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Flashcards in Heme/Onc Deck (103):
0

How do you tell if a patient with monoclonal gammopathy of undetermined significance has progressed to multiple myeloma?

They would show evidence of myeloma related and organ impairment in the presence of am protein,, monoclonal plasma cells, or both. This may include hypercalcemia, renal failure, anemia, or skeletal lesions. Monoclonal gammopathy of undetermined significance does not progress steadily to multiple myeloma. there is a stable 1% annual risk of progression.

1

A history of low-dose estrogen oral contraceptive use causes a decreased risk of what kinds of cancer? What kinds of cancer do not show reduce rates due to taking these contraceptives?

There is at least a 50% lower risk of subsequent epithelial ovarian cancer in women who have ever used low-dose estrogen oral contraceptives. data also suggest that other potential long-term benefits of oral contraception include a reduction of risk of endometrial and colorectal cancers and of postmenopausal fractures. Oral contraceptives do not reduce the risk of carcinoma of the breast, cervix, lung, or head and neck.

2

What is the treatment for hypercalcemia of malignancy?

First-line therapy is fluid replacement with normal saline to correct the volume depletion that is invariably present and to enhance renal calcium excretion. Intravenous pamidronate has become the mainstay of treatment for hypercalcemia of malignancy, but it is considered only after the hypercalcemic patient has been rendered euvolemic. This would also be true for other calcium lowering agents. Loop diuretic use should be restricted to patients who are in danger of fluid overload.

3

Who is at higher risk for colon cancer, and how often should they be screened?

Higher risk patients include those who have one first-degree relative diagnosed with colorectal cancer or adenomatous polyps before age 60, or at least two second-degree relatives with colorectal cancer. They should start colon cancer screening at age 40, or 10 years before the earliest age at which an affected relative was diagnosed (whichever comes first. )They should be rescreened every five years. Colonoscopy is the preferred screening method for this group as high-risk patients are more likely to have right sided: lesions.

4

What kinds of conditions cause eosinophilia?

C connective tissue diseases such as Churg Strauss vasculitis

H Hellminthic infections including ascariasis, schistosomiasis, and trichinosis

I idiopathic hypereosinophilic syndrome

N neoplasia such as lymphoma

A allergies and atopic diseases such as asthma and allergic rhinitis

5

What side effect of treatment is more common after radical prostatectomy than with radiation therapy or androgen deprivation?

Urinary leakage

6

What is the most appropriate follow up for a healthy 60-year-old black man with a family history of colon cancer and minor symptoms of gastrointestinal illness, with multiple adenomas with high-grade dysplasia, and who was surgically treated for multiple one centimeter polyps found at the rectosigmoid junction?

Initial polypectomy, complete colonoscopy and repeat colonoscopy in three years to check for metachronous adenomas

7

What individuals are at relatively high risk for developing advanced adenomas of the colon?

When should they be rescreened after their first colonoscopy?

Patients with multiple adenomas (greater than three), A large adenoma (greater than 1 cm), a high-grade dysplasia, or a positive family history of colon cancer.

Their first follow-up colonoscopy should be done at three years.

8

What is Wilms tumor?

What is the common presentation of Wilms tumor?

Neuroblastoma

It usually presents in the first year of life. It presents as an abdominal mass. Hematuria occurs as the disease progresses.

9

What are the clinical features of thrombotic thrombocytopenic purpura? How is it treated?

It is a condition of unknown etiology characterized by thrombocytopenia, microangiopathic hemolytic anemia, and various neurologic abnormalities. CNS symptoms occur in 50% of patients. The symptoms are often fluctuating but may include headache, altered mental status, seizures and stroke. Fever and renal abnormalities can also be present to varying degrees. Hyperbilirubinemia is often present because of the hemolysis and the LDH is frequently elevated out of proportion to the hemolysis.

