Usmle Step 1 Flashcards - Hematology

Front 1

Mechanism and AEs of cisplatin and carboplatin?

Back 1

Cross-link DNA

AE: nephrotoxicity, acoustic nerve damage

Front 2

Risk of endometrial carcinoma occurs with which SERMs?

Back 2

Tamoxifen: increases risk (partial agonist effects)

Raloxifene: no increased risk (endometrial antagonist)

Front 3

Paclitaxel prevents progression to which phase of the cell cycle?

Back 3

Inhibits M phase: progression to anaphase
- hyperstabilizes polymerized microtubule so mitotic spindle can't break down

Front 4

Microtubule inhibitors that block polymerization so mitotic spindles can't form?

Back 4

Vincristine and vinblastine

Front 5

Cytarabine mechanism and AEs?

Back 5

Pyrimidine analog --> inhibit DNA polymerase
- used for AML, ALL, non-Hodgkin's lymphoma

AEs: leukopenia, thrombocytopenia, megaloblastic anemia

Front 6

Relationship between 5-FU and methotrexate?

Back 6

Synergistic relationship

Front 7

AE of 5-FU?

Back 7

Myelosuppression (NOT reversible with leucovorin)
- Can "rescue" with thymidine

Photosensitivity

Front 8

Non-neoplastic uses of methotrexate?

Back 8

Abortion
Ectopic pregnancy
Rheumatoid arthritis
Psoriasis

Front 9

Leucovorin is used to rescue what?

Back 9

Folinic acid that rescues myelosuppression as a result of methotrexate

Front 10

Drug similar to 6-MP that can be given with allopurinol?

Back 10

6-thioguanine (6-TG)

Front 11

Direct thrombin inhibitors and use?

Back 11

Lepirudin, Bivalirudin

Used as alternative to anticoagulation in patients with HIT.

Front 12

Enoxaparin vs Heparin?

Back 12

Enoxaparin: low molecular weight heparin, acts more on Xa, better bioavailability, 2-4x longer half life
- administered subQ without lab monitoring

Front 13

Mechanism of heparin induced thrombocytopenia (HIT)?

Back 13

Heparin binds platelet factor IV --> Ab production --> binds to/activates platelets --> clearance of platelets --> thrombocytopenic, hypercoagulable state

Front 14

RBC count vs mass?

Back 14

Count: RBCs/ul blood

Mass: total RBCs in body (ml/kg)

RBC count = RBC mass / plasma volume

Front 15

Key lab values to differentiate polycythemia vera from other forms of polycythemia?

Back 15

Increased plasma volume
Decreased EPO
Significantly increased RBC mass

Front 16

AE of ticlopidine (gpIIb/IIIa inhibitor)?

Back 16

Neutropenia

Front 17

What are auer rods?

Back 17

Peroxidase-positive cytoplasmic inclusions in granulocytes and myeloblasts
- commonly seen in AML M3 (APL)

Front 18

S-100, CD1a, and Birbeck granules are characteristic of what?

Back 18

Langerhans cell histiocytosis (LCH)

Front 19

Mantle cell lymphoma translocation and gene?

Back 19

t(11;14)

Cyclin D activation

Front 20

List 4 thrombolytics.

Back 20

Streptokinase
Urokinase
tPA (alteplase)
APSAC (anistreplase)

Front 21

Toxicity of Trastuzumab? Imatinib?

Back 21

Trastuzumab (anti HER-2/erb-B2): cardiotoxicity

Imatinib (bcr-abl TKI): fluid retention

Front 22

Alkylating agents?

Back 22

Cyclophosphamide, ifosfamide
Nitrosoureas (carmustine, lomustine, semustine, streptozocin)
Busulfan

Front 23

Toxicity of nitrosoureas?

Back 23

CNS toxicity (dizziness, ataxia)
- crosses BBB --> used for brain tumors (GBM, etc)

Front 24

Mechanism of cyclophosphamide?

Back 24

Alkylating agent
- covalently cross link DNA at guanine N-7
- requires bioactivation by the liver

Front 25

What mechanism do doxorubicin/daunorubicin and bleomycin have in common?

Back 25

Generate free radicals --> cause breaks in DNA strands

- Doxo/daunorubicin also noncovalently intercalate in DNA

Front 26

Which chronic myeloproliferative disorders are JAK2 positive?

Back 26

Polycythemia vera
Essential thrombocytosis
Myelofibrosis

(all except CML)

Front 27

Mechanism of bleomycin vs busulfan?

Back 27

Bleomycin: induces free radical formation --> breaks in DNA strands

Busulfan: alkylates DNA

Front 28

What can happen with treatment of AML?

Back 28

Auer rods (peroxidase positive cytoplasmic inclusions) can be released and cause DIC

Front 29

What do defective cells in Langerhans cell histiocytosis (LCH) express?

Back 29

S-100 and CD1a

Front 30

Where are leukemic infiltrates often seen?

Back 30

Liver, spleen, and lymph nodes

Front 31

What are 3 common features of any leukemia?

Back 31

Anemia (decreased RBCs)
Infections (decreased mature WBCs)
Hemorrhage (decreased platelets)

Front 32

Which translocation confers a better prognosis in ALL?

Back 32

t(12;21)

Front 33

Most common cause of generalized lymphadenopathy > 60 yo?

Back 33

SLL/CLL

Front 34

TRAP positive leukemia?

Back 34

Hairy cell leukemia

Cells have filamentous, hairlike projections.

Front 35

Most common adult non-Hodgkin's lymphoma?

Back 35

Diffuse large B cell

Front 36

CD5+ tumor?

Back 36

Mantle cell lymphoma

Front 37

Which type of lymphoma has more constitutional/B symptoms?

