Hematology/oncology Flashcards

1
Q

How are gastrinomas diagnosed?

A

If serum gastrin level is >1000, it is diagnostic (should also check gastric pH because achlorhydria can cause elevated levels). If level is from 110-1000, it is non-diagnostic and a secretin stim test should be done (inhibits normal gastric G cells and stimulates release from gastrinoma cells)

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2
Q

What diseases are burr cells associated with?

A

ESRD and liver disease

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3
Q

What type of lung cancer is associated with parathyroid hormone-related protein (PTHrP) production?

A

Squamous cell carcinoma

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4
Q

What is Waldenstrom macroglobulinemia?

A

Lymphoplasmocystic malignancy characterized by excessive production of monoclonal IgM. Elevated IgM leads to hyperviscosity syndrome (diplopia, tinnitus, headache, dilated/segmented funduscopic findings), neuropathy, cryoglobulinemia. Neoplastic infiltration of tissue causes hepatosplenomegaly, lymphadenopathy, and cytopenias. Peripheral smear may show rouleaux formations. Dx confirmed by bone marrow biopsy showing >10% clonal B cells.

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5
Q

What are signs and symptoms of polycythemia vera?

A

Increased blood viscosity leading to HTN, erythromelalgia (burning cyanosis in hands/feet), and transient visual disturbances. Increased RBC turnover can cause gouty arthritis. Pruritus in hot water, facial plethora, splenomegaly, and bleeding.

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6
Q

How is polycythemia vera treated?

A

Serial phlebotomy. Can add hyroxyurea (bone marrow suppression) if increased risk for thrombus

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7
Q

What are complications of polycythemia vera?

A

Thrombosis, myelofibrosis and acute leukemia

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8
Q

How are EPO levels affected by polycythemia vera?

A

Low. Caused by JAK2 mutation, not EPO

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9
Q

What type of tumor produces beta-hCG and AFP?

A

A nonseminomatous germ cell tumor.

Note that both seminomatous and nonseminomatous germ cell tumors produce beta-hCG, but only nonseminomatous produce AFP.

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10
Q

What is the treatment of choice for CNS lymphoma?

A

High-dose methotrexate. Also responds to whole brain radiation

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11
Q

What coagulopathy is a common cause of a false positive VDRL?

A

Antiphospholipid antibody syndrome

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12
Q

How does thyroid lymphoma present?

A

Often in patients with Hashimoto thyroiditis though very rare. Presents as a rapidly enlarging, firm goiter associated with compressive symptoms (e.g. dysphagia, hoarseness). May also have systemic B symptoms. If extends into the retrosternal space, can compress vascular structures leading to distended neck veins and facial plethora

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13
Q

How does a cardiac myxoma present?

A

80% are located in L atrium. Get constitutional sx of fever, weight loss, and possible Raynaud phenomenon. Can lead to mitral disease and heart failure. Can even invade into lung parenchyma causing respiratory sx.

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14
Q

What is Trousseu’s syndrome and what is associated with?

A

Migratory superficial thrombophlebitis. Associated with an occult visceral malignancy: pancreatic is most common but also lung, prostate, stomach, and colon cancer and acute leukemias

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15
Q

What vitamin deficiency may result from carcinoid syndrome?

A

Niacin deficiency. Both serotonin and niacin use tryptophan as a precursor so advanced carcinoid syndrome can deplete tryptophan

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16
Q

What lab test can help differentiate chronic myeloid leukemia from a leukamoid reaction?

A

The leukocyte alkaline phosphatase score (low=CML, high=leukamoid)

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17
Q

What cancer are patients with pernicious anemia at risk for?

A

Gastric cancer - leads to chronic atrophic gastritis which puts patients at risk for developing gastric malignancy

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18
Q

What are the colonoscopy guidelines for patients with familial adenomatous polyposis?

A

Start at age 10-12 and get annually

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19
Q

What are the colonoscopy guidelines for patients with HNPCC (Lynch syndrome)?

A

Start at age 20-25 and repeat every 1-2 years

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20
Q

What are clinical features of paroxysmal nocturnal hemoglobinuria?

A

An autoimmune hemolytic disorder characterized by intra and extravascular hemolysis and hemoglobinuria. Due to an acquired genetic defect leading to lack of CD55 and CD59 which protect the cell from complement and MAC
–> hemolysis. Pts have a tendency towards venous thromboembolism. On average patients manifest with symptoms in the fourth decade of life and will classically have fatigue, hemolysis, cytopenias, and venous thrombosus.

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21
Q

How are brain mets usually managed?

A

Surgical resection is recommended for solitary brain mets in patients with good performance status and stable extracranial disease. In patients with multiple brain mets, whole brain radiation therapy is typically used

22
Q

What are common features of non-small cell lung cancer?

A

Cxr showing peripheral cavitations in lungs and CT showing distant mets

23
Q

When comparing ITP, TTP and DIC, which are associated with schistocytes?

A

DIC and TTP

24
Q

How are PT and PTT affected in ITP, TTP, and DIC?

