heme/onc 2 Flashcards
What is an inability to mobilize stored iron adequately for erythropoiesis?
It is not likely to cause functional iron deficiency.
What are the major criteria for Polycythemia Vera (PV)?
- Elevated hemoglobin level (>16.0 g/dL in women; >16.5 g/dL in men) or elevated hematocrit (>48% in women; >49% in men) or elevated red-cell mass.
- Bone-marrow biopsy consistent with PV.
- Presence of the JAK2 V617F or another similar JAK2 mutation.
What is a minor criterion for Polycythemia Vera (PV)?
Serum erythropoietin level below the normal reference range.
Does serum alpha-fetoprotein testing alone confirm the diagnosis of HCC?
No, it does not confirm the diagnosis of HCC.
However, elevations in the setting of imaging studies of high suspicion can be helpful, especially for small nodules.
What are clinical manifestations of Gaucher disease?
Clinical manifestations include hepatosplenomegaly and bone disease.
How may bone disease manifest in Gaucher disease?
Bone disease may manifest as bone pain, swelling, aseptic necrosis, or osteoporosis.
What skin lesions are consistent with a diagnosis of urticaria pigmentosa?
Red-brown maculopapular skin lesions that darken and swell when rubbed (Darier’s sign).
What is Von Hippel–Lindau disease?
A rare autosomal dominant familial cancer syndrome consisting chiefly of retinal angioma, hemangioblastoma of the central nervous system, pheochromocytoma, and clear-cell renal-cell carcinoma.
What is the most appropriate approach to managing intractable back pain in a patient with metastatic cancer?
Initiate bolus doses of intravenous opioids.
What is thrombotic thrombocytopenic purpura classically associated with?
It is classically associated with microangiopathy, thrombocytopenia, renal insufficiency, mental status changes, and fever.
What is renal insufficiency?
A condition where the kidneys fail to adequately filter waste products from the blood.
What are common symptoms of renal insufficiency?
Mental status changes and fever.
What is the most effective management option for symptomatic progressive prostate cancer in an older man with a life expectancy of <10 years who refuses or has a contraindication to surgery?
Radiation therapy with consideration for androgen-deprivation therapy.
What are the effects of Tamoxifen?
Tamoxifen has antiestrogenic effects in the breast and estrogenic effects in the uterus and bone.
What is the most appropriate treatment for patients with chemotherapy-induced nausea and vomiting who do not respond to preventive treatment with prochlorperazine?
Intravenous palonosetron and dexamethasone.
What are clinical manifestations of Gaucher disease?
Hepatosplenomegaly and bone disease.
What complications can arise from Gaucher disease?
Thrombocytopenia and anemia from splenic sequestration.
What is a common finding in PCT?
Excessive hair growth on the face.
What is the most appropriate pharmacologic intervention for smoking cessation in a woman with a history of seizures who cannot tolerate varenicline?
Dual nicotine replacement therapy (patch and oral).
Where do osteosarcomas generally arise?
In the metaphysis of the bone and are characterized by lytic and blastic features.
What is smoldering multiple myeloma?
An asymptomatic clonal plasma cell dyscrasia differentiated from MGUS by a significantly higher risk of progression to multiple myeloma.
What percentage of patients with CLL experience autoimmune hemolytic anemia?
10% to 20%.
What is lupus anticoagulant?
An antibody that leads to a prolonged prothrombin time or partial-thromboplastin time.
What condition is suggested by microcytic anemia in a patient with a normal red-cell distribution width and normal iron stores?
A thalassemia.
When does drug-induced immune-mediated thrombocytopenia typically occur after medication exposure?
A median of 5 to 10 days after exposure to the medication.
What is the median time for lupus anticoagulant detection after medication exposure?
A median of 5 to 10 days after exposure to the medication.
What does lupus anticoagulant lead to?
A prolonged prothrombin time or partial-thromboplastin time.
What does an increase in clotting indicate?
It does not indicate bleeding.
What imaging studies are mainly performed after a malignancy?
Staging studies including CT of the chest, abdomen, and pelvis.
What is confirmed in patients with accelerated-phase CML?
Patients have a blast count of 10% to 20%.
What characterizes patients with CML in blast crisis?
They have >20% blasts in the peripheral blood or bone marrow.
How is localized Kaposi sarcoma managed?
By close observation after initiation of antiretroviral therapy.
What are the symptoms associated with TTP?
Thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, acute kidney injury, and fever.
What is the characteristic of lung involvement in PAN?
