Hematology
Oncology
Rheumatology
Immune
Miscellaneous
100


A 60-year-old man on warfarin presents to the emergency depart-ment after a fall. His non-contrast head CT shows a subdural hema-toma, and his INR is 4. Which of the following is the recommended agent to reverse his coagulopathy?     

a.     Cryoprecipitate     

b.     Fresh frozen plasma    

c.     Prothrombin complex concentrate     

d.     Oral vitamin K

Answer: C.

 Prothrombin complex concentrate (PCC) is the recom-mended agent for reversal of elevated INR in life-threatening bleed-ing, including intracranial bleeding. 4 Factor PCC contains clotting factors II, VII, IX, and X making it the ideal agent for patients with elevated INR from warfarin as this medication inhibits synthesis of these vitamin K–dependent clotting factors. Cryoprecipitate con-tains fibrinogen, factor VIII, factor XII, von Willebrand factors, and fibronectin and is used most often clinically in cases of low fibrinogen

such as massive hemorrhage and DIC. Fresh frozen plasma can be used to reverse warfarin-induced coagulopathy; however, it often requires the administration of significantly more fluid volume and PCC more reliably lowers the INR in a shorter time frame. For these reasons PCC is the preferred treatment. Vitamin K will take hours to days to reverse the coagulopathy, as it relies on the bodies metabolic processes to synthesize clotting factors. It is, however, generally recommended to give IV vitamin K along with PCC, so that once the transfused factors have been consumed the body will have started to produce its own factors. 

100

63-year-old man presents to the ED 2 days after his last chemotherapy session for induction treatment of acute myelogenous leukemia. He has been vomiting and having diarrhea since his last session. He reports feeling dehydrated and is unable to tolerate oral intake. What lab studies are consistent with a diagnosis of tumor lysis syndrome in this patient?

A. High calcium, high phosphate, high potassium

B.High calcium, low phosphate, low potassium 

C.Low calcium, high phosphate, high potassium

D.Low calcium, low phosphate, low potassium

Answer C.

Tumor lysis syndrome occurs within a few hours to a few days after the initiation of chemotherapy or radiation therapy to treat bulky tumors. It is most commonly seen after chemotherapy of hematologic malignancies, including acute leukemias and high-grade non-Hodgkin lymphomas, particularly Burkitt lymphoma. Biochemical hallmarks of this syndrome include hyperkalemia from cytosol breakdown, hyperphosphatemia from protein breakdown, and hypocalcemia secondary to hyperphosphatemia.



100

28-year-old woman presents with fever, malaise, blurry vision, and extremity pain. Vital signs are significant for a temperature of 100.9°F and blood pressure of 170/95 mm Hg. On exam, she has diminished upper extremity pulses. Her blood work shows an erythrocyte sedimentation rate of 81 mm/h and C-reactive protein of 21 mg/dL. MRI shows inflammatory thickening of the vasculature. Which of the following is most likely to be affected in this patient?

       
A.Aortic arch

B.Coronary artery

C.Middle meningeal artery
D.Temporal artery


Correct Answer ( A )

This patient most likely has Takayasu arteritis, a large-vessel granulomatous vasculitis. Takayasu arteritis typically affects the aortic arch and carotid arteries, in contrast to giant cell (temporal) arteritis, a large-vessel vasculitis that predominantly affects the temporal artery. Patients with Takayasu arteritis may present with nonspecific constitutional symptoms such as fever, malaise, and weight loss.Large artery inflammation and damage can lead to decreased upper extremity pulses, unequal blood pressures in the arms,tenderness over the carotid arteries, bruits, hypertension, retinopathy, and limb claudication. Diagnosis is typically made via MRI, which shows inflammatory thickening of affected arteries. A CT angiography scan can also be used and would show stenosis, occlusion, and dilation of arteries. Since Takayasu arteritis is an inflammatory condition, patients may have elevated inflammatory markers such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Treatment is dependent on the severity of the disease and includes corticosteroids and immunosuppressive medications.

100

A 20-year-old man with a medical history of asthma presents with a rash and difficulty breathing. His symptoms started shortly after taking the first dose of an antibiotic that he was prescribed for cellulitis. Vital signs are a T of 98.5°F (36.9°C), BP of 89/50 mm Hg, HR of 130 bpm, RR of 22/min, and SpO2 of 92% on room air. On physical exam, he appears anxious and has diffuse urticaria. He is using accessory muscles and has wheezing on lung exam. What is the best next step in management?