TTP is considered a medical emergency and is initially treated by daily plasmapheresis until a safe platelet count (150,000 per microliter) is achieved and serum LDH begins to fall. Long-term responses have been achieved in about 90% of patients with this therapy. Corticosteroids such as prednisone are often used in conjunction with plasmapheresis but are ineffective when used as a sole agent. Antiplatelet agents such as dyperidamole and aspirin remain of unproven benefit. Platelet transfusion is of no benefit and may actually lead to worsening of symptoms, although the cause of this is unclear. Splenectomy maybe beneficial but is generally reserved for refractivity cases or for preventing the occurrence of relapses.

10

What is the Philadelphia chromosome? In what disease is it seen?

It is a translocation of chromosomes 9 and 22, which creates the Bcr-abl tyrosine kinase oncogene. It is most commonly associated with CML. It can be seen in AML, usually in the AML – M1 subtype, and carries a poor prognosis.

11

What percent of patients with AML have identifiable chromosomal abnormalities?

Approximately 80% of patients with AML have identifiable chromosomal abnormalities. Most commonly seen are translocations, which are often associated with specific subtypes of AML, and can have prognostic significance.

12

What is Pelger-Huet anomaly?

It is a congenital Autosomal dominant disorder in which nuclei fail to segment normally. In the homozygous state the nucleus is round. In heterozygotes most granulocytes have bilobed nucleus nuclei ("pince-nez" cells)

13

What is the most reliable laboratory test for iron deficiency anemia, especially when it is early?

Serum ferritin. Although serum ferritin is an acute phase reactants it is elevated or normal in the face of an inflammatory process. A low serum ferritin is diagnostic for iron deficiency even in its early stages.

14

What are the common types of polyps found at colonoscopy, and which are most likely to be benign or malignant?

Hamartomatous polyps and hyperplastic polyps are benign lesions and are not considered to be premalignant. Adenomas have the potential to become malignant. So sorry I will adenomas and lesions greater than 1 cm have a higher risk for becoming malignant. Of the three types of adenomas: tubular, tubulovillous, and villous, villous adenomas are the most likely to develop into an adenocarcinoma.

15

What is the target hemoglobin level for an anemic patient treated with Erythrpoetin?

A hemoglobin level of 10 to 12. The incidences of stroke, heart failure and death increase in patients targeted to normal hemoglobin levels, and there is no demonstrable decrease in cardiovascular events.

16

At what platelet counts should patients with thrombocytopenia receive platelet transfusions?

For most patients a platelet count of less than 10,000 indicates the need for transfusion. A count below 50,000 is an indication for platelet transfusion in patients undergoing invasive procedures.

17

What is Burkitt's lymphoma?

It is a form of non-Hodgkin's lymphoma. It is endemic in parts of Africa and usually related to Epstein-Barr virus infection.

18

What kinds of non-Hodgkin lymphoma have a better prognosis?

Aggressive and highly aggressive non-Hodgkin's lymphoma's have a better prognosis then indolent lymphomas.

19

What it does reported atypical or reactive lymphocytes on a peripheral blood smear mean?

This is usually a benign finding. It occurs with most viral infections.

20

What does report of abnormal lymphocytes on a peripheral blood smear mean?

This is usually not a benign finding. It often means leukemia or lymphoma.

21

In what clinical situations do you see neutrophilia?

In leukemoid reactions. These include infection (bacterial includes chronic bronchitis related to smoking), inflammatory response, and medication effect (corticosteroids). They also include chronic leukemia.

22

What are the causes of eosinophilia?

Allergic reactions, malignancies, parasitic infections, autoimmune diseases, some medications, and hypereosinophilic syndrome.

23

What famous syndrome was caused by tryptophan contaminants?

Eosinophilia myalgia syndrome.

24

What finding on peripheral blood smears is pathognomonic for AML?

Auer rods.

25

What are the causes of aplastic anemia?

Drugs or chemicals such as benzene or chloramphenicol, infections particularly hepatitis C, or idiopathic/immunologic. 70% falls in the idiopathic/immunologic category.

