide through CD95L or lytic granules

Th cells see antigen on dendritic cell, B cell, or macrophage via MHC Class 2; activate immune response and divide

Question 11

characteristics of T independent antigens

Answer 11

T independent antigens usually have same epitope repeated over and over (common in carbs- streptococcus pneumoniae)
carb chains bind to B cell antibodies; cell activates/divides
response is almost all IgM, so a person deficient in T cells will still make carb antibodies

with protein antigen (rare)- NO IgM or IgG is made without T cell help

Question 12

experiment where antibody response can be T-dependent

Answer 12

test two leukocyte populations’ ability to make antibodies to the same antigen- 1 with full complement of T and B cells, and 1 with T cells killed by radiation

Question 13

define Human Major Histocompatibility Complex MHC

distinguish between HLA-A and HLA-B antigens and HLA-D

Answer 13

MHC- group of strongest histocompatibility antigens coded for by a family of genes on a single chromosome

Th- recognize MHC antigens on HLA-D loci (Class 2)

CTL- recognize MHC antigens on HLA-A and HLA-B loci (Class 1)

Question 14

class 1 vs class 2 histocompatibility antigens

Answer 14

Class 1 antigens- found on all nucleated cells

Class 2 antigens- restricted to B cells, macrophages, dendritic cells, and a few others

Question 15

define alloantigen and haplotype

Answer 15

alloantigen- part of animal’s self-recognition system (like MHCs)
when injected into another animal, they trigger an immune response aimed at eliminating them
present in some members of a species, but not common to all

haplotype: MHC gene set that you inherited from one parent

Question 16

Given the HLA‐A, HLA‐B and HLA‐DR phenotypes of 2 parents and their child, work out the 4 haplotypes involved.

Answer 16

Typing at the HLA-A and HLA-B loci can be done by treating the patient’s leukocytes with allele-specific anti-HLA antisera and complement. The most sophisticated labs actually sequence the HLA genes themselves for typing.
D3, B7, A1, etc., are each individual’s haplotypes.
The cells show their phenotypes, the actual proteins expressed on the surface of their cells. Every cell expresses both alleles.

Question 17

identify the best probable donors of tissues or bone marrow to an individual

Answer 17

good DR match is most important (Class 2)

for Class 1- HLA-A and HLA-B are most important
cells not identical will not stimulate Th1 cells and the huge response; they’ll activate everything else

cells identical at HLA-A and B will not stimulate CTL, but the Th1 cells will still be stimulated and won’t be great

identical twin or sibling is best chance for match
look for bone marrow matches at A, B, C, DR, and DQ

Question 18

one-way mixed leukocyte reaction (MLR) and its use

Answer 18

cells from donor are treated to prevent their division (via DNA synthesis inhibitors/radiation)

observe recipient’s Th cells dividing in response to donor’s HLA-D (mostly DR)

a strong rxn may preclude doing the transplant

Question 19

distinguish between HLA-D and DR, DP, DQ

Answer 19

HLA-D is general term for group of loci that give rise to MHC type 2 antigen-presenting proteins

DR, DP, DQ- individual loci within the D region of Chromosome 6

Question 20

interaction of T cells recognizing antigen plus HLA-D and A/B in graft patient

Answer 20

Th are programmed to recognize HLA-D (class 2)

CTL recognize HLA-A and B (Class 1)

rejection: dendritic and macrophage cells from graft move to host lymph node; host Th1 cells recognize foreign HLA-D and synthesize lymphokines and up regulate cell-surface receptors for GFs like IL-2; Th1 also will secrete IFNgamma that attract M1 macrophage inflammation

CTL- recognize foreign HLA-A and B, but also require Th1-derived IL’s as a second signal for activation; once activated, highly cytotoxic and may proliferate

similar to virus, except:
normal response: peptide plus self-MHC recognition
rejection: foreign MHC recognition