It is rare.
What does radiation pneumonitis treatment typically involve?
Glucocorticoid therapy, usually a 2-week course of prednisone followed by a gradual taper.
What medications are used for bone pain from metastatic lesions?
Anti-inflammatory medications such as glucocorticoids and nonsteroidal anti-inflammatory drugs (NSAIDs).
Is LCIS considered a precursor to invasive carcinoma?
No, LCIS is not considered a precursor to invasive carcinoma.
What causes hereditary hemochromatosis (HH)?
Mutations in the HFE gene, such as C282Y or H63D.
What are common culprits for drug-induced immune-mediated thrombocytopenia?
Trimethoprim-sulfamethoxazole, quinine, vancomycin, and penicillin.
What should be ensured for a patient’s platelet count?
It should provide an adequate safety margin for normal daily activities.
What is the safety margin for normal daily activities regarding platelet count?
If a patient’s platelet count is typically above 50, glucocorticoids are not indicated because the side effects outweigh any benefits.
What fibrinogen level indicates increased bleeding risk?
Fibrinogen levels <100 mg/dL indicate that patients are at increased bleeding risk.
How can superwarfarin intoxication be managed?
Patients with superwarfarin intoxication can be managed with fresh frozen plasma and vitamin K, followed by several months of tapering vitamin K therapy.
What mutation is identified in patients with primary myelofibrosis?
The JAK2 V617F mutation is identified in 50% to 60% of patients with primary myelofibrosis.
How is mild asymptomatic carbamazepine-induced leukopenia and neutropenia managed?
Mild asymptomatic carbamazepine-induced leukopenia and neutropenia are best managed by ongoing observation with serial hematologic surveillance.
What symptoms do patients with primary CNS lymphomas present with?
Patients with primary CNS lymphomas present with neurologic deficits or seizures rather than the B symptoms typically seen in patients with systemic lymphomas.
How are adults with ITP usually treated?
Adults with ITP are usually treated with dexamethasone, and most are responsive.
What rearrangement is common in CML?
BCR-ABL rearrangement is common in chronic myeloid leukemia (CML).
What is the treatment for lobular carcinoma in-situ?
The treatment of lobular carcinoma in-situ is surgical excision followed by tamoxifen administration.
What defines a febrile transfusion reaction?
A febrile transfusion reaction is defined as fever >38°C or an increase of at least 1°C from baseline that occurs during or up to 4 hours after a blood transfusion.
What type of lymphoma is Burkitt lymphoma?
Burkitt lymphoma is an aggressive non-Hodgkin lymphoma associated with c-myc translocation, usually in association with Epstein–Barr virus infection.
What is the most appropriate imaging study for suspected renal-cell carcinoma?
The most appropriate imaging study to perform in a patient suspected of having renal-cell carcinoma is CT of the abdomen and pelvis.
What happens to hepcidin production in chronic inflammation?
In patients with chronic inflammation, hepatic production of hepcidin is increased, which causes reduced iron absorption in the duodenum and blockage of iron release from macrophages.
What is the most likely diagnosis in a patient with pancytopenia?
The most likely diagnosis in a patient with pancytopenia is not specified in the provided text.
What is the most likely diagnosis in a patient with pancytopenia?
Acute promyelocytic leukemia (APL)
Auer rods, disseminated intravascular coagulation, and the chromosomal translocation t(15;17) are associated with APL.
What characterizes T-cell large granular lymphocyte leukemia?
The lymphocytes are large with azurophilic granules and are of T-cell origin.
What is the most appropriate next step for a woman with an inflamed breast that has not responded to initial oral antibiotics?
Biopsy of the inflamed breast tissue.
What happens to hepatic production of hepcidin in patients with chronic inflammation?
Hepatic production of hepcidin is increased, causing reduced iron absorption in the duodenum and blockage of iron release from macrophages.
What does evidence of hemolysis and peripheral spherocytosis suggest?
Autoimmune hemolytic anemia.
What is the goal ferritin level for therapeutic phlebotomy in hereditary hemochromatosis (HH)?
50 to 100 ng/mL
Although some centers perform phlebotomy to achieve a ferritin level of <50 ng/mL, many patients will feel the effects of iron deficiency at this level.
What is an uncommon but potentially fatal complication in patients receiving oral agents that inhibit the tyrosine kinase activity of epidermal growth factor receptor?
Interstitial lung disease
Example agents include Erlotinib and Gefitinib.
What does pancytopenia without peripheral blood smear evidence of dysplasia or circulating blasts suggest?
