Sileramram Usmle Heme/onc

Front 1

contents of platelet dense granules?

Back 1

ADP, calcium

Front 2

contents of platelet alpha granules?

Back 2

vWF, fibrinogen

Front 3

Contents of basophils?

Back 3

Dense granules
1. heparin
2. histamine
3. vasoactive amines
4. leukotrienes

Unlike mast cells, basophils are found in the blood.

Front 4

Ways that eosinophils decrease mast cell effects?

Back 4

histaminase
arylsulfatase

Front 5

Causes of eosinophilia

Back 5

NAACP
Neoplastic
Asthma
Allergic processes
Collagen vascular diseases
Parasites (invasive)

Front 6

hypersegmented polys

Back 6

B12 and folate deficiency

Front 7

anti-AB antibodies are of what isotype?

Back 7

IgM- don't cross the placenta

anti Rh are IgG- can cross the placenta

Front 8

Activators and inactivators of bradykinin?

Back 8

Kallikrein*- activator
ACE- inactivator

Kallikrein- also activates plasminogen --> plasmin

Front 9

Activity of plasmin in the complement pathway?

Back 9

C3 --> C3a

Front 10

Hemophilia B

Back 10

deficiency of factor IX

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Aggregates platelets

Back 11

GpIIb/IIIa + fibrinogen

Glanzmann's thrombastheneia- deficiency in GpIIb/IIIa

Front 12

Adheres platelets to subendothelial collagen

Back 12

GpIb (platelet) and vWF

Bernard-Soulier- deficient in GpI
Von Willebrand disease- deficient in vWF

Front 13

Irreversibly blocked by clopidogrel and ticlopidine

Back 13

ADP receptor
- blocks expression of GpIIb/IIIa

Front 14

Signals for platelet aggregation

Back 14

1. TxA2 released by platelets
2. PGI2 and NO release by endothelial cells

Front 15

Abciximab

Back 15

inhibits GpIIb/IIIa

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warfarin inhibits...

Back 16

epoxide reductase - no activated vit K

Front 17

Action of protein C (APC)

Back 17

cleaves and inactivates Va, VIIIa

*Factor V Leiden mutation - V resistant to APC --> procoag

Front 18

tpa action

Back 18

Activates plasminogen to plasmin

Front 19

Acanthocyte (spur cell)

Back 19

Liver disease
abetalipoproteinemia

- Looks like a star

Front 20

Basophilic stippling

Back 20

Thalassemias
Anemia of chronic disease
Iron deficiency
Lead poisoning

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Bite cell

Back 21

G6PD deficiency

Front 22

Teardrop cell

Back 22

Bone marrow infiltration (myelofibrosis)

Front 23

Target cell

Back 23

HbC disease- mild sickle cell
Asplenia
Liver disease
Thalassemia

Front 24

Heinz body

Back 24

denature hemoglobin- from oxidated iron (ferrous to ferric)

- leads to bite cells

Alpha thalassemia, G6PD deficiency

Front 25

Howell-Jolly bodies

Back 25

basophilic nuclear remnants in rbcs

- hypo or asplenia

Front 26

Hb Barts

Back 26

alpha thal
- deletion of 4 genes- not compatible with life
- tetrameric gamma

Front 27

HbH disease

Back 27

alpha thal
- deletion of 3 genes
- tetrameric beta

Front 28

diagnosis of beta-thal minor

Back 28

Increased HbA2, HbF (alpha2, gamma 2)
- heterozygote

Front 29

beta thal major

Back 29

absent beta chain
- requires blood transfusion
- marrow expansion --> crew cut on skull xray (skeletal deformities)
- target cells

Front 30

Lb poisoning effect on RBCs?

Back 30

Inhibits ferrochelatase and d-ALA dehydratase --> decrease heme synthesis
- protoporphyrin elevated in blood

Inhibits RNA degradation --> basophilic stippling

Front 31

Causes of sideroblastic anemia

Back 31

- defect in heme synthesis

- hereditary (d-ALA synthase)
- EtOH
- Lb
- Increased Fe, normal TIBC, increased ferritin

Front 32

Nonmegaloblastic macrocytic anemias

Back 32

1. liver disease
2. EtOHism w/o B12 or folate def- bone marrow suppression
3. Reticulocytosis- increase MCV
4. Metabolic- purine and pyrimidine synthesis disorders
5. Drugs: 5-Fu, AZT, hydroxyurea

Front 33

Intravascular hemolysis, normocytic, normochromic anemia

Back 33

1. PNH
2. Autoimmune - cold agglutinins (IgM)
3. Mechanical destruction (aortic stenosis, prosthetic valve)

Front 34

Anemia of chronic disease

Back 34

inflammation --> increased hepcidin --> decreased release of iron from macrophages --> decreased iron, TIBC and increased ferritin

* normocytic --> microcytic

Front 35

viral agents that cause aplastic anemia

Back 35

1. Parbovirus B19
2. EBV
3. HIV

Front 36

Drugs that can cause aplastic anemia?

