heme/onc 1 Flashcards
What is the possibility of pure red cell aplasia being reversed sometimes by what procedure
Thymectomy
What should a patient have to evaluate for thymoma?
A chest radiograph.
What is the typical cause of pure red cell aplasia?
It is typically idiopathic but can occur in patients with hematologic malignancies.
Which hematologic malignancy is associated with pure red cell aplasia?
Chronic lymphocytic leukemia.
What viral illnesses can lead to pure red cell aplasia?
Parvovirus, HIV, and hepatitis C.
What medications are associated with pure red cell aplasia?
Phenytoin and trimethoprim–sulfamethoxazole.
What activates factor X and factor IX in the extrinsic pathway of coagulation?
TF-FVIIa complex.
What is a common finding in a patient with iron deficiency anemia?
Severe anemia by the time the MCV has reached the mid- to low 60s.
How does aortic stenosis cause acquired von Willebrand disease?
Increased shear stress at the defective valve leads to unfolding of the von Willebrand factor (VWF) polymer and subsequent proteolytic cleavage by ADAMTS13.
What type of cancer can produce a variety of ectopic hormones?
Small-cell lung cancer.
What hormones can small-cell lung cancer produce?
Adrenocorticotropic hormone and antidiuretic hormone.
What syndrome can develop due to ectopic hormone secretion from small-cell lung cancer?
Paraneoplastic Cushing syndrome and the syndrome of inappropriate diuretic hormone secretion.
What is the most likely diagnosis in a person with HIV showing thrombocytopenia?
Immune thrombocytopenia.
What is the typical timeline for HIT to occur after heparin exposure?
5 to 14 days after exposure.
What are the major criteria for Polycythemia Vera (PV)?
- Elevated hemoglobin level (>16.0 g/dL in women; >16.5 g/dL in men). 2. Elevated hematocrit (>48% in women; >49% in men). 3. Bone-marrow biopsy consistent with PV.
What genetic mutation is associated with Polycythemia Vera?
Presence of the JAK2 V617F mutation.
What is the JAK2 V617F mutation?
The JAK2 V617F mutation is a specific mutation in the JAK2 gene associated with certain blood disorders.
What is a minor criterion for diagnosing certain blood disorders?
A serum erythropoietin level below the normal reference range.
What is important to rule out before further diagnostics in isolated thrombocytopenia?
It is important to rule out pseudothrombocytopenia.
What can cause pseudothrombocytopenia?
EDTA altered platelet autoantibodies resulting in clumping.
What is a common consequence of radiotherapy for head and neck cancers?
Development of hypothyroidism within 5 years.
What is PNH?
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired bone-marrow disorder caused by mutations in the phosphatidylinositol glycan anchor gene.
What INR level is associated with an increased risk for bleeding?
An INR greater than 4.0.
How does the risk for intracranial hemorrhage change with INR levels?
The risk increases approximately twofold for every one-unit increase in INR.
What do guidelines recommend for patients with liver cirrhosis?
Patients should undergo surveillance ultrasound with or without alpha-fetoprotein testing every 6 months.
Who are candidates for treatment with rasburicase?
Patients at high risk for tumor lysis syndrome or with uric acid levels >8 mg/dL despite aggressive volume expansion.
What percentage of GISTs have mutations in the PDGFRA gene?
Up to 10% of GISTs have mutations in the PDGFRA gene.
What are common culprits for drug-induced immune-mediated thrombocytopenia?
Trimethoprim-sulfamethoxazole, quinine, vancomycin, and penicillin.
How can low-risk neutropenia and fever be managed?
With ciprofloxacin and amoxicillin-clavulanate.
What is the next step in a patient with parasthesias and borderline-low vitamin B12 level?
Measurement of serum methylmalonic acid and homocysteine levels.
What should patients with essential thrombocythemia who have risk factors be monitored for?
They should be monitored for complications related to their condition.
What should patients with risk factors for complications (age >60 years, presence of a JAK2 mutation, or a history of bleeding or thrombosis) be treated with?
They should be treated with cytoreductive therapy with hydroxyurea.
