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ABIM - Practice Exam
Practice Exam #2
| Question | Answer |
|---|---|
| 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