Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

STEP 1 RECALL > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Pyruvate Kinase deficiency has what effect on RBC’s?

A

last step in glycolysis , no PK–> you can’t generate the ATP from glycolysis in your RBC’s, they become senescent and the spleen removes them Spleen will hypertrophy from this overwork

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

mTOR signaling pathway (the more this is turned on, more cancer)

A

growth factor binds to Tyrosine Kinase –> autophosphorylation of tyrosine –> activation of PI3K –> activation of PK-B (akt) –> akt activates mTOR –> mTOR goes to nucleus to induce genes involved in cell survival / anti apoptosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Soon after a woman is started on Warfarin, she develops areas of skin and fat necrosis… why?

A

Warfarin inhibits all the vit. K dep factors (Factors 2,7,9,10 & protein C & S)—> but proteins C & S have shorter half lives & are therefore depleted first this leaves you with a period of time with no protein C (which normally INactivates factor V & VII) and plenty of the other clotting factors this sets you up for clots & “ Warfarin Skin Necrosis” —> so you must always give Heparin too for the 1st few days of warfarin therapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Protein C function

A

inactivates factors V & VII by proteolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

In HbS – what causes the Hb to be able to polymerize under low oxygen conditions and therefore “sickle”?

A

HbS contains a valine in place of the glutamic acid at position 6 in the beta subunit, this promotes HYDROPHOBIC INTERACTION among the Hb molecules and polymerization

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

HbC and Sickle cell disease are result of ________ mutations

A

Missense mutations (one aa sub’d for another aa) HbC = lysine for glu at position 6 HbS = valine for glu at position 6

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

blasts in peripheral smear w/ TdT + and PAS + material

A

Lymphoblasts (ex: Acute Lymphoblastic Leukemia) -CD 19 & 10 = B cell blasts -CD 1, 2, 5 = T cell blasts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

blasts in peripheral smear w/ peroxidase + granules

A

Auer Rods – this means Myeloblasts (AML) (found abundantly in AML M3)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

treatment of Tumor Lysis Syndrome (hyperkalemia, hyperphosphatemia, hyperuricemia)

A

Rasburicase or Allopurinol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

anemia with decreased Ferritin, increased Transferrin, increased TIBC, MCV < 80

A

Iron deficiency anemia (microcytic, hypochromic anemia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

reasons for Burr cells on peripheral smear

A

Uremia, Pyruvate Kinase deficiency, Microangiopathic hemolytic anemias (TTP & HUS), or mechanical trauma to the RBC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

2,3 BPG effect on O2 binding Hb

A

2.3 BPG allosterically decreases the affinity of Hb for O2 –> makes it easier to dump oxygen in the tissues

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

a pt with ESRD who has not had dialysis for over a week develops excessive bleeding when changing his catheter

A

Uremic platelet dysfunction - only the bleeding time will be prolonged

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

most commonly used drug for long term anticoagulation after a PE

A

Warfarin -inhibits Vit K dependent carboxylation of glutamic acid residues on the Vit K dep clotting factors & inhibits Epoxide Reductase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

drug of choice for ACUTE management of DVT

A

Heparin (activates antithrombin III)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

type of leukemia that is more likely to present with a large mass in the mediastinum or neck, and cause symptoms like dysphagia

A

T-cell ALL (as apposed to B-cell ALL which presents w/ fever, malaise, bleeding, bone pain, hep/splenomegaly)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

in a normal distribution, _____% lie within 1 SD, _____% within 2 SD and _______% within 3 SD’d

A

68% – 1 SD 95% – 2 SD 99.7% – 3 SD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

most common inherited bleeding disorder, causes a prolonged bleeding time and PTT

A

von Willebrand disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what has the main control over Iron absorption and excretion in the body?

