Brainscape's Knowledge GenomeTM

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


See full index

Board questions > HemeOnc First Aid > Flashcards

HemeOnc First Aid Flashcards

1
Q

What do RBC membranes contain?

A

Cl-/HCO3- antiporter; allows export of HCO3 and transport CO2 from periphery to lungs

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

What granules do thromboyctes have and what do they contain?

A
  • dense granules
    • ADP
    • Ca2+
  • alpha granules
    • vWF
    • fibrinogen
    • fibronectin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

vWF receptor is:

Fibrinogen receptor is:

A

GpIb

GpIIb/IIIa

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

What do specific granules of neutrophils contain?

A

leukocyte alkaline phosphatase (LAP), collagenase, lysozyme, and lactoferrin

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

what do azurophilic granules (lysosomes) of neutrophils contain?

A

proteinases, acid phosphatase, myeloperoxidase, and B-glucuronidase

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

Hypersegmented neutrophils are seen in

A

Vit B12/folate deficiency

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

increased number of band cells reflect states of

A

increased myeloid proliferation (bacterial infections, CML)

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

found in the blood, this cell has a large, kidney shaped nucleus and extensive “frosted glass” cytoplasm

A

monocyte

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

What role do macrophages have in precipitating septic shock?

A

Lipid A from bacterial LPS binds CD14 on macrophages to initiate septic shock

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

causes of eosinophilia include

A

NAACP

  • Neoplasia
  • Asthma
  • Allergic process
  • Chronic adrenal insufficiency
  • Parasites (invasive)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What do basophils contain?

A

heparin and histamine

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

basophilia is uncommon but can be a sign of

A

myeloproliferative disease, esp CML

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

What do mast cells release once they bind the Fc portion of IgE and IgE crosslinks?

A

histamine, heparin, tryptase, and eosinophil chemotactic factors

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

What do dendritic cells express on their surface? What are these cells called in the skin?

A

MHC class II and Fc receptors

Langerhans cells in the skin

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

Once mature, B cells migrate to peripheral tissues including

A

follicles of lymph nodes, white pulp of spleen, and unencapsulated lymphoid tissue

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

All T cells have what CD marker?

A

CD28 (costimulatory signal) necessary for T cell activation

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

What cells have “clock-face” chromatin distribution and eccentric nucleus, abundant RER, and well-developed Golgi?

A

plasma cell

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

vWF is from where?

A

Weibel-Palade bodies of endothelial cells and alpha-granules of platelets

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

What drugs inhibit GpIIb/IIIa directly?

A

abciximab, eptifibatide, and tirofiban

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

What activates vWF to bind GpIb?

failure of aggregation of this is seen in…

A

ristocetin

von Willebrand disease and Bernard-Soulier syndrome

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

what does an acanthocyte look like and what pathology is associated with it?

A

spur cell

Liver disease, abetalipoproteinemia (states of cholesterol dysregulation)

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

What is a dacrocyte and when would I see it?

A

teardrop cell

bone marrow infiltration (eg myelofibrosis); mechanically squeezed out of its home in the bone marrow

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

What is the official name of a bite cell?

A

Degmacyte

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

When would I see echinocytes?

A

burr cells

ESRD, liver disease, pyruvate kinase deficiency

projections more uniform than acanthocyte

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

When do you see target cells?

A

HbC disease, Asplenia, Liver disease, and thalassemia

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

4 alpha globin allele deletions

A

Hb Barts - gamma4

hydrops fetalis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

3 alpha-globin allele deletions

A

HbH (beta 4)

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

secondary hemochromatosis, “crew cut” on skull XR, “chipmunk facies”, increased risk of Parvovirus B19

A

Beta-thalassemia major

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

lead inhibits ferrochelatase and ALA dehydratase leading to

A

decreased heme synthesis and increased RBC protoporphyrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Ssx of lead poisoning?

A
  • Lead lines on gingivae (Burton lines) and on metaphyses of long bones on XR
  • Encephalopathy and erythrocyte basophilic stippling
  • Abdominal colic and sideroblastic anemia
  • Drops - foot and wrist
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What is used for lead chelation in kids?

A

succimer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

genetic cause of sideroblastic anemia is

A

Xlinked defect in ALA synthase gene

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

How do you differentiate orotic aciduria from ornithine transcarbamylase deficiency?

