Hematology Oncology III Flashcards
What is the main cause of death in patients with paroxysmal nocturnal hemoglobinuria?
Thrombosis
e.g. hepatic, portal, or cerebral thrombosis; destroyed platelets release cytoplasmic contents into circulation, inducing thrombosis
What is the mode of inheritance of hemophilia A and B?
X-linked recessive
versus von Willebrand disease, which is autosomal dominant
What is the most appropriate pain management for a patient with advanced prostate cancer with bony metastasis, status-post orchiectomy?
Radiation therapy
bisphosphonates are useful for controlling chronic pain, but radiation is more acute; anti-androgen therapy is not needed in patients that have undergone orchiectomy
What is the most common cause of megaloblastic anemia in chronic alcoholics?
Folate deficiency
conversely, alcohol abuse is the most common nutritional cause of folate deficiency in the United States
What is the most common cause of vitamin B12 deficiency?
Pernicious anemia
often associated with other autoimmune conditions (e.g. thyroid disease, vitiligo)
What is the most common mode of inheritance of hereditary spherocytosis?
Autosomal dominant (75%)
What is the most common side effect associated with recombinant erythropoietin?
Worsening of hypertension (30%)
hypertension is generally mild, but can be severe, leading to end organ damage; other common side effects include headache (15% of patients) and flu-like symptoms (5% of patients)
What is the most common source of symptomatic pulmonary embolism?
Proximal deep leg veins (e.g. femoral, popliteal, iliac)
distal veins (e.g. calf veins) are less likely to embolize
What is the most common underlying etiology of pancytopenia in patients with SLE?
Immune-mediated destruction
What is the most important initial screening test in a Mediterranean female receiving preconception counseling due to possible thalassemia?
CBC
hemoglobin electrophoresis would be obtained only if the initial CBC is abnormal with low MCV and proof of normal iron status
What is the most likely underlying etiology of DVT in a young, Caucasian patient that presents with leg swelling, positive D-dimer, and shortness of breath?
Factor V Leiden
mutated factor V gene is unresponsive to activated protein C (an anticoagulant); most commonly inherited hypercoagulable disorder in the Caucasian population (mutated prothrombin is the second most common)
What is the next step in management for a patient with a year history of fatigue, weakness, and difficulty remembering things? Laboratory examination reveals macrocytic anemia and basophilic stippling.
Measure serum B12 and folate levels
basophilic stippling is a non-specific finding; the initial evaluation of macrocytic anemia should involve measuring folate/B12 levels
What is the next step in management for a patient with moderate-high probability of DVT (modified Wells > 2)?
Compression ultrasonography
may use D-dimer to rule-out DVT in patients with low pretest probability; diagnosis of DVT should be confirmed before anticoagulation is started if patients have no evidence of PE
What is the next step in management for an older patient with a significant smoking history that presents with painful, pruritic red streaks on the arm, with a similar episode two weeks prior on the chest that self-resolved, and mild epigastric pain?
Abdominal CT
this patient has migratory superficial thrombophlebitis (Trousseau’s syndrome), which is associated with occult visceral malignancy (pancreas, stomach, lung, prostate)
What is the pathophysiologic mechanism underlying polycythemia in patients with obstructive sleep apnea?
Increased EPO production (secondary to hypoxemia)
What is the recommended management for a patient with a DVT and contraindications to anticoagulation (e.g. warfarin)?
IVC filter
IVC filters are also useful for patients with recurrent proximal DVT who have failed anticoagulation
What is the recommended pharmacologic treatment for patients with cancer-related anorexia/cachexia syndrome (CACS)?
Progesterone analogs (preferred) or corticosteroids
e.g. megestrol acetate; synthetic cannibinoids are useful for HIV cachexia, but not as useful for CACS
What is the recommended pharmacologic treatment for patients with chemotherapy-induced nausea and vomiting?
5-HT3 (serotonin) receptor antagonists
e.g. ondansetron; D2 antagonists (e.g. metoclopramide, prochlorperazine) are considered second or third-line treatment options
What is the recommended RBC pre-treatment for a patient who will receive a transfusion of pRBCs and has a history of febrile non-hemolytic transfusion reaction?
