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ABIM - Practice Exam

Practice Exam #2

QuestionAnswer
Pregnant 22-year-old has a left colon flare of her Crohn dz. she responded to prednisone in the past, but is not on maintenance therapy. How to treat her? Certolizumab, a TNF alpha inhibitor. It is pegylated, so can't cross placental barrier.
How to treat a patient with ileal colonic Crohn's disease Controlled ileal release budesonide
In a non-pregnant patient, what can you treat Crohn's disease with? Methotrexate
23-year-old sexually active patient with one week history of fevers, malaise, aches of her elbows, left knee, wrist, hands, ankles – worse with movement. Also with transient pustular skin lesions. No urinary symptoms or genital discharge. How to dx? Do a cervical culture. She has disseminated Neisseria gonorrhea. Can also do cultures of the throat, skin, rectum.
22-year-old woman with fatigue, weight loss over two months. Has decreased appetite, unintentional weight loss, nausea and vomiting. Family history of hypothyroidism and diabetes type I. Serum cortisol level at 8 AM is 2. What is the next step? Give her hydrocortisone and fludrocortisone since she has primary adrenal failure. Adrenal insufficiency is diagnosed by an early-morning serum cortisol level <3 in the setting of signs and symptoms of cortisol deficiency.
82-year-old patient with giant cell arteritis, confirmed by temporal artery biopsy. What is she at increased risk for and how to treat? When is this risk the highest? She is increased risk for cardiovascular events (myocardial infarction) and strokes. Treat with daily low-dose aspirin. Highest risk in the first year after diagnosis.
Patients with giant cell arteritis should have what type of imaging? Patients with giant cell arteritis are at risk for thoracic aortic aneurysms and should have baseline chest x-ray as well as annual chest x-ray for 10 years.
62-year-old man status post myocardial infarction with placement of drug-eluting stent. His medications are aspirin, open the grill, metoprolol, lisinopril, atorvastatin. LDL is 69 and total cholesterol is 152. What to add? Ezetimibe, which, whenUsed in combination with statin therapy, significantly reduces the risk of future cardiovascular events in patients with prior ACS.
production of an IgM κ or λ M protein Waldenström macroglobulinemia is an indolent B-cell non-Hodgkin lymphoma
neoplastic infiltrate of clonal lymphocytes, plasmacytoid lymphocytes, plasma cells, and immunoblasts comprising 10% or more of the bone marrow, M protein level of 3 g/dL or more and the absence of disease-related signs, symptoms, or organ dysfunction Smoldering (asymptomatic) Waldenström macroglobulinemia
IgM M protein level of 3 g/dL or more and the absence of disease-related signs, symptoms, or organ dysfunction Smoldering (asymptomatic) Waldenström macroglobulinemia
≥10% bone marrow clonal or lymphoid cells or biopsy evidence of a bony or extramedullary plasmacytoma, M protein present Multiple myeloma requiring therapy
patients with an IgG gammopathy measuring less than 1.5 g/dL, a normal serum FLC ratio, and no evidence of disease-specific end-organ damage - What to do? Defer testing
the most sensitive test for detecting monoclonal FLC gammopathies serum free light chain (FLC) assay can detect monoclonal FLCs before they are detectable by urine protein electrophoresis
MGUS defined as what M protein level less than 3 g/dL (or less than 500 mg/24 hr of urinary monoclonal free light chains), clonal plasma cells comprising less than 10% of the bone marrow cellularity, and the absence of plasma cell dyscrasia–related signs or symptoms
non-IgM and light-chain MGUS are more likely to develop... multiple myeloma
IgM MGUS are more likely to develop ... Waldenström macroglobulinemia or other B-cell non-Hodgkin lymphoma
What determines the likelihood of Non-IgM MGUS, IgM MGUS progressing to multiple myeloma presence of an IgA or IgM gammopathy, an M protein level of 1.5 g/dL or more, and an abnormal serum FLC ratio are predictive of progression to multiple myeloma or other PCD in non–light-chain MGUS
How often to monitor MGUS MGUS are reassessed 6 months after initial diagnosis, and, if stable, yearly thereafter
Patients with IgG MGUS, an M protein level less than 1.5 g/dL, and a normal serum FLC ratio may undergo follow-up ... once every 2 to 3 years
Patients with MGUS are at increased risk of.... What study should they get? osteoporosis and associated skeletal complications, most notably vertebral body compression fractures (hazard ratio 2.37) and should be considered for bone mineral density testing.
