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Heme/Onc > FA Notes > Flashcards

FA Notes Flashcards

0
Q

Hereditary cause of sideroblastic anemia? Treatment?

A

X linked delta-ALA synthase

B6, pyridoxine

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1
Q

How does lead poisoning leads to anemia?

A

Lead inhibits ferrochelatase and ALA dehydratase decreasing heme synthesis and inc RBC protoporphyrin

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2
Q

What are the effects of LEAD poisoning? Treatment?

A

Lead lines on gingivae and long bone
Encephalopathy and Erythrocyte basophilic stippling (blocked rRNA degradation)
Abdominal colic and sideroblastic Anemia
Drop of wrist or foot, Dimercaprol and EDTA

Kids: Succimer

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4
Q

What causes orotic aciduria? What’s the clinical manifestation? How is it differentiated from ornithine transcarbamylase def?
Treatment?

A

Inability to convert orotic acid to UMP from UMP synthase, causes megaloblastic anemia, no hyperammonemia
Treatment: uridine monophosphate

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5
Q

What does heparin do? How is it reversed?

A

It activates antithrombin, decreasing both thrombin and factor Xa. Reversal by protamine sulfate

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6
Q

What is HIT?

A

Heparin-induced thrombocytopenia, IgG antibodies against heparin bound PF4. The complex activates platelets and causes thrombosis and thrombocytopenia

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7
Q

What do Argatroban, bivalrudin, and dabigatran do? When are they used?

A

Inhibit thrombin directly, used for patients with HIT

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8
Q

What apixaban, rivaroxaban do?

A

Bind directly to Xa

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9
Q

Which anticoagulant is safe in pregnancy?

A

heparin (not warfarin). It does not cross the placenta

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10
Q

What is warfarin’s toxicity? How is it reversed?

A

Skin/tissue necrosis. Reversed by vit K. Rapidly by FFP

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11
Q

What do alteplase, streptokinase do? How is toxicity treated?

A

They are thrombolytics. They aid activation of plasminogen to plasmin causing cleavage of thrombin and fibrin clots. . Treat toxicity with aminocaproic acid (inhibits fibrinolysis)

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12
Q

What does aspirin do? Toxicity?

A

Irreversible inhibition of COX1 and 2, decreases TXA2 and prostaglandins.
-Causes ulcers, tinnitus, acute renal failure, interstitial nephritis, and Reye syndrome in kids with viral illness

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13
Q

What does overdose look like (acid-base)?

A

Hyperventilation and respiratory alkalosis -> mixed metabolic acidosis and respiratory alkalosis

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14
Q

What does clopidogrel do?

A

It inhibits platelet aggregation by blocking ADP receptors, prevents IIb/IIIa expression on platelet

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15
Q

What cilostazol and dipyridamole do?

A

They are phosphodiesterase III inhibitors. By dec cAMP in platelets, they inhibit aggregation. Vasodilators.

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16
Q

What do abciximab, eptifibatide, and tirofiban do?

A

Bind to IIb/IIIa receptor on activated platelets to prevent aggregation

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17
Q

What does vemurafenib do?

A

It inhibits BRAF positive melanoma

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18
Q

What is trastuzumab? Toxicity?

A

It is a monoclonal antibody against HER-2, tyrosine kinase receptor. Cardiotoxicity

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19
Q

What is tamoxifen, raloxifine? Tamoxifen inc risk of which cancer?

A

Selective estrogen receptor modulator (antagonist in breast, agonist in bone). Tamoxifen inc risk of endometrial cancer

20
Q

What does rituximab do? Toxicity?

A

monoclonal antibody against CD20, inc risk of progressive multifocal leukoencephalopathy

21
Q

What is imatinib?

A

Tyrosine kinase inhibitor of BCR-ABL (Philadelphia in CML)

22
Q

What is erlotinib?

A

EGFR tyrosine kinas inhibitor

23
Q

Bevacizumab

A

Monoclonal antibody against VEGF, inhibits angiogenesis

24
Q

What does Cisplastin/carbolatin do?

A

cross-links DNA, can cause nephrotoxicity (prevent with amifostine and chloride diuresis) or ototoxicity

25
Q

What do etoposide and teniposide do?

A

It inhibits topoisomerase II, causing DNA degredation

26
Q

What does irrnotecan/toptecan do?

A

inhibits topoisomerase I, preventing DNA winding and replication

27
Q

What does hydroxyurea do?

A

Inhibits ribonucleotide reductase, decreases DNA synthesis, increases HbF

28
Q

What cancer drugs are microtubule inhibitors?

A

Paclitaxel, vincristine, vinblastine

29
Q

What does paclitaxel do?

A

It hyperstabilizes polymerized microtubules in M phase, prevents breakdown of mitotic spindle

30
Q

What does vincristine/vinblastine do? Toxicity?

A

Binds to Beta-tubulin and inhibits microtuble polymerization (M phase arrest). Neurotoxic (vincristine) and marrow suppression (vinblastine)

31
Q

What drugs are alkylating agents?

A

Busulfan, cyclophosphamide, nitrosoureas

32
Q

What does busulfan do?

A

Cross-links DNA

33
Q

What does cyclophosphamide/ifosfamide do? Important toxicity?

A

Cross-links DNA at guanine N-7, hemorrhagic cystitis

34
Q

What do nitrosoureas do? Toxicity?

A

Cross blood brain barrier to cross link DNA. Causes CNS toxicity (convulsions, dizziness, ataxia)

35
Q

What does bleomycin do?

A

Induces free radical formation

36
Q

What does dactinomycin do?

A

Intercalates in DNA, used for childhood tumors

37
Q

What does Doxorubicin do? Important toxicity?

A

Generates free radicals, dilated cardiomyopathy

38
Q

What is methotrexate? Important toxicity?

A

Folic acid analog, competitive inhibition of dihydrofolate reductase, dec DNA synthesis. Myelosuppresion, reversible with leucovorin

39
Q

What is 5-fluorouracil? Toxicity?

A

Pyrimidine analog activated to dUMP, inhibits thymidylate synthase, myelosuppression, not revesible

40
Q

What is cytarabine? Toxicity?

A

Pyrimidine analog, inhibits DNA polymerase, causes pancytopenia

41
Q

What is cladribine?

A

Purine analog, used for hairy cell leukemia

42
Q

What is azathioprine/6-MP? Toxicity?

A

Purine analog, activated by HGPRT, myelosuppression. Metabolized by xanthine oxidase

43
Q

First step of Heme synthesis? Co-factor?

A

Glycine + succinyl CoA -> aminovulinic acid. Pyroxidine

44
Q

Defective enzyme in acute intermittent porphyria?

A

Prophobilinogen deaminase

45
Q

Defective enzyme in porphyria cutanea tarda?

A

Uroporphyrinogen decarboxylase

Heme/Onc (3 decks)

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