A

both will have increase orotic acid, but ornithine transcarbamylase deficiency will also have hyperammonemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

in orotic aciduria, what is the defect in?

A

defect in UMP synthase with inability to convert orotic acid to UMP (de novo pyrimidine synthesis pathway)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Historically, how was vit B12 deficiency diagnosed?

A

diagnosed with Schilling test, a 4 stage test that determines if the cause is dietary insufficiency v malabsorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Nonmegaloblastic macrocytic anemia is likely caused by…

RBCs still have macrocytosis but without

A

alcoholism, liver disease

hypersegmented neutrophils

37
Q

rapid onset of anemia within first year of life due to intrinsic defect in erythroid progenitor cells

dx?

what is a major physcial feature of this person?

A

Diamond Blackfan anemia

triphalangeal thumbs

38
Q

notable causes of intravascular hemolysis

A

mechanical hemolysis

paroxysmal nocturnal hemoglobinuria

microangiopathic hemolytic anemias

39
Q

hemolytic anemia in a newborn

A

pyruvate dehydrogenase deficiency

AR, decrease ATP, rigid RBCs, extravascular hemolysis

increase 2,3BPG leads to decreased hemoglobin affinity for O2

40
Q

coombs negative hemolytic anemia, pancytopenia, and venous thrombosis

A

paroxysmal nocturnal hemoglobinuria

41
Q

What labs will you see with PNH?

A

CD55/59 negative RBCs on flow cytometry

42
Q

Porphyria cutanea tarda condition affects what enzyme?

A

uroporphyrinogen decarboxylase (AD mutation)

43
Q

Prophobilinogen deaminase, previously known as uroprophyrinogen I synthase has an autosomal dominant mutation in what condition?

A

acute intermittent porphyria

44
Q

acute intermittent porphyria has what symptoms?

How is this treated?

A
  • painful abdomen
  • Port-wine colored urine
  • Polyneuropathy
  • Psychological disturbances
  • Precipitated by drugs (P450 inducers, alcohol, starvation)

tx with glucose and heme, which inhibit ALA synthase

45
Q

PT tests function of what factors?

A

common and extrinsic pathway (I, II, V, VII, X)

46
Q

PTT tests function of what factors?

A

common and intrinsic pathway (all factors except VII and XIII)

47
Q

In a mixing study, where normal plasma is added to a pt’s plasma, how do you know if you have a clotting factor deficiency v factor inhibitor?

A

clotting factor deficiencies should correct (PT or PTT returns to normal) whereas factor inhibitors will not correct

48
Q

hemophilia A, B, and C are deficiencies of what factors?

What are their inheritence patterns?

A

A - factor VIII (X linked recessive)

B - factor IX (X linked recessive)

C - factor XI (autosomal recessive)

49
Q

What will vitamin K deficiency do to PT, PTT and bleeding time?

A

increase both PT and PTT

bleeding time is normal

50
Q

inhibition or deficiency of ADAMTS 13 causes

A

Thrombotic thrombocytopenic purpura

decreased degradation of vWF multimers; increased platelet adhesion, increased platelet aggregation and thrombosis

51
Q

Anti-GpIIb/IIIa antibodies are seen in

A

immune thrombocytopenia

52
Q

defect in platelet integrin alpha IIB beta3 (GpIIb/IIIa) leading to defect in platelet-to-platelet aggregation, and therefore platelet plug formation

normal platelet count

A

Glanzmann thrombasthenia

53
Q

Defect in platelet plug formation causing large platelets, decreased GpIb (Defect of platelet to vWF adhesion)

A

Bernard-Soulier Syndrome

54
Q

tx for von Willebrand disease?

A

desmopressin, which releases vWF stored in endothelium

55
Q

What are complications of Factor V Leiden?

A

DVT, cerebral vein thromboses, recurrent pregnancy loss

56
Q

thrombotic skin necrosis with hemorrhage after administration with warfarin, I am thinking…

A

pt has protein C deficiency

57
Q

What does cryoprecipitate have in it?

A

fibrinogen, factor VIII, factor XIII, vWF, and fibronectin

58
Q

Why is there a risk of hyperkalemia with blood transfusions?

A

RBCs may lyse in old blood units and then get transfused with their contents everywhere

59
Q

What are the four types of Hodgkin lymphoma?