What is the best diagnostic test for a patient with a history of prostate cancer who develops microcytic anemia and fatigue with a positive fecal occult-blood test?
The best diagnostic test is colonoscopy.
Where do osteosarcomas generally arise?
Osteosarcomas generally arise in the metaphysis of the bone.
What are essential features of the cancer-associated hypercoagulable state?
Venous thrombophlebitis and arterial thromboembolism.
What does a prolonged activated partial-thromboplastin time that is not corrected by a mixing study suggest?
It suggests the presence of a factor inhibitor, most commonly a factor VIII inhibitor.
What is the best strategy for managing intractable cancer-related pain despite intermittent opioids?
The best strategy is intravenous opioids administered as patient-controlled analgesia.
What is the standard immunosuppressive therapy for aplastic anemia?
Standard immunosuppressive therapy includes antithymocyte globulin and calcineurin inhibition.
What should patients receiving primary therapy for multiple myeloma with skeletal lesions be considered for?
They should be considered candidates for at least 2 years of treatment with an osteoclast inhibitor.
What is the most likely diagnosis in a patient who presents with bone-marrow failure 5 years after receiving chemotherapy and radiotherapy?
The most likely diagnosis is secondary malignancy or treatment-related myelodysplastic syndrome.
What is therapy-related myelodysplastic syndrome associated with?
It is associated with chemotherapy and radiotherapy.
Which virus infection is associated with Burkitt lymphoma?
Epstein-Barr virus infection.
What is thrombotic thrombocytopenic purpura classically associated with?
It is classically associated with microangiopathy, thrombocytopenia, renal insufficiency, mental status changes, and fever.
What is the most common serious adverse event associated with the use of bevacizumab?
Severe hemorrhage.
What are features associated with increased risk for progression of MGUS?
- A serum monoclonal protein level >1.5 g/dL
- Non–immunoglobulin G monoclonal protein
- A markedly abnormal free-light-chain-kappa-to-lambda ratio.
What is the perioperative management for a patient with prolonged partial-thromboplastin time and low factor XII level?
To proceed with surgery without additional treatment.
Do patients with deficiency of factor XII exhibit an increased bleeding diathesis?
No, they do not exhibit an increased bleeding diathesis despite the in vitro partial-thromboplastin time assay being prolonged.
What infections commonly cause cold agglutinin hemolytic anemia?
Typical infections include mycoplasma and Epstein–Barr virus.
Where do osteosarcomas generally arise?
They generally arise in the metaphysis of the bone.
What does a CT scan of radiation pneumonitis typically show?
It shows areas of consolidation in a nonanatomical distribution that does not conform to lobes or bronchopulmonary segments, but rather to the radiation port.
What are the major clinical manifestations of mixed cryoglobulinemic vasculitis?
They include palpable purpura, kidney disease, arthralgia, myalgia, and peripheral neuropathy.
What is the significance of hypocomplementemia in TLS?
It is significant for smaller solid cancers and indolent lymphomas.
What is hypocomplementemia associated with?
Hypocomplementemia is associated with smaller solid cancers and indolent lymphomas.
What cancers are associated with the highest risk?
Aggressive leukemias and cancers with large tumor burdens are associated with the highest risk.
What is the typical duration for pharmacologic prophylaxis after major orthopedic surgery?
Pharmacologic prophylaxis is typically continued for at least 14 days or until the patient regains ambulatory function with full weight-bearing.
What are the most common solid tumors in adolescents and young adults?
Germ-cell tumors are the most common solid tumors in adolescents and young adults.
What are examples of extracranial germ-cell tumors?
Extracranial germ-cell tumors include yolk sac tumors, embryonal carcinomas, choriocarcinomas, and mixed tumors.
What symptoms do germ-cell tumors often manifest with?
Germ-cell tumors often manifest with abdominal pain and distention associated with a palpable pelvic-abdominal mass.
What does Vitamin A deficiency cause?
Vitamin A deficiency causes night blindness but is not associated with anemia.
What is polycythemia vera?
Polycythemia vera is a myeloproliferative neoplasm characterized by increased red-cell mass and elevated hemoglobin.
What are common symptoms of polycythemia vera?