Question 21

cellular and molecular events of a graft rejection- normal and hyperacute

Answer 21

most important mech is via CTL and Th1 cells (via lymphokines and monocyte/macrophage inflammatory response)

normal rejection: Th1 cells are activated by “almost me” MHC type 2’s; activate Th2 cells, which activate B cells to produce antibody against graft; and CTL attach tissue directly once they bind to MHC type 1s
Th1 also brings other inflammatory cytokines, like TNF-alpha (tissue necrosis factor)

hyperacute rejection: graft tissue rejected immediately- stays white/bloodless even after reperfusion

• there was a circulating antibody against the graft from a previous/failed graft or against graft’s residual blood
• antibodies attach to endothelium, activate lots of complement, set off anaphylatoxin release (C3a, C4a, C5a) from mast cells; leads to vasospasm and tissue ischemia; can lead to systemic inflammation
• T-cell mediated rejection is slower than complement-mediated
• always cross-type the ABO blood antigens from donor and recipient
• immunosuppressants are typically given for a lifetime after a transplant

Question 22

how T cells recognize “self + x” and foreign MHC (allorecognition)

Answer 22

receptors are selected to recognize “self + x”

recognition of foreign MHC is a “chance” cross-reaction
5% of T cells will bind a foreign MHC strong enough to cause activation
can’t give other people T-cells because MHCs are different and T cells are specifically selected for an MHC

Question 23

example of disease whose incidence is tightly linked to a particular HLA allele and its mech

Answer 23

ankylosing sponditis- involves chronic inflammation and eventual calcification of the insertions of tendons into bones

95% of people w/ this have a specific HLA-B allele that will also disease rats

-price to pay for genetic variability in HLA region- eventually it’s going to look similar to an antigen and you’ll develop an autoimmune response to own tissues

also HLA-linked cases of diabetes, lupus, and kidney/lung degenerative disorder

Question 24

basic structure and general movement of lymph and lymphocytes through a lymph node

Answer 24

lymph circulates to lymph node via afferent lymphatic vessels and drains into node just beneath capsule called sub scapular sinus
subcapscullar sinus drains into trabecular sinuses then to medullary sinuses
sinus space is criss-crossed by pseudopods of macrophages, which filter lymph
medullary sinuses converge at hilum and leave via efferent lymphatic vessel
ultimately drain to central venous subclavian blood vessel via post-capillary venules; cross wall via diapedesis