26

What is the treatment and prognosis for AML?

Chemotherapy. The prognosis is poor, as the disease usually relapses.

27

What is JAK-2?

It is an enzymatic marker for normal cell growth. Acquired clonal mutations of this gene have been identified which lead to uncontrolled cell growth and survival. These abnormal clones are present in 90% of patients with polycythemia vera, in 50% of patients with essential thrombocytosis, and in 50% of patients with primary myelofibrosis.

28

How is the diagnosis of polycythemia vera made?

By the presence of JAK-2 mutation and a low Epo level. The red cell count is usually high, but may be low in the spent phase.

29

What is the treatment and prognosis for polycythemia vera?

Low-dose aspirin decreases the risk of all vascular complications without an increased risk for major bleed. It does not affect long-term survival. Other treatments include phlebotomy and hydroxyurea. The survival is greater than 10 years.

30

What other conditions besides polycythemia may cause erythrocytosis? How are these conditions distinguished from polycythemia vera?

Conditioned that are associated with a chronic hypoxia, which include COPD and sleep apnea. Erythrocytosis may also be associated with an increase in testosterone. They're distinguished from polycythemia by the absence of the JAk-2 mutation and by the Epo level being normal to high.

31

How is the diagnosis of essential thrombocytosis made?

There are sustained platelet counts of greater than 600,000 per microliter. There are bone marrow changes of essential thrombosis. Criteria are not met for polycythemia vera, CML, myelofibrosis, or MDS. There is no evidence of a reactive state such as iron deficiency, inflammation, infection, or other malignancy. The patient has not had a splenectomy. Note that the JAK-2 mutation occurs in 50% of patients.

32

What are the clinical findings in CML?

60% have clinical findings such as fatigue anorexia and weight loss, but splenomegaly or hepato- make a way. 40% are asymptomatic. Blood findings include elevated white blood count of over 25,000 per microliter, thrombocytosis in 30 to 50%, basophilia, and reduced leukocyte alkaline phosphatase.

33

What is the treatment and prognosis for CML?

There are four commercially available inhibitors of the Bcr-abl oncogene. There is a 90% response rate with prolonged remission.

34

Define monoclonal gammopathy of undetermined significance

It is a disease found largely in old people. It occurs in about 5% of all individuals over age 70. 75% of it occurs over age 70, with 10 to 15% over age 80. It is associated with high serum paraprotein levels but to make the diagnosis the level must be less than 3 g. There can be no lytic bone lesions, and no evidence of end organ damage such as anemia, hypercalcemia, or renal insufficiency. About 1% per year of patients get myeloma. There's higher risk if the protein level is higher.

35

What ethnicity is it highest risk for multiple myeloma?

African-Americans

36

What are the clinical features of primary amyloidosis?

30 to 40% of patients have neuropathies such as polyneuropathy, orthostatic hypotension, or carpal tunnel syndrome. 50 to 60% have enteropathy including hepatomegaly, macroglossia, and diarrhea with or without malabsorption. 80% have nephropathy, which includes renal function loss and proteinurea. 40 to 50% have cardiomyopathy, often with heart failure.

37

What tissue stain is used to make the diagnosis of amyloidosis?

Congo red stain

38

How is the diagnosis of primary amyloidosis made?

90% of patients will have an abnormality of either serum peptide electrophoresis or urine peptide electrophoresis. The diagnosis is confirmed by Congo bad stains of the bone marrow, subcutaneous fat pad, or other infected tissue.

39

What are the stages and treatments for CLL?

Staging is done using Rai staging. Stage 0 has lymphocytosis only. Stage I involves lymphocytosis and lymphadenopathy. Stage II has lymphocytosis, lymphadenopathy and hepatosplenomegaly. Stage III has lymphocytosis lymphadenopathy hepatosplenomegaly and anemia. Stage IV has lymphocytosis, lymphadenopathy, hepatosplenomegaly and thrombocytopenia with or without anemia. Stages zero and one are observed only. Stages two through four are treated with chemotherapy.

