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Step 1 (Steffanie) > Hematology/Oncology > Flashcards

Hematology/Oncology Flashcards

1
Q

Prolonged prothrombin time

A

Defect in extrinsic coagulation system, factor VII

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2
Q

Osler-Weber-Rendu Syndrome

A

Hereditary hemorrhagic telangiectasia. Autosomal dominant. Presence of telangiectasias in the skin as well as mucous membranes of the lips, oropharynx, respiratory tract, GI tract, and urinary tract. Rupture may cause epistaxis, GI bleeding, or hematuria

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3
Q

What drives angiogenesis?

A

Vascular endothelial growth factor (VEGF) and Fibroblast growth factor (FGF)

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4
Q

What is HER2 positivity associated with in breast cancer?

A

Poorly differentiated, rapidly growing tumors

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5
Q

Kawasaki diagnosis criteria

A

Fever for greater than or equal to 5 days plus 4 of the following

  1. Bilateral non-exudative conjunctival injection
  2. Cervical lymphadenopathy
  3. Mucositis: Erythema of the palatine mucosa, fissured erythematous lips, “strawberry tongue”
  4. Extremity changes: Edema of the hands and feet, erythema of palms and soles, desquamation of the fingertips
  5. Rash: polymorphous erythematous rash on the extremities that spreads centripetally to the trunk
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6
Q

What is a serious complication of Kawasaki disease?

A

Coronary artery inflammation leading to the development of coronary artery aneurysms

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7
Q

t(15;17)

A

Acute promyelocytic leukemia (APL)

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8
Q

Warfarin induced skin necrosis can occur in individuals with what deficiency?

A

Underlying protein C or S deficiency

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9
Q

How do renal cortical cells respond to hypoxia?

A

Synthesize and release erythropoietin

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10
Q

When should inherited causes of hypercoagulability be considered?

A

In patients younger than 5 who present with thrombosis and no obvious explanation for an acquired prothrombotic state

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11
Q

Describe mechanism of factor V Leiden mutation

A

Causes factor Va resistance to inactivation by activated protein C

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12
Q

IgE-independent mast cell activation common symptoms

A

Diffuse itching and pain, bronchospasm, and localized swelling (urticaria)

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13
Q

What is the most common trigger of DIC in pregnancy?

A

Release of tissue factor (thromboplastin) from an injured placenta into the maternal circulation

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14
Q

Which arteries are typically spared in polyarteritis nodosa?

A

Pulmonary

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15
Q

What causes pancytopenia seen with lupus erythematosus?

A

Formation of autoantibodies against blood cells (type II hypersensitivity)

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16
Q

Pure red cell aplasia is associated with?

A

Thymoma, lymphocytic leukemias, parvovirus B19

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17
Q

Actions of active/inactive Rb protein

A

Active (hypophosphorylated) RB protein prevents damaged cells from preceding past G1 to S checkpoint, while inactive (hyperphosphorylated) Rb protein allows the damaged cells to enter mitosis.

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18
Q

What does binding of programmed death receptor 1 to its ligand do?

A

Down regulates the immune response against tumor cells by inhibiting cytotoxic T cells. Many types of cancers increase expression of PD-L1

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19
Q

Triad of eczema, thrombocytopenia, and combined B and T lymphocyte deficiency. Repeated infections by encapsulated organisms. Presents at 6-12 months of age.

A

The Wiskott-Aldrich syndrome

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20
Q

Li-Fraumeni Syndrome

A

Aut. dominant mutation in TP53. Leukemia, sarcomas, tumors of the breast, brain, and adrenal cortex are most common

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21
Q

Functions of vWF

A

Promoter of platelet adhesion at sites of vascular injury by binding platelet glycoproteins ti subendothelial collagen on injured blood vessels. Also acts as a protective carrier protein for circulating factor VIII

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22
Q

Anticoagulant reversal of heparin

A

Protamine sulfate - binds to heparin causing chemical inactivation

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23
Q

Treatment for PNH

A

Eculizumab - inhibits terminal complement formation

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24
Q

Mutation in sickle cell anemia

A

Single amino acid replacement in beta chain (substitution of glutamic acid with valine)

25
Q

Mutation in HbC disease

A

Glutamic acid to lysine mutation in beta globin

26
Q

Hodgkin lymphoma is characterized by?