Back 37

Hodgkin's

- low grade fever, night sweats, weight loss

Front 38

Spread of Hodgkin's vs non-Hodgkin's lymphoma?

Back 38

Hodgkin's: more localized, contiguous spread, extranodal is rare

Non-Hodgkin's: peripheral nodes, extranodal involvement, non-contiguous spread

Front 39

Age distribution of Hodgkin's vs non-Hodgkin's lymphoma?

Back 39

Hodgkin's: bimodal distribution (and more common in men, except nodular sclerosing)

Non-Hodgkin's: ~20-40 yo

Front 40

Lacunar cells are seen in which variant of Hodgkin's lymphoma?

Back 40

Nodular sclerosing

Front 41

Best to worse prognosis of Hodgkin's lymphoma?

Back 41

Excellent: nodular sclerosing & lymphocyte predominant

Intermediate: mixed cellularity

Poor: lymphocyte depleted

Front 42

Causes of DIC?

Back 42

Sepsis
Trauma
Obstetric
Pancreatitis (acute)
Malignancy
Nephrotic syndrome
Transfusion

Front 43

Receptor deficiency in Bernard-Soulier disease vs Glanzmann's thrombasthenia?

Back 43

Bernard-Soulier: Gp1b

Glanzmann's: Gp IIb/IIIa

Front 44

Pentad of symptoms seen in TTP?

Back 44

Neurologic sx
Renal sx
Fever
Thrombocytopenia
Microangiopathic hemolytic anemia

Front 45

Enzyme deficient in acute intermittent porphyria?

Back 45

Porphobilinogen deaminase

Get buildup of porphobilinogen, d-ALA, and uroporphyrin

Front 46

Enzyme deficient in porphyria cutanea tarda?

Back 46

Uroporphyrinogen decarboxylase.

Get buildup of uroporphyrin

Front 47

1st reaction of heme synthesis?

Back 47

Glycine + succinyl CoA --> B6 + d-ALA synthase --> d-aminolevulinic acid (d-ALA)

Front 48

Warm agglutinins causing AIHA result from what?

Back 48

Chronic anemia in SLE
CLL
Alpha-methyldopa, penicillin, Quinidine

Front 49

Complications in sickle cell homozygotes?

Back 49

1. Aplastic crisis
2. Autosplenectomy
3. Salmonella osteomyelitis
4. Painful vaso-occlusive crisis
5. Renal papillary necrosis and microhematuria
6. Splenic sequestration crisis

Front 50

What happens to glucose in RBCs?

Back 50

90% is anaerobically degraded to lactate
10% goes though HMP shunt

Front 51

Different granules that platelets contain?

Back 51

Dense granules (ADP, Ca)
Alpha-granules (vWF, fibrinogen)

Front 52

What do the large, spherical azurophilic granules in neutrophils contain?

Back 52

Hydrolytic enzymes
Lysozyme
Myeloperoxidase
Lactoferrin

Front 53

Cells with frosted glass cytoplasm and kidney shaped nucleus?

Back 53

Monocytes

Front 54

2 things eosinophils produce and their function?

Back 54

Histaminase and Arylsulfatase

Help limit reaction following mast cell degranulation

Front 55

Causes of eosinophilia?

Back 55

Neoplastic
Asthma
Addison's
Allergic processes
Collagen vascular diseases (PAN)
Parasites (invasive)

Front 56

Degranulation of mast cells releases what?

Back 56

Histamine
Heparin
Eosinophil chemotactic factors

Front 57

Basophilic granules in basophils contain what?

Back 57

Heparin
Histamine
Vasoactive amines
Leukotrienes (LTD-4)

Front 58

What activates factors VIIIa and Va?

Back 58

IIa (thrombin)

Front 59

What is kallikrein activated by and what does it do?

Back 59

Activated by XIIa

Converts HMWK to bradykinin

Front 60

Acanthocytes are seen in what diseases?

Back 60

Liver disease & Abetalipoproteinemia

Echinocytes (more moderate): seen in uremia

Front 61

Basophilic stippling?

Back 61

Thalassemias
Anemic of chronic disease
Iron deficiency
Lead poisoning

Front 62

Target cells?

Back 62

HbC disease
Asplenia
Liver disease
Thalassemia

Front 63

Heinz bodies?

Back 63

Oxidation of iron leads to denatured Hgb precipitation and damage to RBC membrane --> formation of bite cells

Alpha thalassemia, G6PD deficiency

Front 64

Barts hemoglobin results from what?

Back 64

Deletion of 4 alpha genes
Barts: gamma4
- incompatible with life (hydrops fetalis)

Front 65

HbH disease results from what?

Back 65

Deletion of 3 alpha genes
HbH = beta4

Front 66

Racial distribution of alpha vs beta thal?

Back 66

Alpha: Asia/Africa

Beta: Mediterranean

Front 67

"Crew cut" on x-ray could mean what?

Back 67

Sickle cell disease or beta-thalassemia major

Front 68

Causes of aplastic anemia?

Back 68

Radiation/drugs (benzene, chloramphenicol, alkylating agents, antimetabolites)
Viral agents (B19, EBV, HIV, HCV, CMV)
Fanconi's anemia (inherited DNA repair defect)
Idiopathic (follows acute hepatitis)

Front 69

Treatment of aplastic anemia?

Back 69

Immunosuppression (antithymocyte globulin, cyclosporine)
Allogeneic BM transplant
Irradiated RBC, platelet transfusion
G-CSF or GM-CSF

Front 70

Mechanism that Dactinomycin and doxo/daunorubicin share?

Back 70

Both intercalate in DNA