Hematology & Oncology Flashcards
Immune thrombocytopenic purpura can be idiopathic, triggered by medications, or associated with other disorders, such as systemic lupus erythematosus, chronic lymphocytic leukemia, lymphoma, hepatitis C, or Helicobacter pylori infection &?
HIV
Patients who present with symptoms of mucocutaneous bleeding* and a *normal platelet count* should be evaluated for *acquired platelet dysfunction using?
Platelet Function Analyzer-100
Note: this test replaced the bleeding time test. Also, SSRIs & ginkgo biloba can increase platelet dysfunction
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
a non–vitamin K antagonist oral anticoagulant, such as apixaban or rivaroxaban.
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 treatment
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 & alloimmunization. Second-line treatments: azacitidine and decitabine
Note: 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).
Hypereosinophilic syndrome is characterized by moderate eosinophilia* and end-organ damage commonly involving the skin, lungs, gastrointestinal tract, and heart (e.g. *restrictive cardiomyopathy); what is the next step in management?
Secondary causes of eosinophilia should be excluded.
Note: causes of eosinophilia can be recalled using the mnemonic CHINA (Collagen vascular disease, Helminthic infection, Idiopathic, Neoplasia, Allergy/Atopy/Asthma).
Acute viral infections may trigger a transient aplastic crisis in patients with hereditary spherocytosis & should be managed with?
Observation
HS- characterized by a mild lifelong anemia in assoc. w/ 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 & more susceptible to osmotic stress & hemolysis. Patients with this disorder may have mild anemia, an elevated reticulocyte response, & few or no symptoms. The development of pigmented gallstones resulting from excess bilirubin production may result in symptomatic cholelithiasis.
Treat a patient with malignancy-associated pulmonary embolism with?
Low-molecular-weight heparin is the anticoagulant of choice for patients with active cancer and a venous thromboembolism.
Note: NOAC have been shown to be equally effective
In the presence of normal Doppler studies of the lower extremities, diagnose pulmonary embolism in a pregnant patient?
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.
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.
Should be used to reverse the effects of warfarin anticoagulation in patients experiencing severe bleeding and those requiring urgent surgery?
Four-factor prothrombin complex concentrate
4f-PCC should be avoided in patients with a history of heparin-induced thrombocytopenia because it contains residual heparin.
Idarucizumab is a monoclonal antibody that binds dabigatran & causes a rapid reduction in available in the body for up to 24 hours.
In approximately 10% of patients in whom an unprovoked venous thromboembolism is diagnosed, cancer will be found within 1 year, so ?
Age-appropriate screening test should be performed
Pancytopenia*, *immature leukocytes* with morphologic features consistent with promyelocytes, and *DIC, all of which are consistent with?
Acute promyelocytic leukemia (APML)
A bone marrow aspirate & biopsy confirm the diagnosis. Treatment is with all-trans retinoic acid, which targets the underlying defect in cellular differentiation.
In patients with IgG monoclonal gammopathy of undetermined significance (MGUS), 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, repeat labs in 6 months
In patients with multiple myeloma, hypogammaglobulinemia, and recurrent infections, treatment should include?
Intravenous immune globulin (IVIG), should be given to provide passive immunity against causative organisms.
A delayed hemolytic transfusion reaction, should be considered in a patient with low-grade fever and features of hemolytic anemia after recent transfusion (7-14 days). Diagnosis is confirmed using a?
Sxs: tea-colored urine, anemia, hyperbilirubinemia, scleral icterus
Direct antiglobulin (Coombs) test
Note: Flow cytometry use for the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), an acquired clonal stem cell disorder that should be considered in patients presenting with hemolytic anemia, pancytopenia, or unprovoked atypical thrombosis.
What is the treatment of choice for patients with bleeding and hypofibrinogenemia secondary to disseminated intravascular coagulation
Cryoprecipitate
This infection preferentially affects erythrocyte precursors in the bone marrow, causing transient pure red cell aplasia* in patients with *sickle cell anemia?
Parvovirus B19 infection
Note: Aplastic anemia (AA) is a condition characterized by pancytopenia with associated neutropenia, anemia, and thrombocytopenia and a severely hypocellular bone marrow.
Myelodysplastic syndrome (MDS) ranges in severity from an asymptomatic disease characterized by mild normocytic or macrocytic anemia to a transfusion-dependent anemia. MDS increases with age. Abnormal erythrocyte forms with basophilic stippling or Howell-Jolly bodies and dysplastic neutrophils with decreased nuclear segmentation and granulation may be present.