Question 25

activated vs non-activated nodules

Answer 25

``` germinal center differentiates the two germinal centers- sites within lymph nodes/nodules in peripheral lymph where mature B cells proliferate and class switch ```

Question 26

vasculature of lymph nodes

Answer 26

blood supply enters through small artery in hilum branches repeatedly to entire node specially lined with HIGH ENDOTHELIAL VENULE- site of diapedesis of lymphocytes from blood to lymph node -allows you to populate all nearby lymph nodes rapidly during an infection leaves via small vein

Question 27

blood flow through thymus

Answer 27

small arteries enter thymus through outer capsule and penetrate into thymus bifurcate within the CT septa between lobules vessels' cells have tight junctions, and surround by endothelioreticular cells- forms blood-thymus barrier for developing thymocytes efferent lymphatics also travel in the septum

Question 28

thymus blood flow and thymus lymph fluid flow

Answer 28

blood- everywhere, blood flow in via arteries, out via veins pierce capsule; trebeculae; cortex; everywhere (incl medulla) lymph flow- none coming in. efferent lymphatic drain lymph fluid (and veins) outwards NO afferent lymphatic to thymus

Question 29

nuclei and cell bodies of reticuloendothelial cells in thymus and Hassall's corpuscles

Answer 29

involved in selection process for thymocytes as they progress toward medulla; provide microenvironment to protect maturing thymocytes Hassall's corpuscles: cells that thickly populate medulla; produce lymphokines that promote thymocyte maturation into adult T cells

Question 30

blood flow through spleen

Answer 30

open blood circulation through porous splenic sinuses receives blood via splenic artery branches into central arterioles into the red pulp lined with discontinuous endothelial cells where RBCs, WBCs, and platelets exit to enter sinuses Periarteriolar lymphoid sheath (PALS): sheath around central arterioles; WHITE PULP; germinal centers within these sheaths drained via splenic vein only has efferent lymph vessels (like thymus), which leave from hilum

Question 31

cell components of red and white pulp

Answer 31

red pulp: 75% of spleen; RBC rich with loose sinuses; filters blood, antigens, microorganisms, and old RBCs white pulp: organized lymph tissue contains T cells, B cells, accessory cells; mount an immune response to antigens in blood; present in form of PALS, containing B cell follicles and T cells

Question 32

regions of mucosal-associated lymphoid tissue

Answer 32

tonsils (palatine, lingual and pharyngeal (adenoids), esophageal nodules, appendix, bronchial nodules, large aggregation of lymphocytes in intestine, colon: abundant nodules both in mucosa and submucosa known as Peyer's patches

Question 33

function and distribution of lymph system

Answer 33

cleanse blood and lymph and provide adaptive immunity produces and stores agranular WBCs or lymphocytes ``` 4 forms of lymph tissue: non-encapsulated aggregates of lymphocytes lymph nodes thymus spleen ``` these are composed of free lymphocytes and a supporting framework of reticular cells

Question 34

types of lymphoid cells

Answer 34

helper T cells (5 kinds) - help B cells - express CD3 and CD4 - recognize MHC Class 2 CTL - express CD3 and CD8 - recognize MHC Class 1 B cells express CD20

Question 35

structure of all major lymph organs

Answer 35

lymph nodes- small; found all over individually or clustered; non-specific filters of debris/microorganisms; site of antigen presentation in adaptive immunity lymphocyte enters small lymphatic vessel; connects to afferent lymphatic vessel; enters node into sub capsular space thymus gland- bilobed thymus with CT capsule where trabeculae divide organ into pseudo lobes, where all thymocytic cells develop to release mature T cells -lymphocyte mass in thymus decreases through childhood NO reticular fibers stromal cells provide support Hassall's corpuscles: circular layer of reticular cells in medulla to suppress autoimmune events CORTEX: densely packed developing thymocytes MEDULLA: more mature thymocytes, less dense thymocytes leave via lymphatics and blood vessels spleen: multi purpose lymphoid organ; role in adaptive immunity MALT: mucosal-associated lymphoid tissue unencapsulated collections of lymph cells and associated support cells to encounter antigens passing through mucosa -tonsils, appendix, nodules, Peyer's patches in intestine -M cells deliver antigen to underlying lymph tissue for adaptive immune response in intestine

Question 36

primary vs secondary lymph organs

Answer 36

primary: bone marrow and thymus major sites of development of B and T cells secondary: seeded with cells from primary tissues (GALT, Peyers patches, etc)

Question 37

encapsulated lymph organs

Answer 37

lymph nodes spleen thymus

Question 38

humoral immunity and cell-mediated immunity

Answer 38

humoral immunity may prevent a viral illness, but T cell immunity is necessary for recovery - antibody maybe prevent virus from establishing an infection - once the infection takes place, you need to kill infected cells before virus multipilies