Leukoreduction
also useful for reducing risk of CMV transmission and HLA alloimmunization
What is the recommended treatment for a patient initiated on phenytoin therapy for seizures that develops anemia?
Folic acid
phenytoin, and other drugs (e.g. TMP, SMX), impair the absorption or inhibit the physiologic effects of folate
What is the recommended treatment for a patient on warfarin that presents with an acute DVT with a subtherapeutic INR due to improper dieting/non-compliance?
Oral factor Xa inhibitors (e.g. rivaroxaban)
oral factor Xa inhibitors have similar efficacy to warfarin but have more rapid onset and do not require bridging therapy or laboratory monitoring
What is the recommended treatment for a patient with suspected sideroblastic anemia after initiating RIPE therapy for tuberculosis?
Pyridoxine (vitamin B6)
due to isoniazid-induced sideroblastic anemia, secondary to vitamin B6 deficiency
What is the recommended treatment for immune thrombocytopenic purpura in adults with platelet counts < 30,000/uL or bleeding?
IVIG or corticosteroids
What is the recommended treatment for patients that develop heparin-induced thrombocytopenia?
discontinue heparin, begin alternative anticoagulant (e.g. direct thrombin inhibitor, fondaparinux)
What is the recommended treatment for polycythemia vera?
Phlebotomy +/- hydroxyurea
What is the recommended treatment for thrombotic thrombocytopenic purpura (TTP)?
Emergent plasma exchange +/- corticosteroids
What is the recommended treatment to reverse the effects of heparin?
Protamine sulfate
What is the underlying pathophysiology leading to sepsis with gram-positive cocci in a patient with a history of “LUQ abdominal surgery”?
Impaired antibody-mediated phagocytosis
the patient likely had a splenectomy, making him/her more susceptible to encapsulated organisms (e.g. S. pneumoniae, H. influenza, N. meningitidis)
What is the usually the underlying etiology behind acute hemolytic transfusion reactions?
ABO incompatibility
What peripheral blood smear finding is associated with chronic lymphocytic leukemia (CLL)?
Smudge cells
What peripheral blood smear finding is associated with multiple myeloma?
Rouleaux formation
secondary to elevated serum protein
What screening tests would be useful for evaluating an elderly patient with chronic arm pain, anemia, and back pain (X-ray below)?
serum/urine protein electrophoresis or free light chain analysis
What two viruses should be tested for in an asymptomatic patient with idiopathic thrombocytopenic purpura?
HIV and hepatitis C
thrombocytopenia may be the initial presentation of HIV infection in 5-10% of patients
What type of anemia is characterized by the following laboratory values?
MCV: low
Serum iron: high
Ferritin: high
What type of anemia is characterized by the following laboratory values?
MCV: low
Serum iron: high
Ferritin: high
Sideroblastic anemia
most commonly due to pyridoxine-dependent impairment in the early steps of protoporphyrin synthesis; may see ringed sideroblasts on bone marrow biopsy
What type of anemia is characterized the following laboratory values?
MCV: normal
Serum iron: low
Ferritin: high
What type of anemia is characterized the following laboratory values?
MCV: normal
Serum iron: low
Ferritin: high
Anemia of chronic disease
commonly occurs in the setting of chronic inflammation; treating the underlying cause often improves the anemia
What underlying cause of DVT must be ruled out in an older patient with their first episode of DVT and no history of immobilization, surgery, or provocative medications?
Malignancy
e.g. age-appropriate cancer screening (colonsocopy, mammogram) and CXR; more detailed testing may be indicated depending on the patient’s symptoms
What vitamins (3) are typically administered to patients with hypercoagulability secondary to increased homocysteine levels?
vitamin B6 (pyridoxine), B9 (folate), and B12 (cobalamin)
Why do patients with multiple myeloma have an increased risk of infection?
Hypogammaglobulinemia
neoplastic infiltration of the bone marrow impairs normal lymphocyte population, resulting in ineffective antibody production