A
  • nodular sclerosis
  • lymphocyte rich
  • mixed cellularity
  • lymphocyte depleted
60
Q

Genetics of Burkitt lymphoma

A

t(8:14)

c-myc (8) and heavy chain Ig(14)

61
Q

genetics of diffuse large B cell lymphoma

A

alterations in Bcl-2, Bcl-6

(MC type NHL in adults)

62
Q

genetics of follicular lymphoma

A

t(14:18)

heavy chain Ig (14) and BCL-2 (18)

63
Q

presents with painless “waxing and waning” lymphadenopathy

A

follicular lymphoma

64
Q

genetics of mantle cell lymphoma

A

t(11:14)

adult males - cyclin D1 (11) and heavy chain Ig (14), CD5+

65
Q

marginal zone lymphoma genetics and association with

A

t(11;18)

cyclin D1 (11) and BCL-2 (18)

a/w chronic inflammation (eg Sjogren syndrome, chronic gastritis/MALT lymphoma)

66
Q

In this AIDS-defining illness, with variable presentation, mass lesions on MRI need to be distinguished from toxoplasmosis via CSF analysis or other lab tests

A

Primary Central Nervous System Lymphoma

67
Q

atypical CD4+ cells with “cerebriform” nuclei and intraepidermal neoplastic cell aggregates (Pautrier microabscesses)

A

mycosis fungoides/Sezary syndrome (T cell leukemia)

68
Q

ssx of multiple myeloma

A

CRAB

  • hyperCalcemia
  • Renal involvement
  • Anemia
  • Bone lytic lesions/Back pain

Multiple myeloma: Monoclonal M protein spike

69
Q

What disease other than multiple myeloma has an M protein spike =IgM?

How can it be differentiated?

A

Waldenstrom macroglobulinemia

hyperviscosity syndrome (eg blurred vision, Raynaud phenomenon, no CRAB ssx)

70
Q

monoclonal expansion of plasma cells but bone marrow shows <10%

A

Monoclonal gammopathy of undetermined significance (MUGS)

no CRAB ssx, can become multiple myeloma

71
Q

Pseudo Pelger Huet anomaly is

A

a myelodysplastic syndrome

neutrophils with bilobed nuclei typically seen after chemo

72
Q

Kids with ALL and this mutation have a better prognosis

A

t(12:21)

73
Q

smudge cells are seen in

A

CLL

74
Q

What is Richter transformation?

A

CLL/SLL transformation into an aggressive lymphoma, most commonly DLBCL

75
Q

dry tap on aspiration

A

hairy cell leukemia

76
Q

hairy cell leukemia will stain positive for

A

TRAP - tartrate-resistant acid phosphatase

has been replaced by flow cytometry for the most part

77
Q

tx for hairy cell leukemia

A

cladribine, pentostatin

78
Q

Aeur rods are found in

A

AML

79
Q

myeloperoxidase positive cytoplasmic inclusions seen mostly in

A

APL (formerly M3 AML)

80
Q

APL: t(15;17) responds to

A

all-trans retinoic acid (vit A), inducing differentiation of promyelocytes

81
Q

Philadelphia chromosome t(9:22), BCR-ABL is in

A

CML

82
Q

CML can accelerate and transform into

A

AML or ALL (blast crisis)

83
Q

tx CML with

A

bcr-abl tyrosine kinase inhibitors like imatinib

84
Q

what disorders are associated with V617F JAK2 mutation?

A

Chronic myeloproliferative disorders

  • polycythemia vera
  • essential thrombocytopenia
  • myelofibrosis
85
Q

What is erythromelalgia and what does it occur in?

A

severe burning pain and red-blue coloration due to episodic blood clots in vessels of extremities

polycythemia vera and essential thrombocythemia

86
Q

Presents in a child as lytic bone lesions and skin rash or as recurrent otitis media with a mass involving the mastoid bone

A

langerhans cell histiocytosis

87
Q

What are characteristics of cells in langerhans cell histiocytosis?

A

cells are functionally immature and do not effectively stimulate primary T cells via antigen presentation.

Cells express S-100 (mesodermal origin) and CD1a

Birbeck granules (tennis raquets or rod shaped on EM)

88
Q

reverse dapigatran with what drug?

A

idarucizumab

89
Q

What class of drugs do abciximab, eptifibatide, and tirofiban go into?

A

Gp IIb/IIIa inhibitors

Board questions (24 decks)

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