Things to remember Flashcards by Lauren Smith

What is the hepatoblastoma mutation?

APC

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What neoplasms associated with t(9;22)?

Philadelphia chromosome, CML, ALL

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What neoplasm associated with t(14;18)?

Follicular lymphoma

BCR; IGH

BCL2/IgH is present in ~80% of follicular lymphoma. Translocation of BCL2 (encoding a protein which opposes apoptosis) to the IgH locus, which results in constitutive transcription of BCL2 and allows the cell to grow despite stresses associated with uncontrolled growth (eg, DNA damage, protein misfolding) which would normally induce apoptosis

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What neoplasm associated with t(11;14)?

Mantle cell lymphoma

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What neoplasm associated with t(8;14,2,22)?

Burkitt lymphoma

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What neoplasm associated with t(2;5)?

ALK gene, anaplastic large cell lymphoma

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What neoplasm associated with t(12;21)?

ALL with good prognosis

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What neoplasm associated with t(15;17)?

APL (AML w/ DIC)

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What is the associated mutation for GIST?

Why is this mutation important?

c-KIT

Allows for prognostic groups based on expected response to imatinib (exon 11 favorable, exon 9 more aggressive)

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Why is PDGFRA wild-type or D842V status important for GIST?

Predicts low response to imatinib

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What is different about lymphatic spread from esophageal cancer compared to other GI cancers?

lymphatics in the esophagus are located in the lamina propria –> LN invasion occurs early and quickly

Compared to rest of the gastrointestinal tract, in which they are located beneath the muscularis mucosa.

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What are the recommendations for lung cancer screening?

Annual screening with low-dose CT in adults ages 55 to 80 years who have a 30 pack-year smoking history and currently smoke or have quit within the past 15 years.

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When will you see target cells?

HALT

HbC disease
Asplenia
Liver disease
Thalassemia

but seems like liver disease and thalassemia are most important

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When will you see acanthocytes (blebs but not around entire cell)?

chronic liver disease

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When will you see burr cells (echinocytes; smaller and more uniform blebs)?

kidney disease

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For a standard dose and average size patient, what change should you see on CBC for that given parameter?

1 dose packed RBC (300 mL per dose) = _____ rise in HgB/dL
300 mL plateletpheresis donor = ____ K/uL rise immediately in platelets
Cryoprecipitate (2 g fibrinogen) = ____ mg/dL rise in fibrinogen

1 dose packed RBC (300 mL per dose) = 1g rise in HgB/dL

300 mL plateletpheresis donor = 25-30 k/uL rise immediately in platelets

Cryoprecipitate (2 g fibrinogen) = 65 mg/dL rise in fibrinogen

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What is the cause of this transfusion rxn: allergic rxn

pt IgE to plasma components

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What is the cause of this transfusion rxn: febrile rxn

pt ab to wbc in blood product

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What is the cause of this transfusion rxn: transfusion associated circulatory overload (TACO)

increased intravascular volume

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What is the cause of this transfusion rxn: acute hemolytic rxn

pt IgG to donor rbcs

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What is the cause of this transfusion rxn: transfusion related acute lung injury (TRALI)

donor abs to pt wbc 2/2 donor multiparity

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What is the cause of this transfusion rxn: sepsis

bacteria in blood bag

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What is the cause of this transfusion rxn: transfusion associated graft vs host disease

donor lymphocytes attack pt’s organs

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What is tx for CML?

Imatinib

Imatinib (and other tyrosine kinase inhibitors) target the constitutive tyrosine kinase signaling through the BCR-ABL fusion protein

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What is tx for chronic myeloproliferative disorders (ET, PV, MF)?

Ruxolitinib Ruxolitinib (and other JAK2 inhibitors) target the constitutive JAK-STAT signaling.

What is mechanism of ATRA for tx of APL?

ATRA (All trans retinoic acid) binds the retinoic acid receptor and causes the blast to mature.

Is this B-ALL or T-ALL? CD19+, t(9;22) or t(12;22), B symptoms

B-ALL 9,22 --> poor prognosis, in adults 12,22 --> good prognosis, in kids

Is this B-ALL or T-ALL? CD3+, mediastinal mass, Cd1a+

T-ALL

How is a soft tissue tumor diagnosed and what is the most important feature?

Core Needle biopsy is needed to diagnose a soft tissue tumor (fine needle aspiration biopsy is insufficient) and the most important prognostic factor is nuclear grade.

