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STEP 2 CK FIRST AID > Heme/Onc > Flashcards

Flashcards in Heme/Onc Deck (63):
1

What is the goal INR for a patient with a mechanical heart valve?

2.5-3.5

2

What initiates the intrinsic pathway? extrinsic pathway?

Intrinsic = collagen exposure following vascular trauma
Extrinsic = endothelium produced TF

3

What causes the paradoxical hypercoagulability when taking warfarin?

This occurs at first b/c warfarin inhibits Protein C and S BEFORE it inhibits factors 2,7,9,10.

4

What is found in cryoprecipitate?

Factor VIII, fibrinogen mainly (this is why you choose cryo over FFP)

some factor XIII, vWF and fibronectin,

5

What is the tx for hemophilia?

Cryoprecipitate

DDAVP can also be used in mild hemophilia (helps the body produce more factor VIII)

6

What is the most common inherited bleeding disorder?

vWF deficiency. It is much milder than hemophilia and results from lack of VIII.

7

What drug worsens VWF disease? Why don't you see petechiae with this disease?

ASA will worsen the bleeding. No petechiae b/c the platelet dysfunction is not that bad.

8

What will labs for VWD look like? Dx? Tx?

prolonged aPTT, prolonged bleeding time
Normal PT and platelet count

Dx = Ristocetin cofactor assay
Tx = DDAVP

9

What is the MOA of clopidogrel and ticlopidine?

They block the ADP receptor, needed for platelet aggregation

10

What is the most common hereditary thrombotic disease? MOA?

factor V Leiden - a polymorphism that makes it resistant to inactivity by activated protein C (factor V is stuck in the "on-position" and makes a ton of activated thrombin (more clots.)

11

How long do you tx w warfarin for first thrombotic event? After doing heparin of course.

3-6 months
6-12 months for second event
lifelong for everything else

12

What is the difference in terms of labs between severe liver disease and DIC?

In DIC, the factor VIII is depressed.

13

What 5 characteristics/signs of TTP?

Low plts
microangiopathic hemolytic anemia
neuro changes (delirium, seizure, stroke)
impaired renal function
fever

14

What differentiates HUS from TTP?

HUS will have high Cr levels

HUS, TTP, and DIC are the only causes of microangiopathic hemolytic anemia

15

What tx is contraindicated in TTP? what tx should you use?

platelet transfustion - this will worsen the condition due to platelet aggregation and microvascular thrombosis.

Tx: steroids to bust the microthrombi, and plasma replacement/plasmapheresis

16

What are the labs in DIC and TTP?

DIC = low clotting factors, plts, fibrinogen. High PT, PTT, d-dimer, and fibrin split products

TTP: everything is normal except low plts.

17

What is the Tx for HUS?

Supportive. No Abx as this could delay toxin expulsion

18

What is the Tx for ITP?

Dont really have to treat acute/childhood forms as these remit spontaneously.

For Chronic form, use corticosteroids, IVIG, and splenectomy (last resort if CTSDs don't work after 6 months.

19

What is a high retic count? What does it indicate?

>2.5% is high, this indicates hemolysis/hemorrhage is occuring and BM is responding by making more RBCs

20

What are the 4 causes of microcytic anemia?

TICS

Thalassemia
Iron Def
Chronic Disease
Sideroblastic anemia

21

What Heme test is HIGHLY SPECIFIC for iron deficiency anemia?

Elevated RDW

22

What is the serious side effect that could occur with IV iron dextran administration?

Anaphylaxis

23

What are the lab differences between iron def anemia and anemia of chronic disease?

Fe Def = low serum iron and ferritin, high TIBC (transferrin) and transferrin R

CD = low serum iron and TIBC (transferrin), high ferritin, normal transferrin R

Both: low serum iron, normal TIBC (transferrin, ferritin, and transferrin R

24

Which class of drugs can cause megaloblastic anemia?

Chemotherapeutic drugs

25

What organism can cause an infection which causes B12 def anemia?

Diphyllobothrium Latum. its a tapeworm. It can cause neuro issues as well (b12 you know.)

26

How will MMA and homocysteine levels look like in B12 and folate def anemia?