Smoldering myeloma is characterized by .... an M protein level of 3 g/dL or more (or ≥500 mg/24 hr of urinary monoclonal FLCs) or clonal plasma cells comprising 10% or more of the marrow cellularity and no evidence of myeloma-related signs or symptoms requiring therapy
Symptomatic myeloma is defined by .... presence of serum M protein, bone marrow plasma clonal cells of 10% or greater, and end-organ damage (hypercalcemia, kidney injury, anemia, bone disease).
Myeloma-related Signs and Symptoms HyperCalcemia>11 mg/dL Renal failure. creatinine >2 mg/dL Anemia. Hemoglobin <10 g/dL (100 g/L) Bone disease. ≥1 lytic bone lesions
Myeloma-defining Biomarkers ≥60% clonal plasma cells on bone marrow examination Involved: uninvolved serum FLC ratio ≥100 ≥1 focal lesion on MRI
Waldenstrom macroglobulinemia related Signs and Symptoms Systemic: Fatigue, B symptoms (fevers, night sweats, weight loss), neuropathy, hyperviscosity; Physical examination findings: Symptomatic lymphadenopathy or hepatosplenomegaly; Laboratory findings: Cytopenias (anemia, thrombocytopenia)
rouleaux formation Multiple Myeloma
How can you tell the pt with smoldering multiple myeloma is about to progress to symptomatic? markers of imminent progression include plasma cells comprising 60% or more of the bone marrow cellularity, more than one focal bone lesion on MRI, or a serum FLC ratio of 0.01 or less or 100 or more; should be considered for early treatment
How to treat multiple myeloma autologous hematopoietic stem cell transplantation with high-dose melphalan after initial induction therapy is associated with improved progression-free and, in some studies, overall survival.
Waldenström macroglobulinemia and may include headache, blurred vision, hearing loss, tinnitus, dizziness, altered mental status, and nasal and oropharyngeal bleeding. What to do for these patients? Symptoms attributable to hyperviscosity, a medical emergency requiring immediate institution of plasmapheresis, are seen in 31% of patients with Waldenström macroglobulinemia
Fundiscopic eval with dilated retinal veins, papilledema, and flame hemorrhages in Waldenstrom Hyperviscosity - emergency plasmapheresis
define Macroglobulinemia paraproteins (usually whole immunoglobulins) that form polymers by aggregating with each other --> viscosity problems
Paraproteinemia Is what Paraproteinemia, also known as monoclonal gammopathy, is the presence of excessive amounts of paraprotein or single monoclonal gammaglobulin in the blood.
What is an M-protein? When para proteins are whole immunoglobulins (As opposed to just light chains or heavy chains)
What is a Bence Jones protein? A light chain that escaped into the urine
Diagnostic criteria for non-IgM MGUS 1. Serum M-protein (IgG, IgA, IgD) < 3 g/dl, 2. < 10% clonal plasma cells in bone marrow, 3. No CRAB
The presence of an IgD M protein often indicates what? Multiple myeloma, amyloid, plasma cell leukemia
Diagnostic criteria for light chain MGUS 1. Abnormal free light chain ratio kappa:lambda ratio <0.26 (lambda predominance) or >1.65 (kappa predominance), 2. No heavy chain ( IgG, IgA, IgD, IgM), 3. no CRAB
IgM MGUS diagnostic criteria 1. Serum M-protein (IgM) < 3 g/dl, 2. < 10% clonal plasma cells in bone marrow, 3. No CRAB, constitutional symptoms, hyper viscosity, lymphadenopathy, or hepatosplenomegaly
Diagnostic criteria for smoldering myeloma 1. Serum monoclonal protein >= 3 g/dL, 2.10% =< Clonal plasma cells in bone marrow < 60%, 3. No CRAB, normal free light chain ratio, no bone lesions
Diagnostic criteria for multiple myeloma 1. clonal plasma cells in bone marrow >= 10%, OR Biopsy proven plasmacytoma, AND 2. CRAB OR bone marrow plasma cells are sent for greater OR free light chain ratio >= 100, OR bone lesions
Created by: christinapham