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Step1 > Heme Onc > Flashcards

Heme Onc Flashcards

0
Q

Lead poisoning

A

Affecting ferrochelatase and ALA dehydrase

(Heme synthesis starts w glycine and succinylCoA w cofactors B6 and delta ALA synthase)

Leads to accumulation of protoporphyrin and delta ALA

Cause microcytic anemia due to lack of heme
Also

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1
Q

Paroxysmal nocturnal hemoglobinuriA

A

Increased complement mediated RBC lysis due to impaired GPI anchor that protects RBC from complement

Located on X chromosome so only one copy of gene is active at a time

acquired mutation in hematopoietic stem cell leads to hemolysis

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2
Q

Hemophilia

A

Deficiency in intrinsic pathway
A: VIII, B: IX
Increases PTT

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3
Q

Vitamin K deficiency (warfin)

A

General coagulation defect

Decreases synthesis of 1972 C and S

Increased PTT and PT

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4
Q

Bernard Soulier syndrome

A

Defect in platelet plug formation

Decreased GpIb leading to defect in platelet vWF adhesion

Increased bleeding time, decreased platelet count

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5
Q

Glanzamann thrombasthenia

A

Defect in platelet plug formation

Decreased GpIIb/IIIa leading to decreased platelet to platelet aggregation

Increased bleeding time

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6
Q

Immune thrombocytopenia

A

Anti GpIIb/IIIa antibodies leading to macrophage destruction of platelet antibody complex

Decreased platelet survival

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7
Q

Thrombotic thrombocytopenia

A

Inhibition or deficiency of ADAMTS 13 leading to decreased degradation of vWF and unnecessary platelet aggregation and thrombosis

Decreased platelet survival time

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8
Q

Von Willebrand disease

A

Intrinsic pathway coagulation defect

Decreased vWF leading to decreased factor VIII and decreased platelet plug formation

Normal PT and increased PTT

Treat w DDAVP (desmopressin, releasing vWF from endothelium)

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9
Q

DIC

A

Widespread activation of clotting leading to deficiency in clotting factors

Caused by sepsis, trauma, malignancy, transfusion

Increased bleeding time, PT, PTT, decreased platelet count

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10
Q

Factor V Leiden

A

Production of mutant factor V that is resistant to degradation by activation of protein C

Most common cause of hypercoaguability

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11
Q

Hodgkin lymphoma

A

Localized single group of nodes, extra nodal rare

Contiguous spread

Constitutional B symptoms: low grade fever, night sweats, weight loss

Good prognosis

50 percent cases associated w EBV

Young adults and greater than 55

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12
Q

Non-Hodgkin lymphoma

A

Multiple peripheral nodes w extra nodal involvement common

Noncontiguous spread

Fewer constitutional symptoms

Peak incidence 20-40

May be associated w HIV and immunosuppression

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13
Q

Reed Steenberg cell

A

Tumor giant cell seen in Hodgkin lymphoma

Binucleated or bilobed w halves as mirror images (owl eye)

Better prognosis w strong stromal or lymphocytic reaction against RS cells

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14
Q

Burkett lymphoma

A

T(8;14) translocation of c-myc

Starry sky appearance

Associated with EBV

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15
Q

Diffuse large B cell lymphoma

A

T (14;18)

Most common type of non Hodgkin

16
Q

Mantle cell lymphoma

A

T(11;14), translocation of cyclin D

Older males

17
Q

Follicular lymphoma

A

T(14;18) w translocation of bcl-2 (inhibits apoptosis)

18
Q

Acute lymphoblastic leukemia

A

Peripheral blood and bone marrow smear have greatly increased lymphoblasts (often around 20%)

T(12;21) better prognosis

19
Q

Chronic lymphocytic leukemia

A

Progress slowly

Smudge cells

Autoimmune hemolytic anemia

20
Q

Hairy cell leukemia

A

Stains w TRAP

Mature B cell tumor, cells have hair like projections

21
Q

Acute myelogenous leukemia

A

Auer rods

T(15;17) M3 subtype responds to retinoids acid

DIC common presentation

22
Q

Chronic myelogenous leukemia

A

T(9;22) Philadelphia chromosome bcr-abl

Increased neutrophils, metamylocytes, basophils

Can accelerate and transform to AML or ALL

Very low leukocyte alkaline phosphatase as opposed to leukemoid reaction

Responds to imatinib(inhibitor of bcr-abl kinase)

23
Q

Langerhan cell histiocytosis

A

Proliferation disorder of dendritic cells in skin

Cells are immature and do not stimulate T cells

Cells express S-100

Present in children w bone lesions and recurrent otitis media

24
Q

Argatroban

A

Anticoagulant used by leeches

Inhibit thrombin directly, use in patients for anticoagulantion after heparin induced thrombocytopenia

25
Q

Rivaroxaban

A

Bind directly and inhibit activity of factor Xa

Treatment and prophylaxis of DVT and PE

Stroke prophylaxis for pts w afib

26
Q

Clopidogrel

A

Inhibit platelet aggregation by irreversibly blocking ADP receptors and further activation of platelets by platelets (ADP binding promotes integration of GIIb/IIIa)

27
Q

Cliostazol

A

Phosphodieserase III inhibitor

Increase cAMP in platelets thus inhibiting platelet aggregation

28
Q

Methotrexate

A

Frolic acid analog

Decreased DNA synthesis due to decreased dTMP

29
Q

5 fluorouracil

A

Pyrimidine analog covalently complexes folic acid

Decreases dTMP and decrease DNA synthesis

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