Common symptoms include erythromelalgia (red, hot, painful extremities), pruritus, and a low serum erythropoietin level.
What is Ewing sarcoma most common in?
Ewing sarcoma is most common in children and adolescents.
Is inflammatory breast cancer often detected by imaging modalities?
Inflammatory breast cancer is often not detected by imaging modalities.
What is the first line drug for management of chemotherapy-induced neuropathic pain?
The first line drug for management of chemotherapy-induced neuropathic pain is duloxetine.
What is the most likely diagnosis in a patient with leukocytosis above 100,000 cells per mm3 with absolute basophilia, thrombocytosis, and splenomegaly?
The most likely diagnosis is chronic myeloid leukemia.
What system estimates the risk of progression from asymptomatic to symptomatic multiple myeloma?
The Mayo Clinic 2018 risk stratification system estimates the risk of progression from asymptomatic to symptomatic multiple myeloma.
What are the three risk factors considered in the Mayo Clinic 2018 risk stratification system?
The three risk factors are: M protein >2 g/dL, bone marrow clonal plasma cells >20%, and involved–uninvolved free-light-chain ratio >20.
What is the risk classification for patients with no risk factors for smoldering multiple myeloma?
Patients with no risk factors are considered to be at low risk for smoldering multiple myeloma.
What is the risk classification for patients with one risk factor for smoldering multiple myeloma?
Patients with one risk factor are considered to be at intermediate risk for smoldering multiple myeloma.
What is the risk classification for patients with two or three risk factors for smoldering multiple myeloma?
Patients with two or three risk factors are considered to be at high risk for smoldering multiple myeloma.
What is the association of squamous-cell carcinoma of the oropharynx in non-smokers?
It is most often associated with human papillomavirus infection.
What do guidelines from the American Association for the Study of Liver Diseases recommend for patients with liver cirrhosis?
They recommend undergoing surveillance ultrasound with or without alpha-fetoprotein testing every 6 months.
What condition is myelofibrosis typically associated with?
It is typically associated with splenomegaly and leukoerythroblastosis.
What can iron overload from transfusion-related etiologies lead to?
It can lead to end-organ dysfunction, especially in the liver and myocardium.
What are the most common primary tumors that metastasize to the brain?
Lung cancer, melanoma, renal-cell carcinoma, and breast cancer.
What is Ewing sarcoma typically associated with?
It is typically associated with an onion-skin periosteal reaction.
What is the likely diagnosis in a patient with recurrent epistaxis, telangiectasia, and anemia?
It is hereditary hemorrhagic telangiectasia.
What is Beck triad associated with?
It is associated with pericardial effusion and includes low systolic blood pressure, jugular venous distention, and muffled heart sounds.
What is pancytopenia in association with splenomegaly indicative of?
It can be indicative of B symptoms, monocytopenia, and atypical lymphocytes.
What is characteristic of hairy-cell leukemia?
It is characterized by projections.
What results from hereditary spherocytosis?
It results in diminished compliance of the cell and degradation by the spleen, leading to splenomegaly, anemia, and jaundice.
What is hereditary hemorrhagic telangiectasia?
It is a genetic disorder that leads to abnormal blood vessel formation.
What is the most likely diagnosis in a patient with a monoclonal gammopathy?
Heart failure.
What is renal failure?
A condition where the kidneys fail to filter waste from the blood.
What condition is associated with periorbital purpura?
Amyloid light-chain amyloidosis.
What is the most likely diagnosis in a patient with pancytopenia?
Auer rods.
What is disseminated intravascular coagulation?
A serious condition involving the formation of blood clots throughout the small blood vessels.
What is acute promyelocytic leukemia associated with?
Chromosomal translocation t(15;17).
What does acute radiation proctitis refer to?
Rectal bleeding that occurs during radiation treatment or within 6 weeks.
What are meningiomas?
Tumors that are adjacent to bone and anchored to the dura.
What classic appearance do meningiomas render on imaging?
The classic appearance of the ‘dural tail’.
What is likely to cause tenesmus and significant anemia?
Anal fissure.
Is it true that anal fissure is likely to cause tenesmus and significant anemia?
True.