40

How is hemophiliac treated in children versus adults?

Prophylactic dosing of replacement factors has been shown to be useful in infants and children. Prophylactic dosing has not been proven useful in adults, and they generally use on-demand therapy.

41

What laboratory abnormality is found in hemophilia?

A prolonged PTT

42

How is Von Willebrand's disease treated?

Mild cases are treated with DDAVP, which increases the release of von Willebrand's factor. More severe cases are treated with replacement of von Willebrand factor itself.

43

What are the clinical manifestations of von Willebrand's disease?

Mucocutaneous bleeding such as epistaxis, G.I. bleed, or menorragia.

44

What is the treatment of vitamin K deficiency?

Vitamin K if it is relatively mild, or fresh frozen plasma for more severe cases.

45

Does von Willebrand disease cause a prolonged PTT?

No

46

What is Trousseau's syndrome?

Migratory thrombophlebitis in a cancer patient

47

What are the features of acute ITP?

It occurs in children aged 2 to 6, the male to female ratio is about 1/1. There is often an antecedent infection. Other associated illnesses are rare. The onset of symptoms his sudden. Platelet count at presentation is less than 20,000 per microliter. The duration is 2 to 6 weeks and spontaneous remission is common.

Bone marrow exam is usually unnecessary and children if they have typical symptoms. Treatment is not generally needed because they have no bleeding or only skin manifestations regardless of platelet count. If treatment is needed it is usually a short course of corticosteroids or IV IG single-dose.

48

What are the features of chronic ITP?

It is a disease of adults aged 20 to 40. The female to male ratio is 2-3/1. There is rarely an antecedent infection. It is occasionally associated with CLL or autoimmune disease. The onset of symptoms is variable. The platelet count at presentation is less than 50,000 platelets/microliter. The duration is long-term and spontaneous remission is rare.

Treatment decisions are based on the individual patients bleeding risk, activity, likely side effects, and preferences. Treatment is usually recommended for newly diagnosed patients with platelet counts of less than 30,000. First-line treatment is corticosteroids such as prednisone 1 mg per kilogram for 21 days then taper. IVIG and corticosteroids are used for a more rapid response. IVIG or anti-– D are used if corticosteroids are contraindicated.

49

What drugs commonly caused a drug induced thrombocytopenia?

Quinidine and quinine, sulfonamides, sulfonylureas, rifampin and vancomycin.

50

If a hospitalized patient develops thrombocytopenia, what drug should you consider as a cause?

Vancomycin

51

What are the features of heparin – induced thrombocytopenia? When does its onset occur?

It is usually a relatively mild thrombocytopenia. It involves the decline of the platelet count to less than 100,000 or I reduction by more than 50%. The median platelet count at diagnosis is 60,000. It occurs at about 14 days after heparin is started. It may occur immediately if the patient had previous happen exposure within three months

52

What is the presentation and treatment of heparin-induced thrombocytopenia?

It presents as a thrombotic event in about 25% of people, venous more than arterial. It can result in amputation in up to 10% due gangrene. It can cause death in 10 to 20%. It is treated with an alternate anticoagulant which should not include any kind of heparin or low molecular weight heparin.

53

What are the clinical findings in the thrombotic thrombocytopenic Purpera? How is it treated?

Fever, neurologic changes, renal impairment, microangiopathic hemolytic anemia, and thrombocytopenia. The hemoglobin is usually less than 10 g/dL and there are laboratory findings of hemolysis. The thrombocyte count is usually less than 20,000. It is treated with plasma exchange plasmapheresis daily.

54

What is hemolytic uremic syndrome?

It is a state that is triggered by E. coli 0157: H7. It involves microangiopathy, thrombocytopenia and renal failure. There are atypical HUS syndromes which are not associated with diarrhea. They include drug-induced HUS, which can be caused by cyclosporine or tacrolimus. They can also be caused by an inherited complement deficiency. They are not associated with the abnormality in the antibody to ADAMTS – 13 which is seen with typical HUS.