Aplastic anemia.
What characterizes anemia of chronic inflammation?
It is also characterized by a low reticulocyte count.
What factors are thought to precipitate porphyria cutanea tarda (PCT)?
Multiple risk factors contributing to iron accumulation and/or oxidative stress in hepatocytes.
What occurs after platelet aggregation?
Formation of a white thrombus composed of platelets and fibrinogen.
What is the most frequent myeloproliferative neoplasm associated with Budd Chiari syndrome?
Polycythemia vera.
What syndrome is associated with primary CNS lymphomas?
Budd Chiari syndrome
What symptoms do patients with primary CNS lymphomas present with?
Neurologic deficits or seizures
What symptoms are typically seen in patients with systemic lymphomas?
B symptoms
What is the first-line therapy for hairy-cell leukemia?
Cladribine
Cladribine is associated with a high remission rate as well as durability of remission lasting for many years.
What is the most appropriate management for a cancer pain crisis with evidence of opioid-induced neurotoxicity?
Opioid rotation with dose reduction to account for incomplete cross-tolerance.
What is the correct initial management for a microinvasive squamous-cell carcinoma?
Biopsy of the cervix
What procedure is performed for biopsy of the cervix?
Cervical cold-knife conization
What mutation is identified in 50% to 60% of patients with primary myelofibrosis?
JAK2 V617F mutation
What does a pelvic examination in patients with cervical cancer typically reveal?
A lesion at the transformation zone of the cervix
What should raise suspicion for acral lentiginous melanoma?
A dark pigmented macular lesion under the nail bed with associated periungual pigmentation
What is the only melanoma subtype not associated with extreme sun exposure?
Acral lentiginous melanoma
What is the most likely cause of a sore tongue in a patient with macrocytosis and pancytopenia?
Vitamin B12 deficiency
What should women with LCIS undergo annually?
Diagnostic mammography
What is indicated if the initial laboratory evaluation for MGUS is low risk?
Labs can be repeated in approximately 3 months and then at 6- to 12-month intervals.
What is the most appropriate test for a patient who becomes febrile after receiving a correctly matched unit of packed red cells?
Postreaction direct antiglobulin test to rule out a hemolytic transfusion reaction.
Do current guidelines recommend routine prophylaxis against catheter-related thrombosis in patients with cancer outpatient?
No, current guidelines do not recommend routine prophylaxis.
What may a persistently low platelet count despite repeated platelet transfusions indicate?
Immunologically mediated refractoriness warranting a screening.
What may be indicative of immunologically mediated refractoriness?
Repeated platelet transfusions
Warranting a screening for human leukocyte antigen antibodies.
What should men undergo before starting androgen deprivation therapy?
Dual-energy x-ray absorptiometry
They should also implement lifestyle modifications such as calcium and vitamin D supplementation and increased weight-bearing exercise.
What can be added for TLS to achieve a target urine output?
A loop diuretic
Target urine output of around 150–200 mL/hour for most adults.
What does variable expressivity refer to?
Phenotypic differences
In the degree of presentation of disease; disease severity may vary from mild to severe in different family members.
What is characteristic of chronic liver disease with elevated serum alpha-fetoprotein level?
Imaging findings of HCC
Sufficiently specific and obviate the need for tissue diagnosis.
What is associated with pancytopenia in the presence of splenomegaly?
Monocytopenia
Atypical lymphocytes with hairy projections is characteristic of hairy-cell leukemia.
What indicates a diagnosis of a superior pulmonary sulcus (Pancoast) tumor?
Gradual development of arm weakness and Horner syndrome
In an older patient with a history of cigarette smoking.
What is the most common cancer associated with hereditary nonpolyposis colorectal cancer?
Endometrial cancer
To a lesser extent, cancers of the ovary, stomach, small bowel, pancreas, hepatobiliary tract, brain, and upper uroepithelial tract.
What type of lymphoma is Burkitt lymphoma?
An aggressive non-Hodgkin lymphoma
Associated with c-myc translocation usually in association with Epstein–Barr virus infection.
What is consistent with a diagnosis of cryoglobulinemic vasculitis?
Presence of vasculitis in a patient with lymphoma
Rheumatoid factor positivity and low complement levels.
What is rheumatoid factor positivity and low complement levels consistent with?
A diagnosis of cryoglobulinemic vasculitis.
What are meningiomas?
Diffusely enhancing lesions that occur most commonly in the parasellar regions of the base of the skull or cerebral convexities.