Back 36

1. benzene
2. chloramphenicol
3. alkylating agents
4. antimetabolites

Front 37

hemolytic anemia in a newborn

Back 37

pyruvate kinase deficiency
- decreased ATP --> rigid RBCs
- membrane damage --> extravascular hemolysis

Front 38

pink urine- heme synthesis disease

Back 38

Acute intermittent porphyria
- porphobilinogen deaminase defective --> accumulated porphobilinogen, d-ALA and uroporphyrin

5Ps
Painful abdomen
Pink urine
Polyneuropathy
Pscyhological disturbances
Precipitated by durgs

tx: Glu, heme--> inhibit ALA synthase

Front 39

blistering cutaneous photosensitivity- heme synthesis disease

Back 39

Porphyria Cutanea Tarda
- Urophyrinogen decarboxylase defect
- Uroporphyrin accumulates --> tea colored urine

*most common porphyria

Front 40

Dimercaprol and EDTA

Back 40

1st line for Lb poisoning in adults

- succimer in kids

Front 41

Increased bleeding time but normal platelet count

Back 41

Glanzmann's thrombasthenia
- decreased platelet to platelet aggregation

Front 42

ITP

Back 42

- antibodies against GpIIb/IIIa --> platelet destruction

decrease platelet count, increased bleeding time

Front 43

Neuro, renal, fever, thrombocytopenia, microangiopathic hemolytic anemia

Back 43

TTP
- ADAMTS 13 deficiency--> large vWF multimers --> aggregation and thrombosis

labs: shistocytes and increased LDH

Front 44

hemophilia lab abnormality

Back 44

Increased PTT

Front 45

Haptoglobin in hemolytic anemia?

Back 45

Decreased because binds hemoglobin and carries it out in urine etc

Front 46

CD markers on Reed-Sternberg cells

Back 46

CD30
CD15

Hodgkin's lymphoma

Front 47

Hodgkin's lymphoma more common in women

Back 47

Nodular sclerosing
- young adults
- collagen banding
- lacunar cells
- excellent prognosis

Front 48

Hodgkin's lymphoma- roughly equal lymp/RS ratio

Back 48

Mixed cellularity
- LOTs of RS cells
- Intermediate prognosis

Front 49

"Hodgkin's lymphoma" with different pathophys than others

Back 49

Nodular Lymphoctye predominant Hodgkin's Lymhoma (NLPHL)
- RS cells (popcorn cells) express CD20 (CD15, CD30 negative) --> can progress to diffuse large B cell lymphoma
- high lymph/RS
- Excellent prognosis
- males less than 35

Front 50

Hodgkin's with worst prognosis

Back 50

Lymphocyte depleted
- rare
- RS
- older males with disseminated disease

Front 51

Starry sky appearance

Back 51

Burkitt's
t(8,14)- c-myc next to Ig gene
- sheets of lymphs with macrophages interspersed
- EBV
-

Front 52

t (11,14)

Back 52

Mantle cell lymphoma
- older males
- poor prognosis
- CD5 +

Front 53

t (14, 18)

Back 53

Follicular lymphoma
- bcl-2 expression --> inhibits apoptosis
- Can develop into diffuse B cell lymphoma
- difficult to cure

Front 54

HTLV-1 causes this

Back 54

Adult T cell lymphoma
- cutaneous lesions
- Japan, West Africa, Caribbean
- Aggressive

Front 55

Mycosis fungoides/ Sezary syndrome

Back 55

cutaneous patches and nodules
- INDOLENT

Front 56

What do the two T cell NHLs have in common

Back 56

T-cell lymphoma and Mycosis fungoides
1. Cutaneous lesions
2. Adults

Front 57

t (12, 21)