55

What is Virchow's triad for risks for venous thromboembolism?

Venous stasis, vascular stasis, and hypercoagulability.

56

What is the most common inherited risk factor for venous thromboembolism?

Factor V Leiden

57

What is the most effective way to rule out pulmonary embolism in a low risk patient?

A D – dimer level. This has a high negative predictive value.

58

What lab is used to monitor levels of low molecular weight heparin?

Antifactor Xa activity.

59

In what conditions are Bence-Jones proteins found in the urine?

Multiple myeloma and Waldenström's macroglobulinemia

60

Serum neuron specific enolase, or NSE, testing is useful for diagnosis of what types of tumors?

Neuroblastoma and pheochromocytoma

61

Homovanillic or HVA, and vanillylmandelic or VMA are useful for monitoring what types of tumors?

Neuroblastoma's and pheochromocytomas

62

Beta human chorionic gonadotropin levels are useful for monitoring what types of tumors?

Liver tumors and germ cell tumors

63

Alpha-fetoprotein levels are useful for monitoring what types of tumors?

Malignant teratomas, liver and germ cell tumors

64

What are the risk factors for endometrial cancer?

Conditions involving increased or prolonged estrogen exposure including obesity, nulliparity, early menarche, late menopause, chronic anovulation, and unopposed exogenous estrogen use. In addition advancing age, hypertension, diabetes, and tamoxifen use our risk factors.

65

What are the protective factors for endometrial cancer?

Progesterone use, oral contraceptive pills, cigarette smoking, multiparity, breast-feeding, and physical activity.

66

What is hereditary non-polyposis colorectal cancer?

It is also called Lynch syndrome. It is an autosomal dominant genetic condition that causes a high-risk for cancers including colon, which has an 80% lifetime risk, endometrium, which has an 80% lifetime risk, ovarian, stomach, small intestine, hepatobiliary tract, upper urinary tract, brain and skin.

67

What are raloxifene and tamoxifen? What is their effect on the risk for uterine cancer?

They are SERMs or selective estrogen receptor modulators. They are both used to treat breast cancer. Raloxifene is also used to treat osteoporosis. Raloxifene has not been shown to increase the risk of uterine cancer, whereas tamoxifen has.

68

Post menopausal uterine bleeding has about what relationship to endometrial cancer?

Abnormal uterine bleeding is the presenting sign in 85% of women with endometrial cancer. However endometrial cancer is the cause of only 10% of post menopausal uterine bleeding. Most bleeding is due to benign endometrial abnormalities such as atrophy, polyps and fibroids.

69

What Is the treatment for a reproductive age woman who has an endometrial biopsy that shows hyperplasia without cytologic atypia?

What about with cytologic atypia?

If there is simple and Demetrail hyperplasia with abnormal bleeding the woman is treated with progestin withdrawal for six months then rebiopsy. If there is complex or adenomatous endometrial hyperplasia the treatment is also progestin withdrawal then rebiopsy.

If there is cytologic atypia, the treatment is high dose progestins or Megace or Depo-Provera for three months and then re-biopsy.

70

What is the treatment for a post menopausal woman who has abnormal uterine bleeding and a uterine biopsy without or with atypia?

If there is no cytologic atypia the patient is given progestins for six months and then re-biopsy. A total abdominal hysterectomy is performed for recurrent endometrial hyperplasia or bleeding.

If there is cytologic atypia, a hysterectomy is performed.

71

How is transvaginal ultrasound used to monitor postmenopausal women with abnormal uterine bleeding?

Originally the recommendation was that women who have an endometrial stripe of less than 4 to 5 mm can be observed and those with greater than 5 mm should get endometrial biopsy.

Subsequent meta-analysis has shown that this test is not sensitive enough to used as the only test in women with abnormal uterine bleeding. There is a 50% false positive rate. 4% of cancers are missed even when liberal guidelines for referral and D&C are used. They concluded that the cut off should be an endometrial stripe of less than or equal to 4 mm if women are at high risk and also that a histological sample should be obtained if possible.