A

Reed-Sternberg cells: distinctive tumor giant cells; binucleate or bilobed with 2 halves as mirror images (“owl eyes”). CD15+ and CD30+ B cells

27
Q

Burkitt lymphoma translocation

A

t(8;14) - translocation of c-myc (8) and heavy chain Ig (14)

28
Q

Starry sky appearance. Associated with EBV

A

Burkitt lymphoma

29
Q

Follicular lymphoma translocation

A

t(14;18) - translocation of heavy chain Ig (14) and BCL-2 (18)

30
Q

Mantle cell lymphoma translocation

A

t(11;14) - translocation of cyclin D1 (11) and heavy chain Ig (14)

31
Q

Marginal zone lymphoma translocation

32
Q

Characterized by atypical CD4+ cells with “cerebriform” nuclei and intraepidermal neoplastic cell aggregates

A

Mycosis fungoides/Sezary syndrome

33
Q

Monoclonal plasma cell (“fried egg appearance) cancer that arises in the marrow and produces large amounts of IgG or IgA. Ig light chains in urine, rouleaux formation, plasma cells with “clock face” chromatin.

A

Multiple myeloma

34
Q

TdT+, CD10+

A

Acute lymphoblastic leukemia/lymphoma

35
Q

Which cancers associated with Down Syndrome

36
Q

CD20+, CD23+, CD5+, B-cell neoplasm. Smudge cells

37
Q

TRAP +

A

Hairy cell leukemia

38
Q

Treatment for t(15;17) AML

A

all-trans retinoic acid (Vit A)

39
Q

t(9;22)

A

Philadelphia chromosome, BCR-ABL. Defines CML

40
Q

What mutation are chronic myeloproliferative disorders associated with?

A

JAK2 mutation

41
Q

Cells express S-100 and CD1a, Birbeck granules (tennis racket or rod shaped)

A

Langerhans cell histiocytosis

42
Q

Heparin mechanism

A

Activates antithrombin, which decreases action of IIa (thrombin) and factor Xa. Follow PTT

43
Q

What causes HIT

A

Development of IgG antibodies against heparin-bound platelet factor 4. Leads to thrombosis and thrombocytopenia

44
Q

Direct thrombin inhibitors

A

Bivalirudin, Argatroban, Dabigatran

45
Q

Direct thrombin inhibitors mechanism

A

Directly inhibits activity of free and clot-associated thrombin

46
Q

Warfarin mechanism

A

Interferes with gamma-carboxylation of vit-K dependent clotting factors II, VII, IX, X, and proteins C and S. Increase PT.

47
Q

Reversal of heparin

A

Protamine sulfate

48
Q

Direct factor Xa inhibitors

A

Apixaban, rivaroxaban

49
Q

Mechanism of thrombolytics

A

Directly or indirectly aid conversion of plasminogn to plasmin, which cleaves thombin and fibrin clots

50
Q

ADP receptor inhibitors

A

Clopidogrel, prasugrel, ticagrelor, ticlopidine

51
Q

Mechanism of ADP receptor inhibitors

A

Inhibit platelet aggregation by ireversibly blocking ADP (P2Y12) receptor. Prevent expression of IIb/IIIa on platelet surface

52
Q

Persistent infection and coagulopathy causing hemorrhagic signs and symptoms. BM biopsy shows promyelcoytes with intracytoplasmic Auer rods

A

Acute promyleocytic leukemia

53
Q

Translocation for APL

A

t(15;17) - causes a fusion of the retinoic acid receptor alpha gene and promyelocytic leukemia gene

54
Q

What amino acid substitution leads to sickle cell anemia?

A

Valine substitutes for glutamic acid

55
Q

What are the atypical cells seen in peripheral smear of pts with mono?

A

Activated CD8+ T-lymphocytes

56
Q

Henoch-Sconlein purpura

A

IgA mediated hypersensitivity vasculitis that generally affects young children and is preceded by upper respiratory infection. Causes abdominal pain, joint pain, lower extremity palpable purpura and hematuria

57
Q

Xeroderma pigmentosum

A

Defect in nucleotide excision repair. Increased sensitivity to UV radiation and high incidence of cutaneous malignancy

58
Q

bcl-2 overexpression

A

Inhibits apoptosis and promotes survival of tumor cells. Occurs in follicular lymphoma with t(14;18)

Step 1 (Steffanie) (21 decks)

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