A

the liver – specifically Hepcidin -Hepcidin is increased when you don’t need to absorb any more Iron, it binds to and causes downregulation and degradation of Ferroportin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What enzyme converts Heme —> Biliverdin and is thereby responsible for the greenish color of bruises

A

Heme Oxygenase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

woman with heavy periods, her platelet count is normal & they aggregate normally in response to ADP but not to Ristocetin

A

vWF deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

drug treatment for vWF deficiency

A

Desmopressin — b/c ADH stimulates the release of vWF from endothelial cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

A man with non-tender cervical lymph nodes has a biopsy shown here, cytogenetic analysis would likely show overexpression of _______________

A

Bcl-2

  • Follicular lymphoma
  • t(14,18)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

patient with mild jaundice, RBC peripheral smear is shown, the dx was made by osmotic fragility testing & an increased MCHC

A

Hereditary Spherocytosis

-no central pallor, spherocytes seen on smear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

megaloblastic anemia with foot numbness, difficulty walking , took years to develop b/c of large stores of _______ in the liver

A

B12 (cobalamin) deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

t(15,17) – would likely be associated with a malfunctioning _______ receptor

A
  • M3 AML (Acute Promyelocytic Leukemia)
  • assoc w/ defect in RETINOIC ACID RECEPTOR
  • hence you treat w/ ATRA
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

a mutation 3 bases upstream from the AUG start codon on a mRNA would impair ________

A

-mutation in the “Kozak sequence” causes impaired mRNA binding to ribosomes and INITIATING translation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What cancer drug’s toxicity is increased if given with Allopurinol?

A

6-Mercaptopurine

  • b/c it’s metabolized by Xanthine Oxidase
29
Q

mechanism of Celecoxib

A
  • Selective COX 2 inhibitor
  • does not cause GI problems or decr platelet aggregation
30
Q

Common precipitating factors of G6PD deficiency anemia:

A

1- Infection

2- Drugs (Dapsone, Malaria drugs, TMP-SMX)

3- DKA

4- Fava beans

31
Q

a bone marrow sample w/ > 30% plasma cells in diagnostic for ____________

A

Multiple Myeloma

32
Q
  • IL-1, IL-6 –> bone resorption , “punched out” lytic lesions on Xray
  • Rouleaux formation
  • Bence -Jones proteins (monoclonal light chains) in the urine
  • Amyloid deposits
A

all characteristic of **Multiple Myeloma **

33
Q

What kind of cancer is associated with this cell?

A

Hodgkin’s Lymphoma

((Reed Sternberg cell))

34
Q

Use & Mechanism of HYDROXYUREA

A
  • treatment of Sickle Cell Anemia
  • increases the amount of HbF (which protects against sickling)
35
Q

tumor with a high Ki-67 fraction (nearing 100%)

A

Burkitt’s Lymphoma (as a high mitotic index)

36
Q

things that can induce Sickling

A
  • low oxygen states
  • acidity or high 2,3 DPG
37
Q

Upper respiratory tract infection + pancytopenia

A

Parvovirus (can cause aplastic anemia)

38
Q

hypocellular marrow filled with fat and fibrosis

A

Aplastic anemia

39
Q

mainstay of DVT treatment in a pregnant woman

A

Heparin

(Warfarin is a teratogen & Aspirin & Clopidogrel are insufficient to treat a DVT)

40
Q

increased indirect bilirubin

A

should associate w/ _hemolysis / hemolytic anemias _

41
Q

the bluish color of reticulocytes is due to ______

A

increased r RNA

42
Q

child w/ Down Syndrome – at risk for ___________ leukemia before age 5 and _________ leukemias after age 5

A

before age 5 = megakaryoblastic leukemia

after age 5 = ALL

43
Q

Diseases associated with target cells

A

“HALT said the hunter to his Target”

  • HbC disease
  • Asplenia
  • Liver disease
  • Thalassemia
44
Q

conditions associated w/ Basophilic stippling of the rbc

A

“Baste the ox *TAIL*“

  • Thalassemias
  • Anemia of chronic dz
  • Iron deficiency
  • Lead poisoning
45
Q

Megaloblastic anemia with INCREASED methylmalonic acid

A

B12 deficiency

(folate deficiency has normal mm acid)