72

What ethnic groups are at increased risk for ovarian cancer?

Ashkenazi Jews, French-Canadians and Icelanders

73

What is the relationship of the BRCA1/2 gene and ovarian cancer?

Among all women who present with ovarian cancer, only about 8% have a BRCA mutation. Among Ashkenaz he women who present with ovarian cancer, 40% have a BRCA mutation.

74

What factors decrease the risk for ovarian cancer?

It is thought that ovarian cancer arises due to abnormal scar formation and reparative process after ovulation. Therefore anything that inhibits ovulation decreases the risk for ovarian cancer. Factors include oral contraceptive use, breast-feeding especially if over 18 months, one or more full-term pregnancy prior to age 35, early menopause or late menarche, low-fat diet, prophylactic oophorectomy.

Avoiding talc powder in genital hygiene also decreases the risk.

For unknown reasons tubal sterilization decreases the risk of ovarian cancer by 18 to 40%. It is theorized that tubal ligation stops carcinogens from reaching the ovaries after they enter the body via the vagina.

75

What factors increase the risk for ovarian cancer?

Delayed childbearing, early menarche, late menopause, endometriosis, estrogen replacement therapy for more than five years, family history suggesting genetic predisposition, genetic syndromes, high-fat diet, low parity.

76

What are the current USPSTF recommendations in regards to screening for cervical cancer?

Women under 21 should not be screened. Women 21-65 should be screened with cytology every three years if they have had intercourse and have a cervix. Screening cytology and HPV should not be done in women under 30. HPV and cytology should be done in women over 30. If both are negative rescreening may be performed every 3 to 5 years.

77

When should the screening frequency for cervical cancer be increased?

Women with HIV should be screened twice a year after the diagnosis of HIV, then annually if cytology is normal.

Women with DES exposure in utero and women with a history of cervical cancer should be screened annually as long as the patient is in good health and has no life-threatening condition.

78

What is the management of a finding of CIN 1 on a Pap smear?

Observation is preferred for all ages.

79

What is the management of ASC – US or AGC found on a Pap smear?

ASC – US maybe followed with yearly repeat cytology and HPV testing if the original HPV is negative.

A finding of AGC requires colposcopy for all women. In addition if the woman is over age 35 endometrial sampling should be performed. It should also be performed in women under 35 with risk factors for endometrial neoplasia such as unexplained vaginal bleeding or anovulation. HPV testing should be done in all cases. If no CIN 2 or 3, or glandular neoplasia is found, and HPV testing is positive, repeat HPV testing should be done in six months with a referral to colposcopy if there are atypical squamous cells or positive HPV. If HPV is negative repeat HPV testing should be done at 12 months.

80

What is the management of a Pap smear finding of AGC – favor neoplasia or AIS?

If no invasion is found a diagnostic excision is performed. If AIS is found a hysterectomy is preferred. If childbearing is desired conservative management with colposcopy, psychology, and HB testing at six months.

81

What are the indications for cone biopsy of the cervix?

Inability to see the extent of the lesion at colposcopy. Cervical biopsy demonstrating microinvasion. Positive endocervical curettage. Inadequate colposcopy. Discrepancy between Pap smear and biopsy such as HGSIL on Pap smear and normal biopsy or biopsy with minimal atypia.

82

What is the definition of satisfactory cervical cytology?

Conventional Pap smears must have at least 8 to 12,000 well visualized squamous cells. Liquid-based preparations must have a minimum of 5000 well visualized squamous cells. The specimen must be labeled.

83

What is the management of a Pap smear that is read as partially obscured by blood or inflammation?

If the woman is over 30 and HPV positive she should be managed in one of two ways: repeat cytology after 2 to 4 months or colposcopy.

If the woman is over 30 and HPV negative or unknown, repeat cytology should be performed in 2 to 4 months.