A

DIC: both increased
ITP: both normal
TTP: both normal or slightly increased

25
Q

How is TTP treated?

A

Plasma exchange

26
Q

What are causes of TTP?

A

ADAMTS13 enzyme deficiency, ticlopidine, quinine, cyclosporine, HIV, cancer

27
Q

What are symptoms and features of Waldenstrom Macroglobulinemia?

A

Dizziness, HA, hearing/vision problems and monoclonal IgM M-spike

28
Q

What are smudge cells pathognomonic for?

A

Chronic lymphocytic leukemia (CLL)

-Also see mature lymphocytes on smear

29
Q

How should hypercalcemia of malignancy be treated?

A

With bisphosphonates

30
Q

How does intravascular vs extravascular hemolysis affect haptoglobin levels?

A

Intravascular causes marked reductions in haptoglobin whereas extravascular (e.g. reticuloendothelial system) has normal to slightly low haptoglobin levels

31
Q

What conditions are spherocytes associated with?

A

Autoimmune hemolytic anemia (AIHA) and hereditary spherocytosis

32
Q

What kind of lung cancer does asbestos exposure increase the risk for?

A

Bronchogenic carcinoma

33
Q

How are antibiotics used in hemolytic uremic syndrome?

A

Do not use them. They can lyse the bacteria leading to increased toxin release

34
Q

What are the key findings in tumor lysis syndrome?

A

Nausea, vomiting, fatigue, and muscle cramps and laboratory findings of increased potassium, urea nitrogen and phosphorus and decreased calcium

35
Q

What hypercoagulability syndrome will have an unchanged PTT after administration of heparin?

A

Antithrombin III deficiency. Heparin works by potentiating the binding of AT III to thrombin and X. So despite administering heparin, the aPTT remains normal because heparin requires the presence of AT III to achieve its intended effect.

36
Q

Is AML or ALL myeloperoxidase positive?

A

AML

37
Q

What is the most sensitive test for hereditary spherocytosis?

A

The EMA binding test analyzes the interaction between a dye and certain proteins on the RBC surface through flow cytometry. It is a rapid test that is highly sensitive and specific for hereditary spherocytosis.

38
Q

What is the treatment for ITP in adults?

A

Corticosteroids. Splenectomy is indicated for patients with refractory symptoms after starting steroids

39
Q

What is the presentation of CLL?

A

Painless generalized lymphadenopathy and splenomegaly in an older adult. B symptoms are common. Often exposed to organic solvents, e.g., working in a factory producing shoes.

40
Q

Why do patients with Hodgkin’s lymphoma get hypercalcemia?

A

Ectopic vitamin D production as a result of increased 1α-hydroxylase activity in proliferating lymphoid tissue is the most common cause of hypercalcemia in patients with Hodgkin lymphoma. Elevated levels of active vitamin D cause excess intestinal resorption of calcium, resulting in hypercalcemia, which, in turn, causes decreased levels of the parathyroid hormone as a result of negative feedback.

41
Q

Why do patients with hereditary spherocytosis get a macrocytic anemia?

A

The increased RBC turnover leads to folate deficiency. All patients with hemolytic anemia should receive folate supplementation.

42
Q

What is mycosis fungoides?

A

The most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesions, and itchy skin. Can progress to Sézary disease which is an aggressive form

43
Q

What blood cancer is associated with tartrate-resistant acid phosphatase activity?

A

Hairy cell leukemia

44
Q

What are characteristics of Hodgkins lymphoma that are associated with a worse prognosis?

A

An elevated ESR (≥ 50 mm in the first hour in patients without B symptoms; any ESR value in patients with B symptoms), male gender, age > 45 years, anemia (< 10.5 g/dl), stage IV disease, white blood cell count ≥ 15,000/mm3, albumin < 4 g/dL and lymphocyte count < 600/mm3. Also involvement of three or more lymph node areas.

45
Q

What subtypes of Hodgkin lymphoma have the best and worst prognosis?

A

Nodular lymphocyte predominant type tumor (NLPHL) (∼ 5% of cases of Hodgkin lymphoma) has the best prognosis of all types of Hodgkin lymphoma. Lymphocyte-depleted HL (LDHL), by contrast, is a very rare (< 1%) but aggressive type of HL that is associated with a poor prognosis.

46
Q

What blood cancer are smudge cells seen in?

A

CLL

47
Q

What kind of infections are patients with multiple myeloma most susceptible to?

A

UTIs and pneumonia - because of the uncontrolled production of ineffective, monoclonal immunoglobulins (commonly IgA and IgG) and lymphocyte dysfunction.

48
Q

What medication is used to treat primary myelofibrosis?

A

Ruxolitinib is a JAK 2 inhibitor that is used to treat patients with primary myelofibrosis who have severe constitutional symptoms and/or symptomatic splenomegaly and cannot undergo allogeneic stem cell transplantation.

49
Q

What is the gold standard for diagnosing HIT?

A

A serotonin release assay - serotonin is a marker of platelet activation. So high serotonin release indicates HIT

50
Q

What do necrotic lesions at the site of heparin injection indicate?

A

HIT