Question 39

define local immunity

Answer 39

local immunity on the surface that is being invaded can prevent the invasion- secretory IgA Sabin (attenuated, live, oral) polio vaccine was so effective- those immunized had high levels of IgA in their secretions and didn't get colonized by real virus

Question 40

organisms against which cell-mediated immunity is most effective

Answer 40

viruses, fungi, yeasts, intracellular bac

Question 41

organisms against which humoral immunity is most effective

Answer 41

extracellular bac and pathogens

Question 42

human and animal antitoxin killed virus vaccine live virus vaccine longest immunity

Answer 42

human: IgG against tetanus antimal: IgG against tetanus practical difference: IgG solns tend to aggregate when they sit around humans- causes lots of complement activation (pain, inflammation, etc) due to proximity of bound IgG antibodies animal- less complement is activated due to inter-species antibodies not activating each other's complement very well killed vaccine: injected polio (Sabin) vaccine live vaccine: oral polio (Sabin) vaccine longest immunity tends to be live vaccines because body produces MHC Class 2 AND 1 responses from your own, infected cells

Question 43

children immunizations for diphtheria, pertussis, tetanus polio measles

Answer 43

diphtheria, pertussis, tetanus: 15-18 months polio: 2 mo, 4 mo, 6-18 mo, 4-6 years measles: 12-15 mo, 4 years live viral vaccines tend to be ineffective in young; destroyed by mother's circulating IgG before the child develops the antibody

Question 44

IgG and IgM titers in diagnosing infections

Answer 44

IgM- made quickly and goes away quickly, so gives good idea if kid has had a disease recently IgG- measure several times to determine increase/decrease; have they already been sick and made them, or are they just now getting sick?; mother's IgG in utero

Question 45

polio vaccines- oral and parenteral

Answer 45

in US: parenteral polio vaccine used -is a killed (Salk) vaccine; because some kids with weak immune sys's might get sick from exposure to the live Sabin virus, given oral oral is easier to distribute, esp in healthcare access problems - transmissable- immunized kid can spread attenuated virus and spread protection - can cause polio in immunocompromised kids

Question 46

define herd immunity

Answer 46

proportion of a given population that has immunity against a particular infection commonly expressed as percentage

Question 47

morphologic features of monocytes and tissue macrophages

Answer 47

blue/purple stain with u-shaped nucleus derived from myeloid/monocyte precursor under stimulation of GM-CSF and M-CSF develop in bone marrow for 7 days then move to peripheral blood for 3-5 days; some emigrate to tissues turnover: days-months major funcs: migrate to sites of infection and remove microbes, dead/dying inflammatory cells/debris; filter microbes from blood stream (spleen); process and present antigens to adaptive immune sys; remove apoptotic cells

Question 48

morphologic features of eosinophils

Answer 48

RED cytoplasm and bi-lobed nucleus produced in bone marrow from IL-5 move to peripheral blood then mucosal surfaces (GI tract, tracheobronchial tree, etc) turnover: weeks can play a role in allergic reactions, parasitic infections, and response to tumors can be phagocytic and immunostimulatory or inhibitory

Question 49

morphology of basophil

Answer 49

prominent blue-purple primary granules produced in bone marrow receptors for IgE and appear to play major role in hypersensitivity (allergic) reactions

Question 50

neutropenia and clinical consequences

Answer 50

decrease in absolute neutrophil count (bands and sets) below accepted norms adults less than 1500 is bad; newborns less than 3000 is bad risk for infection

Question 51

acquired and congenital causes of neutropenia

Answer 51

acquired: chemotherapy drugs viral infections (EBV, measles, CMV, hepatitis, HIV) nutritional deficiencies: folate, B12, copper, protein/calorie congenital: Kostman Syndrome- severe peripheral neutropenia + decrease in myeloid production -high risk for infection and death before age 2 w/o aggressive treatment; ARREST in neutrophil development Schwachmai-Diamond Syndorme- neutropenia, pancreatic insufficiency (fat malabsorption, bone abnormalities, growth delay); 1/2 develop aplastic anemia or MDS/leukemia; may die early from bone marrow defect; APOPTOSIS of neutrophil precursors cyclic neutropenia- severe neutropenia (5-7 days) with periodicity (15-25 day cycles); low ANC= mouth ulcers chronic idiopathic neutropenia: from myeloid hypoplasia and maturation arrest

Question 52

increased turnover in neutrophils

Answer 52

chronic benign neutropenia of childhood- no risk of infection; resolves after mo 20 autoimmune neutropenia alloimmune neutropenia- mother's Ab's attack baby's neutrophils splenomegaly and hypersplenism severe infection- activate C5a- excessive killing of neutrophil that have eaten bugs

Question 53

major treatment strategies for neutropenia

Answer 53