When cytology is repeated at 2 to 4 months the results are managed as follows: if cytology is negative and the woman is HPV positive, HPV and cytology should be repeated in one year. For women who are HPV negative or unknown the patient may resume routine screening. If the repeat cytology is abnormal it should be managed as appropriate. If the repeat psychology is unsatisfactory, colposcopy
should be performed.

84

What do Pap smear results of ASC – US and ASC – H mean?

ASC – US stands for atypical squamous cells of undetermined significance. ASC – H means that there are atypical squamous cells and a high grade squamous intraepithelial lesion cannot be excluded.

85

What do the findings of LSIL, LSIL-H and HISIL on Pap smear mean?

LSIL stands for low-grade intraepithelial lesion. This category includes changes consistent with HPV, mild dysplasia, or CIN 1.

LSIL-H means low grade intraepithelial lesion, cannot exclude high-grade lesion. These specimens usually contain a few cells that are suspicious for, but not diagnostic of high-grade lesion.

HSIL stands for high-grade intraepithelial lesion. This category includes the changes consistent with moderate or severe dysplasia, CIN 2 or 3, and carcinoma in situ.

86

What does a finding of AGC mean on a Pap smear result? How about AGC, favor neoplastic? How about AIS? How about adenocarcinoma?

AGC stands for atypical glandular cells. Upon further evaluation either high-grade squamous or glandular abnormalities are found in 10 to 39% of women with this finding.

AGC, favor neoplastic is a designation used for specimens that show features suggestive of but not sufficient for an interpretation of adenocarcinoma.

AIS stands for endocervical adenocarcinoma in situ.

Adenocarcinoma means frank adenocarcinoma.

87

What is the Gail model for assessment of breast cancer risk?

It is an assessment tool which identifies six predictors to identify women who may be at risk for breast cancer: Age over 50. Early onset of menses at age less than 12 gives you a relative risk of 1.2. Nulliparity or first live birth age 25 to 29 gives you a relative risk of 1.6. First live birth birth over 30 gives you a relative risk of 1.9. A breast biopsy which shows hyperplasia results in a relative risk of 2.0. A breast biopsy which shows atypical hyperplasia results in a relative risk of 5.0.

88

What are the strongest risk factors for breast cancer?

Age over 50 and BRCA mutation.

89

What family history patterns are associated with increased risk of BRCA1 isms 2 gene mutations?

Non-Ashkenaz he Jewish women with: two first degree relatives with breast cancer or one diagnosed at less than or equal to age 50, greater than or equal to three first or second degree relatives with breast cancer regardless of age, a first or second degree relative with both breast and ovarian cancer, a first degree relative with bilateral breast cancer, and history of breast cancer in a male relative.

An Ashkenazi Jewish woman with: any first degree relative or two second degree relatives on the same side of the family with breast or ovarian cancer.

90

What are the benefits and risks of tamoxifen or raloxifene therapy in order to prevent breast cancer in women with BRCA2 mutations?

There is there evidence that tamoxifen decreases the risk of invasive estrogen receptor positive breast cancer in women who carried the BRCA mutation. There is good evidence that both tamoxifen and Raloxifene increase the risk of thromboembolism. Tamoxifen but not raloxifene increases the risk of endometrial cancer.

91

Does the treatment for ovarian cancer include surgery, radiation or both?

Surgery only. Radiation is not helpful.

92

What is the treatment for hypercalcemia of malignancy?

Initial treatment includes fluid replacement with normal sailing. There is buying depletion in variably present. The use of Loop diuretics such as furosemide should be restricted to patients in danger of fluid overload. After the patient has been rendered euvolemic, the mainstay of of therapy is intravenous pamidronate.

93

What patients are at high risk for colorectal cancer and what sort of screening should be done?