Patients with essential thrombocythemia who are older than 60 years or who have had previous thromboembolic complications should be treated with:
Aspirin and hydroxyurea
Typically appears as an isolated, mild neutropenia (1000-1500/µL [1-1.5 × 109/L]) found on routine testing in asymptomatic Black persons or certain other ethnic groups
Benign ethnic neutropenia
Acute hemolytic anemia, indicated by an acute reduction in the hemoglobin and haptoglobin levels & reticulocytosis, & the bite cells seen on the peripheral blood smear also suggest G6PD deficiency. A drug reaction should always be suspected in such settings?
Discontinue Dapsone, which is one of the most commonly encountered drugs leading to G6PD-mediated hemolysis. G6PD deficiency is X linked, so it is much more common in men, especially in those of African descent.
Normocytic anemia with a low reticulocyte count and normal erythrocyte morphology* in a patient with *underlying chronic kidney disease is usually caused by
Erythropoietin deficiency and will respond to therapy with an erythropoiesis-stimulating agent; however, normalization of the blood count is not advised.
Note: Inflammatory anemia, including a low TIBC & iron level & normal or elevated ferritin level. Myelodysplastic syndrome often have pancytopenia.
The finding of an IgG λ monoclonal gammopathy can also be associated with amyloidosis. Diagnosis 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
Treat iron deficiency due to malabsorption from inflammatory bowel disease with?
Parenteral iron is an appropriate alternative for restoring iron stores
Waldenström macroglobulinemia is an indolent B-cell lymphoma with clonal lymphoplasmacytic infiltration of the bone marrow that secretes IgM in the blood. How is the diagnosis confirmed?
Bone marrow biopsy
Atypical presentations of rheumatoid arthritis and osteoarthritis, particularly with hook-like osteophytes* of the second and third metacarpophalangeal joints, suggest the possibility of *hemochromatosis; what should be evaluated next?
Transferrin saturation and serum ferritin level should be considered.
This should be considered as the initial therapy for patients with iliofemoral deep venous thrombosis with acute limb ischemia.
Thrombolysis with Alteplase
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
Immediately following hematopoietic stem cell transplantation, patients are at increased risk of?
Infection because of compromised function of lymphocytes and neutrophils
The International Myeloma Working Group recommends that all patients with smoldering multiple myeloma, should undergo?
Serum protein electrophoresis and immunofixation show an IgA spike of 3.5 g/dL. Skeletal survey findings are negative.
Whole body MRI to assess for lytic lesions
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
Secondary iron overload (as seen in a MDS patient with ferritin > 1000) from chronic transfusions can be effectively treated with oral chelation agents, such as
Deferasirox
Patients with symptomatic anemia of chronic kidney disease may be treated with erythropoiesis-stimulating agents e.g. Darbepoetin 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.
Minor bleeding in patients taking a non–vitamin K antagonist oral anticoagulant (e.g Dabigatran) can be managed by:
Discontinuation of the anticoagulant alone without additional therapy
Manage preoperative transfusion in sickle cell anemia?
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.
Is associated with warfarin-associated skin necrosis?
Protein C or S deficiency
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. No further testing
Are effective for initial treatment of chronic myeloid leukemia in the chronic phase?
Tyrosine kinase inhibitors such as imatinib
Paroxysmal nocturnal hemoglobinuria (PNH)* presents with *pancytopenia* with significant intravascular hemolysis & *hemoglobinuria.* Patients with PNH commonly note *fatigue & nonspecific abdominal pain, which worsens during times of increased hemolysis. What is the next step?
Flow cytometry results, which can detect CD55 & CD59 deficiency on the surface of peripheral erythrocytes or leukocytes
Patients with coagulopathy of liver disease and low fibrinogen levels who are experiencing bleeding should receive immediate
Cryoprecipitate transfusion
In patients with cancer who are hospitalized, prophylactic anticoagulation with
Low-molecular-weight heparin should be provided to reduce the risk of venous thromboembolism
Patients with sickle cell disease and uncomplicated pregnancy should be
closely monitored and treatment withheld until symptoms appear or some measurable parameter changes
Presents with chronic microcytic anemia with hemoglobin levels of approximately 10 g/dL (100 g/L) and a normal hemoglobin electrophoresis pattern.
α-thalassemia trait
In β-thalassemia, the excess α chains link with δ and γ chains to produce increased amounts of hemoglobin A2 and hemoglobin F, respectively. Patients with thalassemia should receive supplemental folate but should not receive iron supplementation; they are not iron deficient & they have an increased ability to absorb iron, which can lead to iron overload. Genetic counseling may be indicated in reproductive planning.
Patients with asymptomatic INR elevation between 4.5 and 10 are managed
By simply withholding warfarin
Pre-eclampsia, HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelets) syndrome, and thrombotic thrombocytopenic purpura–hemolytic uremic syndrome can all present with microangiopathic hemolytic anemia and thrombocytopenia during pregnancy; pre-eclampsia is defined by hypertension, edema, and proteinuria after the 20th week of gestation.