broad spectrum antibiotics then specific antibiotics if infection can be identified granulocyte colony stimulating factor (G-CSF) to normalize production of neutrophils some antibody mediated syndromes may response to IV gamma-globulin IVIG

Question 54

# define leukocytosis left shift

Answer 54

increase in total WBC count think infection, inflammation, non-specific physiologic stress, malignancy/leukemia left shift- changes in WBC differential with increase in sets and bands and possibly some immature myeloid precursors usually only found in marrow (metamyelocytes of myelocytes)

Question 55

basophilia

Answer 55

increase in basophils primarily seen in drug or food hypersensitivity or urticaria; also in infection/inflammation (rheumatoid arthritis, ulcerative colitis, influenza, chickenpox, smallpox, tuberculosis) as well as myeloproliferative diseases (CML, myeloid metaplasia)

Question 56

eosinophilia

Answer 56

drugs, bugs, allergies allergies/allergic disorders (asthma, hay fever, hives, etc), parasitic infections, drug rxns, more rare: pemphigus, tumors/malignancies, other infections

Question 57

monocytosis

Answer 57

lymphomas, infection, and collagen disease may be found in hematologic (pre) malignancies (AML, pre-leukemia states, lymphoma, Hodgkin's), collagen vascular disease (SLE, RA), granulomatous disease (sarcoid, ulcerative colitis, Crohn's), infection (subacute bacterial endocarditis, syphilis, tuberculosis), and carcinoma

Question 58

normal functions of neutrophils

Answer 58

Adhesion: CD11b and CD18 Ingestion: CD11b to ingest microbe move in laminar flow of blood but are pulled into infected areas via rolling motions with endothelial cells; then firm adhesion with adhesion proteins; then diapedesis through cell junctions; move toward offending organisms via chemotaxis; following chemoattractants (like bac products, C5a, cytokines, chemokines) up the conc gradient to engage the invader at site of infection: microbe has been opsonized with C3b or antibody and is enveloped by several fusing pseudopods, forming a phagosome; eventually initiate respiratory burst and from ROS ROS and oxygen-independent mech's are focused on the phagolysosome and lead to death/dissolution of microbe

Question 59

neutrophil dysfunctions ``` LAD 1, LAD 2, Actin disfunction specific granulocyte deficiency myeloperoxidase deficiency chediak-Higashi syndrome chronic granulomatous disease ```

Answer 59

Leukocyte adhesion deficiency I: CD18 deficiency; dec adherence to endothelial surface- neutrophilia; lack CD18, recurrent soft tissue infections like gingivitis, cellulitis, abscesses, delayed umbilical cord separation LADII: abnormal Sialyl LeX prevents adhesion to selections- neutrophilia; both LAD 1 and 2 have recurrent infection issues with wound healing; decreased adherence, mental impairment, short stature, bombay phenotype actin dysfunction: impaired chemotaxis and ingestion; recurrent infection; defect in actin assembly specific granule deficiency- diminished chemotaxis and bacterial killing= recurrent infections; can't make granule proteins myeloperoxidase deficiency- impaired fungal killing when diabetes is present too; packaging defect in processing of granules; can't kill CANDIDA Chediak-Higashi syndrome- granule defects - leak + big, defects in movement + degranulation and microbicidal activity; oculocutaneous albinism, photophobia, fever, hepatosplenomegaly, neurodegenerative; don't get rid of granules well--> large granules chronic granulomatous disease- absence of respiratory burst and production of ROS; defects in one of 4 oxidase components, so no toxic oxygen metabolites are produced; can't kill COAGULASE + bacteria/fungus; defect in gp91phox

Question 60

NADPH oxidase enzyme system

Answer 60

composed of 6 or more proteins distributed in plasma membrane or specific granule membrane or in cytosol with a phagocytic stimulus, assembly of the cytosolic components with the membrane components assembles the system and results in activity with addition of an electron to oxygen to form supersede anion from which H2O2 and other ROS can rapidly be formed uses and electron from NADPH lab testing: DHR: if you have oxidative burst-fluorescence, measure oxygen power NBT dye reduction, lets for CGD: the higher the blue score the better it is at making ROS defect evidenced by failure to reduce NBT dye, oxidize dihydrohodamine, or produce O2 defect impairs bactericidal activity

Question 61

characterize infections with defects in phagocytes or complement

Answer 61

phagocytes: high bac and fungal infections infections w/ atypical or unusual microorganisms catalase positive organisms in patients with CGD infections of exceptional severity periodontal disease in childhood recurrent infections where body has interface w/ microbial world cellulitis, perianal complement: bac infections which might be seen w/ antibody deficiency terminal complement deficiencies (**C5-C9**) have problems with Neisseria** C3-recurrent bacterial infection C1, C2, C4-SLE, autoimmune, inflammtion

Question 62

screening and confirmatory tests for phagocyte problem

Answer 62