A.Intramuscular epinephrine
Correct Answer

B.Intravenous epinephrine

C.Intravenous methylprednisolone

D.Nebulized albuterol

Correct Answer ( A )

This patient, who presents with urticaria, bronchospasm, and signs of shock, has anaphylaxis, a life-threatening hypersensitivity reaction that can lead to airway, breathing, and circulatory dysfunction. Common triggers include foods, medications, and insect stings. Patients can present with a range of symptoms and disease severity, from mild wheezing and urticaria to shock and complete airway obstruction. Most patients present with mucocutaneous involvement, including urticaria, pruritus, lip and oropharyngeal swelling, periorbital edema, and conjunctival injection. Lower respiratory symptoms include wheezing and shortness of breath, while upper airway involvement can present with stridor, voice changes, throat tightening, and airway obstruction. Gastrointestinal symptomsinclude nausea, vomiting, diarrhea, and abdominal pain. If there is hypotension, patients may have dizziness or syncope. The diagnosis is based on the clinical history and exam findings. According to the World Allergy Organization diagnostic criteria, anaphylaxis is likely in a patient with an acute onset of illness with mucocutaneous involvement and at least one of the following: respiratory compromise, hypotension, or severe gastrointestinal symptoms. In the absence of skin involvement, anaphylaxis is likely in those presenting with hypotension, bronchospasm, or laryngeal involvement (e.g., stridor, voice changes) after exposure to a known or likely allergen. 


The mainstay of treatment for anaphylaxis is intramuscular epinephrine 0.01 mg/kg (maximum 0.5 mg) administered in the anterolateral thigh. Epinephrine acts on the alpha-1, beta-1, and beta-2 adrenergic receptors to decrease mucosal edema and increase systemic vascular resistance, increase inotropy and chronotropy, and promote bronchodilation, respectively. Epinephrine is the only medication that has been shown to be effective in treating anaphylaxis, yet it is underused in emergency settings. This is likely due to the fear of causing unwanted side effects such as cardiac ischemia, especially in older patients. However, when administered at the correct dose, intramuscular epinephrine is safe and should not be withheld due to concerns about side effects. The initial dose of epinephrine can be repeated at 5-minute intervals. For patients requiring multiple doses or who have persistent shock, an intravenous infusion of epinephrine should be started, along with fluid resuscitation. 


Adjunctive medications include glucocorticoids and antihistamines, both H1 and H2 blockers. These medications are routinely administered, but there are no high-quality data showing that glucocorticoids or antihistamines are effective for the acute management of anaphylaxis. Use of these medications should not replace or delay the administration of epinephrine. Inhaled bronchodilators can also be used adjunctively in those with wheezing. Patients who have an adequate response to intramuscular epinephrine can be discharged after a period of observation with a prescription for an epinephrine autoinjector. Practice patterns vary, but in general, prescriptions for glucocorticoids or antihistamines are not necessary and may cause unwanted side effects.

100

A 6-year-old boy with a history of sickle cell disease is brought to the emergency department for decreased activity. He had 3 days of cough and fever that resolved with over-the-counter medications 2 weeks prior. His HR is 150 bpm, BP is 80/50 mm Hg, and RR is 30/min. Physical examination shows pale conjunctivae and reticulated, blanching erythema over the extremities. A review of previous laboratory findings shows a baseline hemoglobin of 10 g/dL with a reticulocyte count of 8%. A chest X-ray is performed and shows no focal infiltrates. Which of the following, if present, would be a formal indication for packed red blood cell transfusion in this patient?

A.Chest pain

B.Hemoglobin of 7.5 g/dL

C.Lack of improvement with IV fluids

D.Reticulocyte count of 4%

E.Splenomegaly


Correct Answer ( B )

