Heme-Onc Part 2

Front 1

RC lifespan =

Back 1

120 days

Front 2

macrocytic anemia MAY be caused by:

Back 2

thyroid disease

Front 3

when you suspect microcytic anemia, check:

(tests)

Back 3

iron studies,

ferritin levels

Front 4

electrophoresis or HPLC test for:

Back 4

thalassemias and SCD

(they find abnormal Hb)

Front 5

electrophoresis CANNOT detect alpha-thal past:

Back 5

infancy

Front 6

increased RDW could mean that NEWER cells are smaller than older ones, not that:

Back 6

macrocytic RC's exist

Front 7

PCR can detect __________________ prenatally

Back 7

sickle mutation

Front 8

hemolytic anemias ~~ dec. in:

Back 8

RC lifespan

Front 9

with HA's, the pt MAY NOT be:

Back 9

anemic

- depends on the degree of marrow compensation

Front 10

clinical features of HA's:

(7)

Back 10

1. jaundice (incl. sclera icterus)

2. tea-colored urine

3. pigmented gallstones

4. ankle ulcers

5. splenomegaly

6. apalstic crises

7. inc. need of folate

Front 11

3 functions of the spleen:

Back 11

1. repository for WBC's or plat's

2. contains macrophages that phag. bact and damaged RBC's

3. contains lymphocytes/plasma cells to make AB's

Front 12

splenectomy is called for if the spleen is:

Back 12

doing the wrong things

Front 13

2 features of RC's following splenectomy:

Back 13

1. all sorts of misshapen RC's

2. Howell-Jolly bodies

Front 14

how is Parvovirus B19 related to HA's?

Back 14

a complication, it infects and lyses RC **precursors** in the *marrow*

=> 7-10 days of cessation of erythropoiesis

Front 15

in pts with HA, a loss of RC production =>

Back 15

Hb value plummets dramatically (aplastic crisis)

Front 16

HA's are classified by:

(3)

Back 16

1 sites of RC destruction

2. acquired vs congenital

3. mechanism of RC damage

Front 17

sites of destruction - RC hemolysis either:

Back 17

extravascular or intravascular

Front 18

extravascular hemolysis occurs wherever there are:

Back 18

macrophages to phag. damaged or AB-coated RC's

(liver, marrow, spleen)

Front 19

in intravascular hemolysis, RC's rupture within the vessels, releasing:

Back 19

free Hb into the circulation

Front 20

5 pieces of lab evidence for hemolysis:

Back 20

1. inc. reticulocyte count

2. maybe high MCV

3. erythroid hyperplasia

4. dec M/E ratio

5. skeletal deformities (e.g. frontal bossing)

Front 21

M/E ratio = myeloid to erythroid; normally =

Back 21

3:1

Front 22

(dec. RC lifespan is no longer used as:

Back 22

a measure for hemolysis

Front 23

3 biochemical consequences of hemolysis:

Back 23

1. inc. LDH

2. inc. unconjugated bilirubin

3. dec. serum haptoglobin

Front 24

LDH is released after lysis of:

Back 24

ANY cell

Front 25

unconjugated bilirubin is a metabolite of:

Back 25

chewed-up Hb

Front 26

dec. serum haptoglobin specifically signifies:

Back 26

*intra*vascular hemolysis

Front 27

serum haptoglobin is a mlcl that circulates and binds free Hb; because it _________________, it can be used as a measure of intravascular hemolysis

Back 27

dies QUICKLY

Front 28

what is the most common defect leading to anemia?

Back 28

hereditary spherocytosis

(a congenital disorder)

Front 29

features of hereditary sphreocytosis:

(6)

Back 29

1. ~~ Europeans

2. AD

3. ~~ lack of central pallor

4. ~~ defect in in prot's of memb. skel => loss of memb => loss of SA

5. ~~ aplastic crises

6. pts can have hemolysis even after minor inf's

Front 30

HS is diagnosed by:

Back 30

*osmotic fragility*

Front 31

treatment of HS =

(2)

Back 31

1. folate

2. splenectomy (if serious0

Front 32

a splenectomy in Hereditary Spherocytosis will abate hemolysis, but:

Back 32

spherocytes will persist

Front 33

G6PD is an enzyme that:

(2)

Back 33

1. detoxifies metabolites of oxidative stress

2. eliminates methemoglobin

Front 34

low G6PD (a congenital defect) => your RC's are more susceptible to:

Back 34

oxidative stress

=> Heinz bodies => bite/blister cells

Front 35

3 facets of G6PD deficiency:

Back 35

1. X-linked

2. blacks lose good G6PD activity as RC's age

3. 10-14% of US black men have this

Front 36

***biggest things with G6PD deficiency is to avoid:***

Back 36

oxidizing agents

Front 37

7 oxidizing agents to avoid in G6PD deficiency:

Back 37

1. anti-malarials

2. sulfa drugs

3. dapsone

4. Vit K

5. fava beans

6. mothballs

7. infections

Front 38

ind's with G6PD deficiency compensate for hemolysis by:

Back 38

increasing reticulocyte production

Front 39

G6PD Def: immediately after a hemolytic episode, G6PD levels in Africans may be NORMAL, since only:

Back 39

young cells (with normal G6PD for now) are around (mature cells have already lysed)

Front 40

RC's can be coated with ______ OR _______

Back 40

AB's, C3

- to be destroyed

Front 41

autoimmune destruction of RBC's is an _____________ condition

Back 41

acquired

Front 42

autoimmune destruction of AB's is either:

(2)

Back 42

1. warm-AB-mediated

OR

2. cold-AB-mediated

Front 43

features of warm AB's:

(3)

Back 43

1. react with RC's best at 37 deg.

2. do NOT cause agglut.

3. ~~ IgG-coated RC's

Front 44

features of cold AB's:

(3)

Back 44

1. react with RC's best at <32 deg.

2. DO cause agglut.

3. ~~ IgM

Front 45

**hallmark test of AutoImmune HA = **

Back 45

+ Coomb's test

Front 46

+ Coomb's result =

Back 46

clumping of RC WITHIN test tubes

Front 47

direct Coomb's (aka DAT) tests for:

(2)

Back 47

IgG AND C3 bound *directly* on the RC's

- a negative result is *neither* IgG nor C3 bound to your RC's

Front 48

indirect Coomb's looks for:

Back 48

IgG in the *serum*

Front 49

signs of Warm AIHA:

(8)

Back 49

1. + indirect Coomb's

2. + direct Coomb's

3. **spherocytes**

4. inc. retic's

5. inc. bilirubin

6. inc. LDH

7. splenomegaly

8. jaundice

Front 50

in Warm AIHA, pts MAY be:

Back 50

anemic

- not necessarily

Front 51

Warm AIHA can also be induced by:

(2)

Back 51

1. drugs

2. autoimmune platelet destruction (as in Evan's syndrome)

Front 52

treatment for Warm AIHA:

(2)

Back 52

1. **immunosuppression**

2. transfusions (if anemic)

Front 53

immunosuppression usually takes the form of:

(3)

Back 53

1. corticosteroids

2. splenectomy (if serious)

3. Rituximab)

Front 54

Cold AIHA =

Back 54

IgM AB's bind RC's in **extremities**

=> attracts C3 => lysed when they come back to the torso

Front 55

due to C3 coating, Cold AIHA RC's agglutinate ==>

Back 55

obstruction in microvasculature

=> cyanosis, ishemia in extremities

Front 56

Cold AIHA comes from:

(3)

Back 56

1. the cold

2. inf's from mononucleosis, mycoplasma

3. lymphoproliferative diseases

Front 57

treatment of Cold AIHA:

(3)

Back 57

1. keep warm

2. immunosuppression MAY be required

3. ***splenectomy and steroids are INeffective***

Front 58

MAHA is a __________________ condition

Back 58

non-immune

Front 59

MAHA =

Back 59

disorder in which obstruction by of microvasculature by fibrin mesh results in distortion and fragmentation of RC's

=> hemolysis

=> anemia.

Front 60

hallmark of MAHA =

Back 60

**schistocytes**

Front 61

MAHA is caused by:

(2)

Back 61

1. TTP/HUS

2. DIC

Front 62

which 2 WBC's are NOT granulocytes?