B12 = hi MMA, low homo
Folate = normal MMA, hi homo

27

What do sperocytes look like? What do they indicate?

They are RBCs that lack central pallor (all 1 color)

They indicate autoimmune hemolysis or hereditary spherocytosis

28

EBV can cause autoimmune RBC destruction

So can mycoplasmal infection, CLL, RA, or meds

29

Reticulocytes will be elevated in hemolytic anemias

Will also have high LDH.

A + Coombs test will diagnose an autoimmune hemolysis

30

Anemic person has a pancytopenia, some cafe au lait spots, short stature, and radial/thumb hypoplasia. Dx? What will the bone marrow bx show?

Fanconi's Anemia. = hereditary destruction of bone marrow cells

Bone marrow bx shows hypocellularity, and space occupied fat

31

Where is the issue in the RBCs in sicklers? Why is this an issue?

They have a B-chain malformation (glu --> val) This causes shorter RBC life which can lead to vaso-occlusion

32

What is the most common cause of osteo in sicklers?

S.A. but they also get salmonella! Watch out for the question being asked!

33

What is the tx for sickle cell?

Hydroxyurea, it stimulates the fetal hemoglobin production

34

What health maintenance should sicklers

PEN BID till age 5

35

Which drug increases the PTT? Which increases the PT?

PTT (intrinsic pathway) = Heparin
PT (extrinsic pathway) = Warfarin

36

What does the hemoglobin electrophoresis evaluation look like in a-thalassemia?

It looks normal! Although this is usually the way to Dx a thalassemia

37

What do pts with 0/2 B chains look like compared to 1/2 B chains?

0/2 chains = severe microcytic anemia and will need chronic transfusions/marrow transplant to survive!

1/2 chains = asymptomatic, but cells are microcytic and hypochromic on peripheral smear.

38

What do pts with 0/4 - 3/4 a chain thalassemia look like?

0/4 chains = pts die in utero
1/4 chains = hi reticulocyte count and skeletal changes due to expanded erythropoiesis
2/4 and 3/4 chains = usually asymptomati

39

What is the mutation that causes polycythemia vera? Tx?

JAK2 mutation causing clonal proliferation of a pluripotent marrow stem cell. All marrow lines will be increased! RBCs significantly affected.

Tx: hydroxyurea and ASA

40

What is the cause of a nonhemoltytic febrile transfusion reaction?

cytokines formed during storage of blood cause fever, chills, rigors, and malaise

41

Signs and symptoms of porpheria? Triggers? Tx for acute attacks?

photodermatitis, neuropsychiatric complaints, colicky abdominal pain, PINK or BROWN URINE the day after drinking/benzo use

Triggers = alcohol, barbs, OCPs

Tx = glucose (will stop the heme synthesis by providing negative feedback to heme synthetic pathway.)

42

What is the most common childhood malignancy? signs?

what will you find on bone marrow bx/aspiration?

ALL

kids will present with bone pain, TCP, and anemia
swollen bleeding gums, hepatosplenomegaly

bone bx will show blasts (myelo or lympho)

43

What would a pt with a very high WBC count in a pt with an acute leukemia be at risk for?

leukostasis (blasts occluding the microcirculation) which can cause pulmonary edema, ischemic injury, or DIC.

Remember, these WBCs are not very functional

44

You see cells that are MUCH larger than RBCs w/ round/kidney shaped nuclei and Auer rods on blood smear and myeloperoxidase...

Tx for APL type?

AML (type M3 APL to be exact.)

APL subtype is treated with all-trans-retinoic acid (ATRA) therapy. translocation between c15-17

45

You see cells that are about the same size as an RBC w/ large nuc:cytoplasmic ratio and visible clefts in their nuclei and NO myeloperoxidase...

ALL

46

Tumor lysis syndrome? How can this be avoided? Tx?

Renal insufficiency resulting from blast lysis during chemo. (can happen during CLL or NHL)

also - hyperkalemia, phosphatemia, uricemia, and hypocalcemia

This can be avoided by checking WBC count before chemo sessions, if it is high then start allopurinol to prevent hyperuricemia

If this is not treated before hand, use hydroxyurea in the setting of leukostasis to decrease WBC rapidly.