46
Q

Pt w/ pallor, early satiety, splenomegaly, fatigue – found to have a Jak 2 mutation that causes constitutive tyrosine kinase activity and STAT activation…

A

**Myelofibrosis **

  • excess megakaryocytes –> produce PDGF –> causes marrow fibrosis
  • the splenomegaly can cause early satiaty & abd discomfort

((The 2 other chronic myeloproliferative disorders –> Polycythemia Vera & Essential Thrombocythemia also have a Jak2 mutation))

47
Q

Leukemia that classically has increased BASOPHILS

A

CML

  • t(9,22) , Bcr-ABL
  • Basophilia, translocation, and a negative Leukocyte Alk Phos (diff from leukemoid rxn)
48
Q

Beta- Thalassemias are caused by __________

A

mutations that cause defective mRNA processing & therby low levels of globin chain synthesis

49
Q

lab abnormalities found in HUS

A
  • prolonged Bleeding Time (due to decrs platelets)
  • incrs BUN, Cr & LDH
50
Q

bleeding after unfractionated Heparin treatment should be treated w/ ________

A

Protamine sulfate

51
Q

increased levels of HbA2 indicate ______

A
  • Beta thalassemia minor (trait)
  • or B-Thalassemia intermedia
52
Q

the _____ will be normal in pt’s with CO poisoning, Anemia, Polycythemia, and Methemoglobinemia…

A

the partial pressure of Oxygen in the blood (the amount dissolved in plasma - doesn’t change even if the Hb isn’t binding O2)

53
Q

Pancytopenia in a patient with SLE is due to _______

A

formation of autoantibodies against blood cells ( a type II hypersensitivity rxn)

54
Q

a patient with recurrent DVT’s and PE’s , the PT & PTT are normal, and the PTT does not change with addition of activated protein C…

A

-Factor V Leiden mutation

(normally activated protein C would inactivate factor V and prolong the PTT)

55
Q

Why are patients w/ sickle cell dz (or another chronic hemolytic dz) predisposed to becoming Folic acid deficient and developing macrocytic changes

A
  • b/c they are constantly turning over erythrocytes and making new ones (using up folic acid)
56
Q

phosphorylation of the Rb gene causes it to be ____________

A

-inactive (ie: let down it’s guard of the genome and ALLOW progression from G1 –> S phase)

57
Q

Her-2/Neu is also known as _________

A

ERB-B2

58
Q

the N-myc proto-oncogene is overexpressed in __________

A

Neuroblastoma

59
Q

the chemo drug Etoposide, works by ________

A

inhiibiting the activity of Topoisomerase II (causing double strand breaks to accumulate in the cancer cells)

60
Q

Small cell carcinoma of the lung is thought to have a ___________ origin

A

Neuroendocrine (will stain for markers such as chromogranin, synaptophysin…)

61
Q

How do you calculate Atrributable Risk %

A

ARP = (RR -1) / RR

or (Risk in the exposed - risk in unexposed) / Risk in the exposed

62
Q

most common indolent (lymph node gets bigger, then smaller in cycles) form of non-Hodgkin’s lymphoma in adults

A

Follicular lymphoma

63
Q

T-cell lymphoma, affects skin causing “Pautrier microabscesses”

A

Mycosis Fungoides

(CD4+ cells invade skin)

64
Q

splenomegaly, pancytopenia in an older man, leukemic cells are + for TRAP (tartrate resistant acid phosphatase)

A

Hairy Cell Leukemia

65
Q

formula for “Number needed to harm”

A

NNH = 1/ Attributable risk

66
Q

What drug can be used to prevent the nephrotoxicity from Cisplatin (a DNA crosslinker) treatment?

A

Amifostine (and hydration)

67
Q

mechanism of Cladribine

A
  • a purine analog that gets to high concentrations within the cell b/c it is resistan to degradation by ADA
  • tx of choice for Hairy Cell Leukemia
68
Q
A

STEP 1 RECALL (12 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions