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STEP2 > Heme-Onc > Flashcards

Heme-Onc Flashcards

1
Q

Multiple Myeloma presentation, dx

A

~60
Bone pain, usually back
Renal failure
Constipation & fatigue 2/2 hypercalcemia

Normocytic anemia
Renal dysfunction
Hypercalcemia
Urine: bence jones proteins
XR:  punched out, osteoporotic lesions
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2
Q

Soft tissue sarcoma locations w/ mets, presentation, dx, staging, tx

A

Extremities, trunk, retroperitoneal
Pulmonary mets

Younger, healthy patients
Unexplained mass…literally just a big lump
Non-tender
No surrounding skin changes (hematoma, erythema)
No systemic symptoms

Core biopsy…not excisional!!
CT for lung mets if higher stage

Stage 1-3: based on size, depth, grade…no mets
Stage 4: mets

Excision with 2cm margin…not amputation
+/- chemo depending on stage

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3
Q

GI stromal tumor (GIST) path, presentation, tx

A

KIT oncogene CD117 overexpression ==> proliferation of interstitial cells of cajal ==> stomach/small bowel tumor

Often asymptomatic or bleeding

Resection + imitinab

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4
Q

Breast cancer staging, dx workup, tx by stage

A

Stage I 3cm –node
Stage III 5 + 1 node
Stage IV: any metastasis

FNA ⇒ core biopsy for staging ⇒ bilateral mammography
Staging: CBC, LFT, CXR always ⇒ CT, bone scan, brain MRI if suspicious for metastases
Sentinel LN biopsy for staging

Stage I: lumpectomy + radiation
Stage II+: surgery + chemo
ER/PR positive: estrogen (aromatase) inhibitors
Her-2 positive: trastuzamab

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5
Q

Inherited hypercoagulable disorders ddx, path w/ commonality*, presentation, dx, tx

A
  1. Factor V leiden (most common) ==> Factor V becomes resistant to inactivation by protein C
  2. Protein C / S deficiency
  3. Antithrombin 3 deficiency

PE, MI, stroke, miscarriage

Heparin ==> warfarin (6 months for first event, 12 months for second, lifelong for 3+)

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6
Q

Retinoblastoma path, presentation*, dx, tx

A

Failed Rb suppressor inactivation ==> rapid metastasis to liver and brain

LEUKOCORIA ==> immediate ophthalmology referral*
Eye pain

CT or US

Surgical removal

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7
Q

Clotting cascade described

A
  1. Fibrin clot formation
    INTRINSIC: 12 ==> 11 ==> 9 ==> 8 ==> 10
    EXTRINSIC: Tissue Factor (3, produced by vessels) ==> 7 ==> 10
    BOTH: 10 ==> 5 ==> 10-5 complex ==> prothrombin (f2) ==> thrombin (f2a) ==> fibrinogen to fibrin ==> fibrin polymer forms clot
  2. Platelet plug formation:
    vWF carries factor VIII and forms “glue” between collagen (exposed @ damaged vessel) & platelet

Thrombin activates platelets ==> activated platelets bind fibrinogen ==> fibrinogen links activated platelets

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8
Q

In-vivo clot inhibitors

A

Antithrombin: inactivates thrombin & f7, 9, 10, 11

Protein C & S: thrombin activated ==> inactivates 5 and 8

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9
Q

Heparin mechanism, lab effects, s/e, reversal, alternative drugs

A

Activates antithrombin 3 ==> inactivates thrombin & f7, 9, 10, 11

Increases PTT (intrinsic pathway)

Heparin-induced thrombocytopenia (HIT) 2/2 autoantibodies against platelets ==> bleeding & thrombosis

Protamine sulfate

Argatroban (thrombin inhibitor)
Enoxaparin (f10 inhibitor)

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10
Q

Warfarin mechanism, lab effects, s/e, reversal

A

Vitamin K antagonist: inhibits vitamin-K dependent factors (7, 9, 10, prothrombin)

Affects PT (extrinsic pathway)

Initially depletes protein C & S ==> causing hypercoagulability…thus heparin bridge required

Vitamin K reversal

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11
Q

PTT measures

A

Intrinsic pathway: 12, 11, 9, 8, 10, 5, prothrombin, thrombin

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12
Q

PT measures

A

Extrinsic pathway: 7, 10, 5, prothrombin, thrombin

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13
Q

Hemophilia path, types, dx, severities, tx

A

X-linked clotting factor deficiency

A: factor 8 deficiency (90% of cases)
B: factor 9 deficiency (9% of cases)

Prolonged PTT only! (normal bleeding time?)
Obtain specific factor assay to determine type

Mild > 5% normal factor levels
Severe <1% normal factor levels

Clotting factor transfusion or cryoprecipitate

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14
Q

Von Willebrand’s disease path, presentation, dx, tx

A

Autosomal dominant deficient/dysfunctional vWF ==> dysfunctional factor 8 (carried by vWF)

Less severe bleeding than hemophilia

Prolonged PTT & bleeding time
Ristocetin assay

DDAVP (releases more factor 8)

