HemeOnc Flashcards
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Immune thrombocytopenic purpura can be idiopathic, triggered by medications, or associated with other disorders, such as systemic lupus erythematosus, chronic lymphocytic leukemia, lymphoma, HIV, hepatitis C, or Helicobacter pylori infection
no diagnostic test is available for immune-mediated thrombocytopenia
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Patients who present with symptoms of mucocutaneous bleeding and a normal platelet count should be evaluated for acquired platelet dysfunction using the Platelet Function Analyzer-100.
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Patients with a Pulmonary Embolism Severity Index score of less than 65 are at low risk of death and may be managed in the outpatient setting with a non–vitamin K antagonist oral anticoagulant, such as apixaban or rivaroxaban.
Dabigatran and edoxaban require bridging with parenteral anticoagulation, whereas apixaban and rivaroxaban can be used as monotherapy in the treatment of VTE.
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Dabigatran and edoxaban require bridging with parenteral anticoagulation, whereas apixaban and rivaroxaban can be used as monotherapy in the treatment of VTE.
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Inflammatory anemia is usually mild to moderate in severity, characterized by low serum iron levels and total iron-binding capacity and elevated serum ferritin level, and usually requires no specific therapy.
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In patients with myelodysplastic syndrome requiring frequent transfusions, supplemental treatments to help decrease transfusion requirements, such as lenalidomide, should be used to improve quality of life and decrease transfusion-associated iron overload and alloimmunization.
Treatment of MDS has two goals. The first goal is to relieve transfusion dependence; the second is to prevent transformation to acute myeloid leukemia (AML).
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vHypereosinophilic syndrome is characterized by moderate eosinophilia and end-organ damage commonly involving the skin, lungs, gastrointestinal tract, and heart; secondary causes of eosinophilia should be excluded.
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Fat pad biopsy is performed during evaluation for systemic amyloidosis. Amyloidosis involving the heart can cause restrictive heart disease.
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The most appropriate management for this patient is clinical observation. He has hereditary spherocytosis (HS) characterized by a mild lifelong anemia in association with symptomatic cholelithiasis at an early age. The presence of spherocytes is supported by an elevated mean corpuscular hemoglobin concentration frequently seen in this disorder. HS is caused by mutations in several scaffolding proteins that make these cells less distensible and more susceptible to osmotic stress and hemolysis. Patients with this disorder may have mild anemia, an elevated reticulocyte response, and few or no symptoms. The development of pigmented gallstones resulting from excess bilirubin production may result in symptomatic cholelithiasis. Symptoms of anemia may arise when the bone marrow is suppressed, most commonly by an acute infection. In this situation, the reticulocyte count falls, and the patient rapidly develops symptomatic anemia. Parvovirus is most classically linked with bone marrow suppression, but many other viral and infectious agents can have a similar effect. The bone marrow suppression following acute infections is self-limited, but some patients may become symptomatic to the point of requiring blood transfusion.
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Prednisone would be indicated to treat warm antibody autoimmune hemolytic anemia (WAIHA). Spherocytes are also seen in patients with WAIHA, but the direct antiglobulin test result would be positive. WAIHA would not explain the lifelong anemia in this patient.
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Low-molecular-weight heparin is the anticoagulant of choice for patients with active cancer and a venous thromboembolism.
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In the presence of normal Doppler studies of the lower extremities, ventilation-perfusion lung scanning is the initial lung imaging study to evaluate for pulmonary embolism in pregnant patients; D-dimer testing has no diagnostic role.
CT angiography, the gold standard in the diagnosis of PE in most patients, should not be the initial study in pregnant patients because of radiation exposure to both the mother and the fetus.
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Patients scheduled for elective surgery who have anemia should be evaluated for iron deficiency; preoperative management of iron deficiency anemia includes oral iron replacement and evaluation to determine the source of blood loss.
Preoperative anemia is associated with increased perioperative mortality in patients with cardiovascular disease; it is also a significant predictor for perioperative blood transfusion, which itself is associated with postoperative morbidity.
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Four-factor prothrombin complex concentrate should be used to reverse the effects of warfarin anticoagulation in patients experiencing severe bleeding and those requiring urgent surgery.
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Cryoprecipitate would be indicated to treat severe hypofibrinogenemia, usually arising as a consequence of disseminated intravascular coagulation (DIC) or severe liver disease
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In approximately 10% of patients in whom an unprovoked venous thromboembolism is diagnosed, cancer will be found within 1 year, so an age-appropriate screening test should be performed.
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pancytopenia, immature leukocytes with morphologic features consistent with promyelocytes, and laboratory features of disseminated intravascular coagulation (DIC), all of which are consistent with acute promyelocytic leukemia (APML).
APML looks like DIC with a low WBC
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In patients with IgG monoclonal gammopathy of undetermined significance, an M spike of less than 1.5 g/dL, and normal findings on serum free light chain assay and urine protein electrophoresis, the risk of progression is low, so extensive evaluation is not recommended.
follow-up in 6 months to 1 year with repeat serum protein electrophoresis, hemoglobin and calcium levels, and kidney function is appropriate
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In patients with multiple myeloma, hypogammaglobulinemia, and recurrent infections, intravenous immune globulin should be given to provide passive immunity against causative organisms.