Back 62

lymphocytes and monocytes

Front 63

band forms = stabs =

Back 63

immature PMN's

- nucleus is not yet segmented

Front 64

functions of PMN's:

(2)

Back 64

1. eat bact

2. chemotaxis

Front 65

eosinophil appearance and function:

(2)

Back 65

1. oranges with sunglasses

2. all PMN functions + attack parasites + reg. of HS rxns

Front 66

monocytes= largest WBC's; appearance and function (3):

Back 66

uneven nucleus

1. phag.

2. antigen presentation

3. release of cytokines

Front 67

basophil appearance and function (2):

Back 67

deeply-purple granulations

1. inflammation

2. HS rxns

Front 68

elevated PMN count occurs in:

(6)

Back 68

1. phys (preg, exercise, neonates)

2. acute inf's

3. acute inflammation

4. myeloproliferative disorders, esp. CML

5. corticosteroids

6. LAD

Front 69

myelo ~~

Back 69

marrow

Front 70

neutropenia occurs in:

(5)

Back 70

1. blacks, normally
(if they don't have recurrent bact inf's, then it's benign)

2. drugs

3. chemo agents

4. immune, e.g. lupus

5. inf's

Front 71

neutropenia esp. occurs with these drugs:

(4)

Back 71

1. anti-psychotics

2. anti-epileptics

3. anti-thyroids

4. some antibiotics

Front 72

agranulocytosis =

Back 72

complete or near absence of **PMN's**

Front 73

agranulocytosis is nearly ALWAYS:

Back 73

**drug-induced**

Front 74

drugs that tend to induce agranulocytosis:

(6)

Back 74

1. clozapine,

2. propyth,

3. anti-convulsants,

4. sulfa,

5. chloram,

6. cocaine cut with Levamisol

Front 75

symptoms of agranulocytosis:

(2)

Back 75

1. severe necrotizing ulcers in mouth and throat

2. **severe risk for life-threatening inf's**

Front 76

eosinophilia is caused by:

Back 76

Neoplasm

Allergy/asthma

Addison's

Collagen def.

Parasites

Front 77

basophilia ~~

Back 77

myeloproliferative disorders

Front 78

lymphocytosis is usually the result of:

Back 78

viral infections

(seen in CLL, a disease of old people)

Front 79

lymphopenia is caused by:

(4)

Back 79

1. immune-deficiencies

2. immuno-suppressive drugs

3. lymphomas

4. granulomatous diseases

Front 80

acquired defects in PMN function are caused by:

(5)

Back 80

1. cortico-steroid use

2. alcoholism

3. leukemias

4. myelodysplasia

5. myeloproliferative disorders

Front 81

normal lifespan of plat's =

Back 81

10 days

Front 82

platelet dysfunction manifests itself as:

(2)

Back 82

1./2. oozing and bruising

3. arterial occlusion (=> gangrene in the limbs)

Front 83

petechiae/purpura tend to appear in:

Back 83

the LOWER extremities

Front 84

thrombocythemia =

Back 84

thrombocytosis = too many platelets

Front 85

when looking at a potential platelet disorder, you MUST rule out:

Back 85

pseudothrombocyopenia

Front 86

pseudothrombocyopenia is AKA:

Back 86

plat. clumping

- plat count is artificially low, as m'd by the machine, due to a substance in some people that makes them clump

- NO clinical sequelae to PTCP

Front 87

100-150K plat's ~~

Back 87

clinically silent - no symptoms

Front 88

first symptoms of platelet deficiency appear b/w:

Back 88

20 and 50K plat's

Front 89

<10K plat's = risk for:

Back 89

spontaneous intracranial hemorrhage

- need immediate treatment

Front 90

thrombocytopenia can be achieved by messing with the marrow, as happens with:

(2)

Back 90

1. marrow infections/mets

2. toxins (ESP. chemo and heavy alcohol)

Front 91

DIC and TTP are 2 ____________________ conditions that cause thrombocytopenia

Back 91

***non-immune***

(both => peripheral destruction)

Front 92

DIC =

Back 92

abnormal activation of coagulation and generation of thrombin

=> consumption of clotting factors, destruction of platelets, activation of fibrinolysis

=> more likely to bleed out b/c the materials are already being used

Front 93

**DIC is diagnosed by:**

(5)

