Heme/Onc Flashcards
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.
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.
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.
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.
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
What side effect of treatment is more common after radical prostatectomy than with radiation therapy or androgen deprivation?
Urinary leakage
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
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.
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.
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.
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.
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.
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)
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.
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.
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.
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.
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.
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.
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.
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.
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.
What are the causes of eosinophilia?
Allergic reactions, malignancies, parasitic infections, autoimmune diseases, some medications, and hypereosinophilic syndrome.
What famous syndrome was caused by tryptophan contaminants?
Eosinophilia myalgia syndrome.
What finding on peripheral blood smears is pathognomonic for AML?
Auer rods.
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.
What is the treatment and prognosis for AML?
Chemotherapy. The prognosis is poor, as the disease usually relapses.
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.
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.
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.
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.
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.
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.
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.
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.
What ethnicity is it highest risk for multiple myeloma?
African-Americans
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.
What tissue stain is used to make the diagnosis of amyloidosis?
Congo red stain
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.
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.
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.