``` screening: CBC, differential Review of morphology Bactericidal activity Chemotaxis assay Expression of CD11b/CD18 NBT dye reduction or DHR oxidation. ``` confirmatory: Adherence to inert surface or endothelial cells. Measurement of CD11b/CD18, L-selection, Sialyl LeX. Response to chemoattractants: shape change, change in direction, rate of movement. Actin assembly. Ingestion of labeled particles or bacteria. Degranulation of specific and azurophilic components. Bactericidal/candidicidal activity. Production of O2-, H2O2 other oxidants. Studies for specific molecular defects in oxidase or other cell constituents.

Question 63

screening and confirmatory tests for complement problem

Answer 63

screening: C3-most common, CH50-total pathway Quantitative Ig’s, Lymphocyte numbers confirmatory: Measurement of specific complement components: alternative and classical pathways. Detailed evaluation of adaptive immune response.

Question 64

management strategies for patients with innate immune disorders

Answer 64

anticipation of infection; aggressive attempt to identify cause surgical procedures for infected sites prompt imitation of broad spectrum antibiotics then switching to specific when identified G-CSF doses for severe quantitative neutropenia prophylactic antibiotics or cytokine therapy (INFgamma for CGD) for specific neutrophil dysfunctions Transplant with hematopoietic stem cells to reconstitute neutrophil numbers/func gene therapy: still a lot of problems to be resolved before it's a practical solution

Question 65

liver disease- suggestive of

Answer 65

macrocytic anemia

Question 66

small MCV means you should get

Answer 66

Hemoglobin electrophoresis

Question 67

thalassemia traits with hemoglobin levels

Answer 67

alpha thal trait- normal Hb alpha thal intermedia- only 1 working alpha; don't need iron therapy; worry about hydrops fetalis beta thalassemia major- low/no Hb H (4 beta chains)

Question 68

Hb H made of | seen in

Answer 68

4 beta chains seen in alpha thalassemia problems

Question 69

chronic transfusion therapy

Answer 69

needed only if patient is symptomatic

Question 70

folic acid treatment

Answer 70

for people who turn over RBCs quickly- hemolytic problems

Question 71

splenectomy treatment

Answer 71

controversial not recommended unless someone is really having trouble with hemolysis

Question 72

bone marrow transplant treatment

Answer 72

for severe beta thalassemia

Question 73

alpha genes missing

Answer 73

1 missing- trivial microcytosis 2 missing- microcytic 3 missing- intermedia 4 missing- hydrops fetalis

Question 74

Hb E

Answer 74

common in SE Asia most common thal on West coast structural variant of Beta globin -potential for beta thalassemia HbEE patients- mild hemolytic anemia and splenomegaly HbE trait- asymptomatic, but maybe low MCV

Question 75

pancytopenia anisocytosis

Answer 75

pancytopenia- reduction in RBC, WBC, and platelets anisocytosis- unusual shape; high RDW