Back 57

ALL
- better prognosis

Front 58

Presents with bone marrow involvement in childhood

Back 58

ALL

Front 59

Presents as a mediastinal mass in adolescent males

Back 59

ALL

Front 60

Markers for ALL

Back 60

TdT+ (pre T and B cells)
CALLA+

Front 61

t (9,22)

Back 61

CML or poor prognosis in ALL
- bcr-abl

Front 62

Smudge cells in peripheral blood smear with warm antibody autoimmune hemolytic anemia

Back 62

SLL
CLL- more peripheral blood lymphocytosis

- Older adults

Front 63

TRAP stain

Back 63

Hairy Cell leukemia
- elderly
- mature B cells- filamentous hairlike projections

Front 64

t (15,17)

Back 64

APML

Front 65

Auer rods

Back 65

azurophilic granules- APML, AML

Front 66

Responds to all-trans retinoic acid

Back 66

APML (M3)
- induces differentiation of myeloblasts
- add arsenic salts
- tx can induce the release of auer rods --> DIC

Front 67

Can progress to AML or ALL

Back 67

CML

Front 68

imatinib

Back 68

anti-bcr-abl
- tx for CML

Front 69

low leuk alk phos

Back 69

CML

Front 70

leukemoid reaction

Back 70

Increase WBC with left shift, increased leuk alkaline phosphatase

Front 71

t (11,22)

Back 71

Ewing's sarcoma

Front 72

histiocytosis X

Back 72

proliferative disorder of Langerhans cells
- Birbeck granules on EM
- S-100 and CD1a markers

Front 73

S-100 and CD1a markers

Back 73

defective Langerhan's cells in Histiocytosis X

Front 74

JAK3 mutations

Back 74

PCV, Essential thromboyctosis, myelofibrosis

NOT in CML

Front 75

Used to anticoagulate patients with heparin induced thrombocytopenia

Back 75

Lepirudin, bivalirudin
- directly inhibits thrombin

Front 76

Tx for tpa or streptokinase toxicity?

Back 76

aminocaproic acid

Front 77

MTX toxicity?

Back 77

myelosuppression- use leukovorin (folinic acid rescue)

*Leukovorin does not work with 5FU because cannot make dTMP from dUMP
- rescue 5-FU with thymidine

Front 78

6-MP variant that CAN be given with allopurinol?

Back 78

6-thioguanine (6-TG)
- purine analog --> decreases de novo purine synthesis

Front 79

Cytarabine (ara-C)

Back 79

Pyrimidine antagonist --> inhibition of DNA polymerase

Front 80

Antimetabolites that inhibit pyrimidine synthesis

Back 80

5-FU, cytarabine

Front 81

Antimetabolites that inhibit purine synthesis

Back 81

6-MP, 6-TG

Front 82

Antitumor antibiotic used to treat childhood tumors

Back 82

Dactinomycin (Actinomycin D)
- intercalates DNA

"Kids ACT up"

Front 83

"A" in ABVD for Hodgkin's

Back 83

Doxorubicin (Adriamycin), daunorubicin
- Intercalates DNA --> breaks DNA --> generates free radicals

toxicity: cardiotoxicity, myelosuppression, alopecia

Front 84

"B" in ABVD for Hodgkin's

Back 84

Bleomycin
- Induces formation of free radicals (G2 phase specific)

toxicity: pulmonary fibrosis

Front 85

Alkylating agent that can result in hemorrhagic cystitis

Back 85

cyclophosphamide, ifosfamide
- treat with mesna
* requires bioactivation by liver

Front 86

Nitrosureas

Back 86

Alkylating agents
- carmustine, lomustine, semustine, streptozocin
- requires bioactivation
- Crosses BBB
- Use in brain tumors

Front 87

Busulfan

Back 87

Alkylates DNA
- CML
- ablate bone marrow pre transplant

Toxicity: Pulmonary fibrosis, hyperpigmentation

Front 88

"O" in MOPP for Hodgkin's

Back 88

Oncovin (vincristine)
- blocks formation of microtubule spindle

toxicity: paralytic ileus, neuro toxicity (areflexia, peripheral neuritis)

*Vinblastine is similar- bone marrow suppression

Front 89

Carboplatin

Back 89

- like cisplatin
- cross links DNA

Front 90

Hydroxyurea mechanism

Back 90

Inhibits Ribonucleotide Reductase

- Melanoma, CML, sickle cell