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15
Q

Disseminated intravascular coagulation (DIC) path, presentation, dx, tx

A

Underlying disorder (obstetric, infection, neoplasm, shock etc.) causing fibrin deposition in small vessels ==> end organ damage & bleeding 2/2 thrombocytopenia and clotting factor deficiency

Bleeding & organ damage

Increased PT & PTT, D-dimer, fibrin split products
Decreased clotting factors, platelets, fibrinogen
+Microangiopathic hemolytic anemia
Basically, nothing normal

Reverse underlying cause

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16
Q

Thrombotic thrombocytopenic purpura (TTP) path, dx, tx

A

Underlying disorder causing platelet microthrombi occlusion of small vessels ==> end organ damage

Often kids... 
5+
Low platelets
Microangiopathic hemolytic anemia
Neurologic sx (seizure, delirium) 
Fever
Renal failure (Cr elevation) 
Smear:  schistocytes 2/2 hemolytic anemia
NORMAL:  PT, PTT, fibrinogen

Steroids
Plasma replacement
NOT: platelet replacement (worsens)

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17
Q

Hemolytic uremic syndrome (HUS) dx, tx

A

Renal failure (more severe than TTP)
Microangiopathic hemolytic anemia
Thrombocytopenia

Steroids
Plasma replacement
NOT: platelet replacement (worsens)

18
Q

Idiopathic thrombocytopenic purpura (ITP) path, presentation, dx, tx

A

IgG autoantibodies to platelets

Less severe bleeding than HUS, TTP, DIC

Exclusion

Steroids

19
Q

Anemia general categories, ddx***

A
  1. Decreased production: low reticulocyte count (100)
    2/2 DNA synthesis problem: B12 (cobalamin) deficiency, folate deficiency, chemotherapy
  2. Increased destruction: elevated reticulocyte count (>2.5), EPO, and indirect bilirubin
  3. Blood loss
20
Q

Iron deficiency anemia vs anemia of chronic disease dx

A 
Fe deficiency:  
Low serum Fe
High TIBC & transferrin (Fe transporter)
Low Fe/TIBC 
Low ferritin (Fe storage) 
High RDW

Chronic disease:
Low serum Fe
Low TIBC & transferrin
High ferritin

21
Q

Megaloblastic anemia path, presentation, dx, tx

A

B12/cobalamin deficiency 2/2 malabsorption (pernicious anemia, tapeworm)
Folate deficiency 2/2 malabsorption, alcoholism, drugs

Fatigue, pallor, diarrhea etc.
B12: demyelination ==> neuropsychiatric and motor/sensory dysfunction

MCV > 100
Hypersegmented neutrophils
Schilling test: saturate liver stores with IM unlabeled B12 ==> ingest radiolabeled B12 ==> if absorbed, radiolabeled will be in urine

22
Q

Hereditary spherocytosis path, presentation, dx, tx

A

RBC membrane fragility ==> hemolytic anemia

Pallor, fatigue, jaundice

Hemoglobinuria (dark urine)
LDH elevation
Elevated indirect bilirubin
Smear:  spherocytes lacking central pallor
\+ osmotic fragility test

Steroids

23
Q

G6PD deficiency path, presentation, dx, tx

A

X-linked recessive RBC sensitivity to oxidative stressors (fava beans, sulfonamides, antimalarials)

Pallor, fatigue, jaundice

Hemoglobinuria (dark urine)
LDH elevation
Elevated indirect bilirubin

Steroids

24
Q

Fanconi’s anemia presentation

A

Short stature
Thumb aplasia
Cafe-au-lait spots

25
Q

Sickle cell anemia path, complications, dx, tx*

A

Autosomal recessive glu==>val replacement on beta-subunit of Hgb ==> anemia and vasoocclusion

Splenic infarction (pneumococcal infection)
Acute chest
Cholecystitis
Aplastic crisis 2/2 parvo
Osteomyelitis (s. aureus, salmonella)
Stroke

Smear: sickle and target cells
Dx: quantitative hemoglobin electrophoresis

Hydroxyurea (stimulates fetal hemoglobin)
Exchange transfusion*
Acute chest: incentive spirometry

26
Q

Alpha thalassemia types w/ presentations, dx, tx

A

Hydrops: 0/4 alpha genes present ==> death in utero

H-disease: 1/4 alpha genes present
Hypochromic microcytic anemia
Chronic hemolysis ==> splenomegaly, jaundice, cholelithiasis, extramedullary hematopoiesis bone changes
Transfusions with deferoxamine iron chelation

Trait: 2/4 genes present
Hypochromic microcytic anemia
Asymptomatic

Carrier: 3/4 genes present

27
Q

Beta thalassemia types w/ presentations, dx, tx

A

Major: 0/2 beta genes present
Hypochromic microcytic anemia
Requires transfusions + deferoxamine

Minor: 1/2 beta genes present
Hypochromic microcytic anemia
Asymptomatic

28
Q

Polycythemia vera path, presentation, dx, tx

A

JAK-2 mutation ==> universal marrow proliferation, but predominantly erythrocytosis