Question 76

increased RBC destruction seen with

Answer 76

high retic count

Question 77

test EPO when you suspect

Answer 77

kidney disease

Question 78

want to see iron levels when

Answer 78

MCV is low, like when surgeon gives patient a lot of blood

Question 79

want to see (high) LDH numbers when

Answer 79

suspect hemolysis or 3-4 days post myocardial infarction

Question 80

high TIBC low iron low ferritin suggests

Answer 80

iron deficiency anemia

Question 81

low/nl TIBC low/nl iron nl/high ferritin suggests

Answer 81

inflammation chronic disease thalassemia

Question 82

low Vit B12 suggests

Answer 82

macrocytic anemia

Question 83

low TIBC doesn't suggest low TIBC and normal serum ferritin suggests

Answer 83

iron deficiency chronic inflammation

Question 84

FE/TIBC levels below 10% mens over 50% mens

Answer 84

iron deficiency iron overload

Question 85

serum creatinine high suggests

Answer 85

kidney function is bad; moderate-chronic kidney disease EPO levels aren't very helpful in mild anemia EPO injections might help

Question 86

high TIBC indicates non-response to EPO injections due to

Answer 86

decreased iron stores lack of iron stores

Question 87

EPO levels are ___ in iron deficiency anemia than anemia of chronic disease

Answer 87

higher

Question 88

EPO is released from kidney in response to

Answer 88

tissue hypoxia

Question 89

when you treat with EPO injections MCV changes

Answer 89

retic count goes up; that's normal if MCV falls to 77fL, treat with iron tablets because you're making blood but MCV is falling because you're iron deficient if MCV goes up to 95, that's normal because retic is going up; don't think they have folate deficiency polycythemia if hypoxia is present Hct >52% usually improved quality of life; but other risk:benefit ratios

Question 90

high MCV suggests

Answer 90

folate deficiency

Question 91

normocytic anemia potential diagnoses:

Answer 91

sickle cell renal failure associated anemia autoimmune hemolytic anemia NOT iron deficiency anemia (microcytic)

Question 92

cytochrome B function

Answer 92

converts ferric to ferrous iron | able to absorb ferrous iron

Question 93

dacryocytes anisocytosis polychromasia spherocytes

Answer 93

teardrop shaped cells variation in size blueish cells,; premature retics being released spheres; no central palor

Question 94

spherocytic anemia

Answer 94

most concerned about DAT level want a direct Coombs test want retic count haptoglobin

Question 95

high retic count suggests:

Answer 95

sickle cell recovery from acute severe GI bleed hemolysis NOT iron deficiency

Question 96

postive Coombs test tells you

Answer 96

it's autoimmune disorder spherocytes- associated with warm antibody autoimmune hemolytic anemia

Question 97

cold agglutinin is usually

Answer 97

IgM and intravascular; C3?

Question 98

warm agglutinin is usually

Answer 98

extravascular, mostly in spleen, so you recycle iron, not lose it low retic count with higher Hct shows successful treatment when abnormally high retic count falls, the MCV falls, because the retics are big

Question 99

possible cause of thrombocytopenia

Answer 99

antibody coating of platelets don't think hemolytic anemia is relapsing if retic count is mostly normal typically not from folate deficiency

Question 100

in most autoimmune hemolytic anemias, you see

Answer 100

high WBCs and platelets

Question 101

hypochromic hyperchromic

Answer 101

pale central palor, sometimes cells break when smeared dark central palor- no transparency

Question 102

melana

Answer 102

black, tarry stools bleeding lowers your iron stores

Question 103

aplastic

Answer 103

not making any RBCs

Question 104

patient with active hemolytic anemia at risk for

Answer 104

folate deficiency but presence of iron in marrow means it isn't deficient

Question 105

high methylmalonic acid indicates

Answer 105

low B12 differentiates B12 vs folate deficiency

Question 106

high homocysteine indicates

Answer 106

either B12 or folate deficiency