What is the impact of prior breast lesions on risk of development of breast cancer? hyperplasia atypical hyperplasia LCIS

Prior lesions (hyperplasia – 2x risk, atypical hyperplasia – 5 x risk, Lobular carcinoma in situ – 8-10x risk).

What phase clinical trial: | Dose escalation studies to define toxicity and determine maximum tolerated dose

Phase I

What phase clinical trial: Drugs given at maximum tolerated dose and response observed as complete (CR), partial (PR), stable disease (SD), or progressive disease (PD)

Phase II

What phase clinical trial: | Studies that test agent in specific line of therapy to assess drug efficacy and benefit of study population

Phase III

What phase clinical trial: | Studies after drug is approved for Post-Market Safety Monitoring

Phase IV

Describe the phases of the cancer immunoediting concept

1. Elimination phase, the immune system is able to detect a developing tumor and destroy it. In the 2. Equilibrium phase, the tumor cell is able to change such that it can survive the elimination but is still contained by the immune system. 3. Escape phase, the tumor cells have changed so much that they can evade immune recognition and destruction and progressively grow.

What is tx for: | CML

imatinib, Bcr-Abl tyrosine kinase inhibitor

What is tx for: | polycythemia vera

aspirin, phlebotomy, hydroxyurea, ruxolitinib

What is tx for: | essential thrombocytosis

aspirin, if thrombosis risk is high; hydroxyurea

What is tx for: | multiple myeloma

daratumumab, anti-CD38 Ab specific for plasma cells

What is tx for: | CLL

ibrutinib, fludarabine

What is tx for: | non-Hodgkin's B cell lymphoma

rituximab, anti-CD20; CAR T cell therapy

What is tx for: | myelofibrosis

hydroxyurea, ruxolitinib

What is tx for: | melanoma

nivolumab/anti-PD1

What is histologic morphology for neuroblastoma?

monotonous small cells with dense, blue nuclei. The characteristic microscopic feature of a neuroblastoma is the pseudorosette, a ring of primitive neuroblasts surrounding a central space filled with fibrillar extensions from the cells.

What is the reason for lead time bias?

Lead-time bias is due to early detection. Remember the "d" in lead is for early detection.

What is the reason for length time bias?

Length-time bias is due to slow cases being detected more often simply because they are slowly progressing. Remember the "g" in length is for slowly progressing.

What is the most important toxicity of bleomycin?

pulmonary fibrosis antibiotic chemotherapeutic agent that produces single- and doublestrand breaks

What is the most important toxicity of cisplatin?

renal toxicity and ototoxicity

What is the most important toxicity of doxorubicin?

cardiomyopathy DNA intercalation

What is the most important toxicity of mechlorethamine?

myelosuppression nitrogen mustard compound used in Hodgkin disease that alkylates DNA, especially at the N7 position of guanine

What is the most important toxicity of vincristine?

peripheral neuropathy, manifested as numbness, tingling, and pain natural product derived from the vinca plant and acts by interfering with microtubule assembly. It is useful in HL. A very similar vinca alkaloid, vinblastine, has the same mechanism of action, but causes myelosuppression rather than neuropathies

Match the presentation to the genetic defect of this variation of thalassemia. 1 alpha-chain gene deleted

Asymptomatic, microcytosis

Match the presentation to the genetic defect of this variation of thalassemia. 2 alpha-chain genes deleted

Mild anemia with increased RBC count

Match the presentation to the genetic defect of this variation of thalassemia. 3 alpha-chain genes deleted

Severe anemia, HbH production (beta4)

Match the presentation to the genetic defect of this variation of thalassemia. 4 alpha-chain genes deleted

Hydrops fetalis, HbBarts (alpha4)

Match the presentation to the genetic defect of this variation of thalassemia. 1 gene with diminished beta-chain production

Asymptomatic with increased RBC count, microcytosis

Match the presentation to the genetic defect of this variation of thalassemia. 2 genes with no beta-chain production

Severe anemia a few months after birth, with massive erythroid hyperplasia and dependence on transfusions

Match the presentation to the genetic defect of these variations of thalassemia. 2 genes with no beta-chain production

Severe anemia a few months after birth, with massive erythroid hyperplasia and dependence on transfusions

Common mutations in adenocarcinoma (non small cell lung cancer)?

KRAS, EGFR, ALK

What does protein S bind to when it's not in its free form?

C4b Note that C4b is increased in hyperestrogenic states like pregnancy, sickle cell disease, HIV