Elderly
CHF 2/2 hyperviscosity
Hepatosplenomegaly

Low EPO (unlike most other polycythemias)

Hydroxyurea

29
Q

Transfusion reactions w/ path, presentation, tx

A

Non-hemolytic febrile:
IL-6 elevation
Fever, chills, rigor hours-post transfusion
Stop transfusion + tylenol

Minor allergic:
Antibody formation against donor RBC
Hives
Stop transfusion + benadryl / epinephrine

Hemolytic transfusion reaction:
Hypotension 2/2 anaphylaxis

30
Q

Acute lymphoblastic leukemia path (ALL), presentation, dx*, tx, prognosis

A

B or T cell proliferation ==> pancytopenia

Most common pediatric cancer
Occurs in adults too
Hepatosplenomegaly
B-ALL:  fatigue, anemia, bone pain
T-ALL:  mediastinal thymus lymphoma causing local sx

Pancytopenia
Smear: lymphoblasts (minimal cytoplasm, granules or nucleoli. negative myeloperoxidase)
Dx: bone marrow biopsy >25% lymphoblasts

Chemo + allopurinol (prevents tumor lysis syndrome)

Children: excellent

31
Q

Acute myelogenous leukemia (AML) path, presentation, dx, tx, prognosis

A

Myeloid lineage proliferation in bone marrow==> pancytopenia

Adults & kids
Benzene exposure
Sludging: pulmonary, CNS symptoms
Rarely hepatosplenomegaly

Smear: myeloblasts (more cytoplasm, granular, prominent nucleoli, + myeloperoxidase, + AUER RODS)

Chemo + allopurinol (prevents tumor lysis syndrome)

Poor prognosis

32
Q

Chronic lymphocytic leukemia path, presentation, dx*, tx

A

Mature lymphocyte proliferation

Elderly, often asymptomatic
Fatigue, malaise
Infection
Hepatosplenomegaly & lymphadenopathy

Lymphocyte > 5000
Granulocytosis, anemia, thrombocytopenia
Smear: lymphocytosis with smudge cells**

Treat only once symptomatic ==> palliative but slowly progressing

33
Q

Chronic myelogenous leukemia (CML) path, presentation, dx, tx

A

BCR-ABL (9,22) translocation ==> granulocytosis
Spectrum of types: chronic to acute blast crisis

Middle-aged adult
Usually asymptomatic
Hepatosplenomegaly
Constitutional

PCR: BCR-ABL translocation
Massive neutrophilia
Low leukocyte alkaline phosphatase
High LDH, uric acid

Imatinib (gleevec) inhibits BCR-ABL tyrosine kinase
Bone marrow transplant

34
Q

Hairy cell leukemia path, presentation, dx, tx

A

Adult B-cells proliferation

Constitutional
Infection and bleeding 2/2 marrow infiltration

Rarely leukocytosis
Tartrate-resistant acid phosphatase (TRAP) stain showing hairy cells

35
Q

Non-hodgkin’s lymphoma path, presentation, dx, tx

A

t14,18 translocation, EBV, H. pylori ==> B & T cell proliferation in lymphoid tissue

Usually adults, sometimes children
Lymphadenopathy and hepatosplenomegaly
Constitutional symptoms
Mass formation & extranodal manifestations when severe

Excisional lymph node biopsy

36
Q

Hodgkin’s lymphoma path, presentation, dx, tx

A

B-cell proliferation

Bimodal ages: 30’s and 60’s
Above diaphragm adenopathy
Oscillating weeks of fevers
Hepatosplenomegaly

Excisional lymph node biopsy: reed-sternberg binucleated B-cells with “owl-eye” appearance

37
Q

Multiple myeloma path, presentation, dx, tx

A

B-cell proliferation with excessive IgA, IgM, and/or kappa/lambda light chains. MGUS precursor.

Elderly w/
Bone pain
Renal failure
Anemia
Constipation 2/2 hypercalcemia

Triad: M-proteins, B-cell proliferation, lytic bone lesions

Melphalan

38
Q

Waldenstrom’s macroglobulinemia path, presentation, dx, tx

A

IgM hyperproliferation ==> hyperviscosity, cold agglutinin disease, amyloid. MGUS precursor.

Raynaud’s
Neurologic problems

?

?

39
Q

Amyloidosis dx

A

Congo red stain showing apple-green birefringence under polarized light

40
Q

Neutropenia pathogens, tx

A

S. aureus, pseudomonas, E. coli, Proteus, Klebsiella

G-CSF
If febrile: broad spectrum abx

41
Q

Transplant types, rejection types

A

Autologous: to self
Allogenic: another person
Syngeneic: twin-twin

Hyperacute: hours post-transplant 2/2 preformed antibodies ==> vascular thrombi ==> cytotoxic treatment

Acute: days to 3 months 2/2 T-cell mediation ==> steroid treatment

Chronic: months to years ==> no tx

Graft-versus-host: donated T-cells attack host skin, GI

42
Q

Angiofibroma path, presentation*, dx, tx

A

Tumor

Bleeding w/ mass

Clinical

Surgery

STEP2 (16 decks)

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