Patients who have one first degree relative diagnosed with colorectal cancer or adenomatous polyps before the age of 60, or at least two second degree relatives with colorectal cancer, are in the highest risk group. They should start colon cancer screening at age 40 or 10 years before the earliest age at which the effective relative was diagnosed (whichever comes first) and be rescreened every five years.

94

What is Lynch syndrome?

It is hereditary non-polyposis colorectal cancer or HNPCC. It is a familial form of CRC caused by gene mutations and accounts for approximately 2 to 3% of colorectal cancers. It is also associated with increased risks for cancers other than colorectal, including endometrial, stomach, ovarian, pancreas, ureters and kidney, biliary tract, and brain (usually glioblastoma). As in other familial cancer syndromes, cancers occur earlier than average and multiple cancers tend to occur.

95

What is the typical presentation and what are the laboratory findings in a patient with polycythemia vera?

Pruritis after a hot shower and the presence of splenomegaly helps to clinically distinguish polycythemia vera from other causes of erythrocytosis. Specific criteria for the diagnosis of polycythemia vera include an elevated red cell mass, a normal arterial oxygen saturation of over 92%, and the presence of splenomegaly. In addition, patients usually exhibit thrombocytosis with a platelet count of greater than 400,000, leukocytosis with a WBC of greater than 12,000, a low serum erythropoietin level, and elevated leukocyte alkaline phosphatase score. High carboxyhemoglobin levels are associated with secondary polycythemia.

96

What is the threshold for transfusion in most adults and children?

A hemoglobin of 7 g/dL

98

What ethnic group tends to have slightly lower hemoglobin levels? How much lower?

African-Americans tend to have hemoglobin levels about 1 g/deciliter lower than other ethnic groups in the US.

99

What other tests can be used to confirm a diagnosis of vitamin B12 or folate deficiency when the vitamin levels are not clearly low?

An increased methylmalonic acid level confirms the diagnosis of B12 deficiency. An increased homocysteine level confirms the diagnosis of folate deficiency.

100

What is the recommended adjuvant therapy for women with non-metastatic hormone receptor positive breast cancer? What are the various side effects associated with the agents?

Tamoxifen or aromatase inhibitors are recommended.

Tamoxifen should be given for at least five years to all women do not meet the definition of menopause at the initial diagnosis of breast cancer.

it's side effects include an increased risk of stroke, an increased risk of uterine cancer, hot flashes, vaginal discharge, sexual dysfunction, and menstrual irregularities.

Aromatase inhibitors such as letrozole are administered to postmenopausal women. The side effects of aromatase inhibitors include a higher risk of osteoporosis, fracture, cardiovascular risk, and hypercholesterolemia compared to tamoxifen. Their associated with a lower risk of venous thrombosis and endometrial cancer. There is an AI associated musculoskeletal syndrome characterized by arthralgia, joint stiffness, and/or bone pain, which can be severe in at least a third of patients

101

What is HER-2 positive breast cancer? What adjuvant therapy is recommended for it?

HER-2 two stands for human epidermal growth factor receptor 2. Patients with breast cancer who are HER-2 positive should receive trastuzumab as adjuvant therapy with their chemotherapy.

102

What are the established risk factors for renal cell carcinoma?

Smoking, hypertension, obesity, acquired cystic disease of the kidney as seen in chronic dialysis patients; occupational exposure to compounds such as cadmium, Asbestos,and petroleum by-products; prolonged ingestion of analgesics including NSAIDs, aspirin, and acetaminophen.

103

List and define the types of brain tumors

Gliomas are tumors of glial cells and are the most common brain tumor. Ependymomas, astrocytomas, and oligodendrogliomas are all types of gliomas. They are also classified by grade with glioblastoma multiforme being the most malignant. Prognosis is generally poor.

Medulloblastoma's are highly malignant tumors of embryonal cells. They generally occur in the cerebellum and are more common in children. The five-year survival rate is about 80%.

Meningiomas are usually benign, but can cause problems if they are large. They are most often seen in adults over the age of 60 and are more common in women. If they need treatment the treatment is surgical, and it is often curative.