Manage pre-eclampsia in a pregnant woman with thrombocytopenia?
Emergent delivery
A history of mucosal or endometrial bleeding, normal prothrombin, activated partial thromboplastin times, prolonged Platelet Function Analyzer-100 result and normal platelet count with evidence of a qualitative platelet defect suggest the diagnosis of
von Willebrand disease
Typically have lifelong mild hemolytic anemia with microcytosis and detectable hemoglobin A on electrophoresis; the amount of hemoglobin A is inversely related to the severity of the symptoms and risk of complications
Hemoglobin S β-thalassemia
Note: Patients with hemoglobin SS disease (sickle cell anemia) have greater than 90% HbS and no HbA.
Manage acute upper gastrointestinal bleeding with a restrictive transfusion strategy.
A hemoglobin transfusion threshold of less than 7 g/dL (70 g/L) for hemodynamically stable patients is associated with less blood use and lower mortality compared with a more liberal hemoglobin transfusion threshold of 9 g/dL (90 g/L).
Prevent complications of sickle cell disease with?
Hydroxyurea therapy has been shown to decrease vaso-occlusive episodes and acute chest syndrome, to decrease transfusion requirements and hospitalizations, and to prolong overall survival
Diagnose polycythemia vera?
Presents with low erythropoietin level and splenomegaly
JAK2 V617F mutation is present in 97% of patients with polycythemia vera, so testing should be performed in patients in whom the disease is suspected.
In a patient with thrombocytopenia & hemolytic anemia establishes a presumptive diagnosis of thrombotic thrombocytopenic purpura, how is the diagnosis confirm?
A peripheral blood smear showing schistocytes, confirms the need to initiate early, life-saving therapy.
Survivors of acute lymphoblastic leukemia are at increased risk for the metabolic syndrome, including high BMI, truncal obesity, dyslipidemia, insulin resistance, and hypertension. Screen for hyperlipidemia and diabetes in adult survivors of pediatric leukemia using?
Lipid profile and fasting blood glucose
Include respiratory distress within 6 hours of transfusion, positive fluid balance, elevated central venous pressure, elevated B-type natriuretic peptide, and radiographic findings of pulmonary edema, what’s the diagnosis?
Transfusion-associated circulatory overload
Note: Transfusion-related acute lung injury (TRALI) is the leading cause of transfusion-related mortality. Characterized by the development of acute lung injury within 6 hours of transfusion of erythrocytes, platelets, or fresh frozen plasma. Signs and symptoms escalate quickly and include dyspnea, hypoxia, fever*, chills, and *hypotension*. The chest radiograph shows *diffuse bilateral pulmonary infiltrates.
Patients with sickle cell disease and acute chest syndrome (defined clinically by fever, respiratory findings that include tachypnea & hypoxia, & evolving infiltrates on CXR), with respiratory distress requiring mechanical ventilation should receive
Emergent exchange transfusion
Note: Mild episodes require no transfusion, moderate episodes are managed with simple or exchange transfusion, & severe episodes require exchange transfusion. The target hemoglobin level is 10 g/dL (100 g/L).
Patients taking Dabigatran and requiring urgent surgery should be given?
Idarucizumab, a monoclonal antibody fragment that binds free and thrombin-bound dabigatran and neutralizes its activity.
This deficiency should be suspected in patients with macrocytic anemia, malnutrition, and alcohol dependence.
Folate deficiency
Note: Unlike folate, cobalamin is effectively stored, & dietary inadequacy is not a common cause of deficiency. Alcohol dependence is not a risk factor for cobalamin deficiency.
Patients with hypercalcemia, renal failure, anemia, and bone disease should have further testing with?
Serum protein electrophoresis and serum free light chain assay to evaluate for multiple myeloma
Patients with pancytopenia, macrocytic erythrocytes, hypersegmented neutrophils, and findings consistent with intramedullary hemolysis should have?
Vitamin B12 & folate levels assessed to determine the cause of megaloblastic anemia
Methylmalonic acid and total homocysteine levels are helpful in differentiating cobalamin deficiency (both levels are elevated) from folate deficiency (elevated homocysteine level but normal methylmalonic acid level).
High sheer force seen in some patients with prosthetic heart valves, abnormal native valves, and left ventricular assist device placement may cause?
An acquired von Willebrand disease with clinical bleeding
Can help confirm the diagnosis of myelodysplastic syndrome in patients with macrocytic anemia and other cytopenias and a peripheral blood smear showing dysplastic, hypogranular neutrophils and nucleated erythrocytes?
Bone marrow biopsy
Which of the following transfusion-transmitted diseases is the most likely diagnosis?
Babesia microti is a transfusion-transmissible pathogen that can be responsible for malaria-like symptoms, hemolytic anemia, thrombocytopenia, and abnormal liver chemistry study results.