Back 93

1. **elevated PT**

2. low plat's

3. low/falling fibrinogen

4. inc. FDP/D-Dimers

5. (sometimes) schistocytes

Front 94

treatment of DIC:

(2)

Back 94

1. treat UC (HUGE)

2. supportive: transfusion of plat's, clotting factors, and fibrinogen

Front 95

DIC occurs with:

(5)

Back 95

1. severe burns

2. shock

3. insect/snake bites

4. OB disasters

5. trauma

Front 96

TTP (thrombotic thrombocytopenic purpura) =

Back 96

abnormal activation of plat's b/c of VWF and fibrin deposition in the microvasculature

=> ***many thromboses throughout the body***

Front 97

TTp occurs as a result of:

Back 97

AB's targeted to ADAMTC protease

=> no proteases

=> VWF is huge and multimeric

=> accumulates throughout the body

=> takes up plat's

=> forms clots throughout the body

Front 98

TTP is diagnosed by the Pentad:

Back 98

1. **MAHA**

2. **low plat's**

3. fever

4. neuro problems

5. renal problems

Front 99

***MAHA is evidenced by:***

(3)

Back 99

1. inc. LDH

2. inc. bilirubin

3. schistocytes

Front 100

no schistocytes =

Back 100

no TTP

Front 101

TTP is NOT familial, but _______________

Back 101

sporadic

(inc. incidence in preg/AIDS)

Front 102

TTP can also be induced by these drugs:

(3)

Back 102

1. quinine

2. cyclosporine

3. tacrolime

Front 103

**treatment of TTP = **

Back 103

PLEX

Front 104

fatality rate of TTP without PLEX =

Back 104

>90%

Front 105

**secondary measures for TTP:**

(2)

Back 105

1. splenectomy

2. Rituximab for relapse

Front 106

Hemolytic Uremic Syndrome often comes with TTP; features =

(3)

Back 106

1. more renal than neuro problems

2. comes from Shiga toxin of Shigella or E. coli

3. plex for adults, supportive care for kids

Front 107

**drugs that induce thrombocytopenia:**

(4)

Back 107

1. B-lactam antibiotics

2. sulfa's

3. quinine

4. heparin

Front 108

***if a pt's plat's fall while on heparin,

Back 108

STOP the transfusion immediately

- might paradoxically lead to thrombosis

Front 109

ITP = immune/idiopathic thrombocytopenia purpura; there is NO:

Back 109

diagnostic test for it

- needs to be arrived at by process of elimination

Front 110

other ITP features:

(2)

Back 110

1. megakaryocytes present in the marrow

2. remits spontaneously in children; caused by virus

Front 111

in adults with ITP, need to treat if:

(2)

Back 111

1. plat's <30K

or

2. pt is bleeding

Front 112

first-line treatment for ITP =

Back 112

corticosteroids

- takes 48-72 days

Front 113

if pt is bleeding of has <10K plat's, corticosteroid treatment isn't fast enough and you need to use:

Back 113

IVIG

Front 114

2/3 of ITP pts won't respond to steroids or will relapse after taper - need to give them:

Back 114

splenectomy

- if refractory to splenectomy, need intense immunosuppression via Rituximab

Front 115

splenic sequestration: normally, only 1/3 of total plat's reside in the spleen; splenic enlargement from any cause =>

Back 115

spleen sequesters plat's

=> serum plat's fall

Front 116

officially, plat's can be given to:

(2)

Back 116

1. treat

2. *prevent* bleeding

- but blood bank has a number above which they will NOT give plat's

Front 117

so in cases of severe bleeding in ITP or splenic sequestration, plat's are given; otherwise, they're:

Back 117

contra-indicated

- plat's are also given for DIC, but NOT for TTP

Front 118

for pts with plat. hypoproduction, trigger to transfuse plat's =

Back 118

<10K plat's OR pt is bleeding

Front 119

thrombocytosis is a result of either primary or secondary causes; primary cause of thrombocytosis =

Back 119

myeloproliferative disorders

Front 120

secondary/reactive causes of thrombocytosis:

(4)

Back 120

1. inflammation

2. inf

3. bleeding

4. iron deficiency

(treat UC)