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A positive direct antiglobulin (Coombs) test will confirm a delayed hemolytic transfusion reaction, a diagnosis that should be considered in a patient with low-grade fever and features of hemolytic anemia after recent transfusion.
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Cryoprecipitate is the treatment of choice for patients with bleeding and hypofibrinogenemia secondary to disseminated intravascular coagulation.
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Parvovirus B19 infection preferentially affects erythrocyte precursors in the bone marrow, causing transient pure red cell aplasia in patients with sickle cell anemia.
Parvo affects only the red cells
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Patients with essential thrombocythemia who are older than 60 years or who have had previous thromboembolic complications should be treated with aspirin and hydroxyurea.
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The most appropriate management for this patient is to discontinue dapsone. He most likely has glucose-6-phosphate dehydrogenase (G6PD) deficiency as a result of his dapsone therapy. He developed an acute hemolytic anemia, indicated by an acute reduction in the hemoglobin and haptoglobin levels and reticulocytosis, and the bite cells seen on the peripheral blood smear also suggest G6PD deficiency. A drug reaction should always be suspected in such settings
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Diagnosis of amyloidosis requires biopsy of the affected organ and demonstration of characteristic apple-green birefringence with Congo red staining; fat pad biopsy is sometimes performed because it is less invasive.
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Patients with inflammatory bowel disease have difficulty absorbing oral iron to balance their increased iron loss from bleeding, so parenteral iron is an appropriate alternative for restoring iron stores.
severe iron deficiency and inflammatory bowel disease, which impairs iron absorption in the duodenum and proximal jejunum and commonly leads to increased gastrointestinal blood loss.
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Waldenström macroglobulinemia is an indolent B-cell lymphoma with clonal lymphoplasmacytic infiltration of the bone marrow that secretes IgM in the blood, and a bone marrow biopsy will confirm the diagnosis.
For patients suspected of having a lymphoma, excisional biopsy of a lymph node is preferred to needle biopsy because it provides better architectural detail for distinguishing malignant from benign causes and for classifying the nature of the malignancy.
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Atypical presentations of rheumatoid arthritis and osteoarthritis, particularly with hook-like osteophytes of the second and third metacarpophalangeal joints, suggest the possibility of hemochromatosis; evaluating the transferrin saturation and serum ferritin level should be considered.
atients with hemochromatosis have rheumatic symptoms that typically involve the small joints of the hand, especially the second and third metacarpophalangeal (MCP) joints associated with characteristic radiographic findings, including hook-like osteophytes.
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Thrombolysis should be considered as the initial therapy for patients with iliofemoral deep venous thrombosis with acute limb ischemia.
Rivaroxaban, a non–vitamin K antagonist oral anticoagulant, is an accepted monotherapy for DVT (heparin not required).
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In patients with severe symptomatic autoimmune hemolytic anemia, the autoantibody typically reacts against all erythrocytes, and a completely crossmatch-compatible unit may be impossible to find; these patients should receive ABO and Rh-matched blood even if it is not crossmatch compatible.
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The International Myeloma Working Group recommends that all patients with smoldering multiple myeloma undergo whole body MRI to assess for lytic lesions.
The International Myeloma Working Group recommends that all patients with smoldering MM undergo whole body MRI (or spine and pelvic MRI if whole body MRI is not available). Whole body MRI is considered the gold standard for imaging of the axial skeleton, for the evaluation of painful lesions, and for distinguishing benign versus malignant osteoporotic vertebral fractures.
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In patients with INR elevation and bleeding associated with warfarin administration, urgent reversal of anticoagulation should be accomplished using vitamin K and prothrombin complex concentrates.
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Secondary iron overload from chronic transfusions can be effectively treated with oral chelation agents, such as deferasirox.
In most cases, secondary iron overload occurs in patients with severe anemia who require chronic transfusion therapy. Because iron excretion has no regulated mechanism, multiple transfusions (for anemias not stemming from blood loss or iron deficiency) lead to iron overload, with subsequent secondary organ damage
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Patients with symptomatic anemia of chronic kidney disease may be treated with erythropoiesis-stimulating agents to reduce transfusion requirements with a target hemoglobin level of 11 to 12 g/dL (110-120 g/L) to avoid increased risk of adverse cardiovascular events.
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Minor bleeding in patients taking a non–vitamin K antagonist oral anticoagulant can be managed by discontinuation of the anticoagulant alone without additional therapy.
Fresh frozen plasma has been used in patients experiencing major bleeding while taking a non–vitamin K antagonist oral anticoagulant, but it is not typically adequate monotherapy in patients experiencing severe bleeding; it is often combined with four-factor prothrombin complex concentrate (4f-PCC). However, neither fresh frozen plasma nor 4f-PCC would be necessary in this patient because she is hemodynamically stable,
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persons with sickle cell disease (SCD) should not receive transfusions unless they have significant symptoms or signs of end-organ failure from their anemia or are preparing for surgery.
Simple transfusion to achieve a hemoglobin level of 10 g/dL (100 g/L) in patients having low- to moderate-risk surgery reduces surgical complications equivalent to exchange transfusion with less risk and cost.
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Protein C or S deficiency is associated with warfarin-associated skin necrosis.
A family history of pulmonary embolism suggests the possibility of inherited protein C deficiency.
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Patients identified as low risk and meeting the Pulmonary Embolism Rule-Out Criteria do not require D-dimer testing to eliminate the need for further diagnostic imaging.