47

What will flow cytometry show for a pt with CLL?

smear?

Tx:

CD20 and CD21 (B cell markers, which would be expected in a B cell proliferative dz) but also CD5 (normally found on T cells)

smear will show a TON of small lymphocytes and SMUDGE CELLS and hypogammaglobulinemia

Tx: Tx is withheld until pts are symptomatic (recurrent infections, splenomegaly, anemia) and then the tx is PALLIATIVE.

Its ok, this happens in really old people

48

Most common translocation with CML?

Tx?

t(9,22) BCL-abl phili chromo

Tx: imatinib (blocks the tyrosine kinase of BCL-abl)

49

71 yo man with lethargy, constipation, dull back pain that is worsening. Normal labs except for hi Cr. Why?

He has Multiple Myeloma

His bad RF is due to urinary immunoglobulins (Bence Jones proteins) that form casts and cause nephropathy

50

What infection do you predict a pt with Hairy Cell Leukemia will present with?

Mycobacterium Avium Intracellulare (atypical)

51

What will the CBC in HCL show?

Tx?

it will show leukopenia (confusing!) and hairy cells on TRAP stain

Tx: nucleoside analogs (cladribine) or a combo of splenectomy and IFN-a

52

What viruses can cause non hodgkin's lymphoma?

What is the most common translocation in NHL?

EBV (although more association with HL), HTLV-1, HCV

translocation = t(14-18)

Most NHL's are of B cell origin, and NHL's are MUCH more common than hodgkins lymphoma

53

What are the symptoms of Hodgkins lymphoma?

Dx?

systemic B symptoms (NS, fever, WL), pruritis, big spleen, Pel-Ebstein fevers (waxing.waning fevers!)

Dx = excisional bx - look for reed sternberg cells

54

What preventative measures are needed in a pt being treated for HL?

mammograph. chemo and radiation can lead to 2* neoplasms like AML, NHL, breast cancer, thyroid cancer

55

What are the 3 criteria for Dx of multiple myeloma?

tx?

>10% plasma cells in bone marrow / plasma cell infiltration
M protein in serum or urine
lytic bone lesion

just M proteins is NOT dx for MM, it can be MGUS or waldenstroms macroglobulinemia at this stage

Also, bone scan may be NEGATIVE b/c this assesses osteoblast function, and MM is an osteoclastic process

Tx: Melphalen (alk agent), prednisone

56

What can hi levels of IgM do to you>

hyperviscosity, coagulation issues, reynauds, cold agglutinin disease (autoimmune hemolytic anemia) and amyloidosis

57

What will labs of MM look like?

marrow?

labs = elevated LDH, ESR, uric acid, alk phos

marrow = PAS+ IgM deposits around the nucleus

58

What amyloids are present in 1* and 2* amyloidosis? where do they deposit?

1* = AL (light chains) associated with MM and waldenstroms
-tx = chemo

2* = AA (acute phase reactant amyloid) associated with chronic inflammatory diseases (RA) or infections
-tx = transplantation or tx underlying inflammation!

Both tend to deposit in the heart, kidneys, and liver

59

What kind of amyloid is present in dialysis?

Dialysis related amyloid deposits B2 microglobulin

60

How do you calculate the absolute neutrophil count (ANC)

tx of neutropenia?

WBC * (%bands + %segs)

An ANC

61

Hematuria with eosinophilia, what is the infection?

schistosomiasis

62

WHAT IS the cutoff for an acute transplant rejection? MOA?
Tx?

5 days - 3 months

MOA for acute is T-cell mediated. hyperacute (minutes) is preformed Abs and GVHD is due to minor histocompatibility Ags.

Tx: CTSDs, OKT3, tacrolimus

63

What malignancy are you concerned for:

1. Girl with facial angiofibromas, seizures, mental retardation
2. atrophic glossitis, esoph webs, and anemia
3. pagets disease of bone
4. acanthosis nigrans

1. This is tuberous sclerosis, be concerned for astrocytoma and cardiac rhabdomyoma

2. this is plummer viston, be concerned for SZqCC of esoph

3. 2* osteosarcoma or fibrosarcoma

4. Visceral malignancy (stomach, lung, breast, uterus)