This child has a classic presentation of erythema infectiosum as a result of parvovirus B19, which is characterized by 2–3 days of a nonspecific viral upper respiratory illness followed by the development of a reticulated lace-like rash over the extremities. A slapped-cheek rash is more classically described but may be absent in some patients. The rash is thought to be immune mediated, and active symptoms are often absent by the time it develops. Patients with sickle cell disease have a decreased average red blood cell lifespan and increased baseline rates of hematopoiesis, resulting in rapid red blood cell turnover. Any illness, nutritional deficiency, or physiologic stressor that reduces the rate of hematopoiesis can result in severe acute exacerbations of chronic anemia. Parvovirus B19 can destroy erythroid precursors, resulting in mild transient anemia in healthy patients but severe anemia in patients with sickle cell disease. A reduction in the patient’s baseline reticulocyte count and red blood cell count is diagnostic of aplastic anemia. Aplastic anemia secondary to parvovirus B19 typically resolves within 2 weeks. Management is focused on transfusion in patients with a drop in hemoglobin of 2 g/dL below their baseline (< 8 g/dL in this patient) or new symptoms suggestive of symptomatic anemia such as hemodynamic instability, tachycardia, dizziness, dyspnea, or mental status change. 

200

A 76-year-old female is receiving a unit of packed red blood cells, when she suddenly develops shortness of breath. On examination, she has crackles on auscultation of her lungs bilaterally. Which of the following is true regarding transfusion-associated circula-tory overload (TACO) and transfusion-related acute lung injury (TRALI)?   

 a.? Chest x-ray can often distinguish between TACO and TRALI.   

b. Only TACO should be treated with noninvasive positive-pressure ventilation.

c. TACO typically requires the presence of a significant comorbid-ity.  

d. TRALI can be treated with aggressive diuresis

Answer: C. 

While it is possible to receive enough blood products for a healthy person to become volume overloaded, TACO is much more commonly seen in patients with underlying heart or renal failure. Both TACO and TRALI result in pulmonary edema, meaning they can present with similar chest x-ray findings and physical exam find-ings. Both entities can be treated with noninvasive positive-pressure ventilation or intubation and mechanical ventilation, as both treat-ments are effective in treating pulmonary edema. TRALI, however, will not respond to diuretics, because the cause of the pulmonary edema is an inflammatory response within the lungs rather than sys-temic volume overload

200

A 33-year-old woman with breast cancer presents with fever and neutropenia. She is status post mastectomy and undergoing che-motherapy. She denies respiratory symptoms and chest x-ray is unremarkable. Vital signs are T 38.8°C; HR, 110 beats/min; and BP, 110/60 mm Hg. Examination is otherwise normal. She has no indwelling catheters. Which of the following is the most appropri-ate empirical antibiotic regimen?

a.Antibiotics should be withheld pending radiographs and cultures     

b.Cefepime as monotherapy    

 c. Ceftazidime and gentamicin     

d.Meropenem and vancomycin

e.Ticarcillin and amikacin


Answer: B.

 Monotherapy with imipenem, meropenem, ceftazidime, or cefepime is as effective as traditional dual therapy with an antipseudo-monal penicillin and aminoglycoside. However, vancomycin should be included as well if any of the following exist: • ? Hypotension or evidence of cardiovascular impairment • ? Clinically suspected catheter infection • ? Positive blood cultures for gram-positive organisms • ? Known colonization with methicillin-resistant Staphylococcus aureus or cephalosporin-resistant Pneumococcus


200

Which of the following clinical findings differentiates rheumatoid arthritis from osteoarthritis?

       
A. Involvement of the proximal interphalangeal joints

B.Polyarticular involvement

C. Presence of constitutional symptoms

D. Symmetric joint involvement

Correct Answer ( C )

Osteoarthritis (OA) and rheumatoid arthritis (RA) share a number of features, but constitutional symptoms are only seen in RA. RA is a chronic inflammatory disease. Patients often present with fever, weakness, and musculoskeletal pain lasting for weeks to months. Arthritis involves symmetric joints of the hands, wrists, and elbows. Morning stiffness is common and usually lasts for about an hour. Inflammation may affect other organs leading to hepatitis, scleritis, myocarditis, pericarditis, and pleuritis. Atlantoaxial subluxation may also occur, and cervical spine precautions should be maintained during intubation.

200

What is the appropriate initial dose and route for a pediatric patient in the treatment of acute anaphylaxis?  

 a. 0.3 to 0.5 mg of 1 mg/mL concentration epinephrine concentra-tion, IM in anterolateral thigh  b. 1 mL of 1: 1000 epinephrine IV   

c. 0.01 mg/kg of 1: 1000 epinephrine, IM in anterolateral thigh   

 d. 2 mL of 1: 10,000 epinephrine IV every 5 minutes   

e.0.01 mg/kg of 1: 10,000 epinephrine IV

Answer: c. 

0.01 mg/kg (1: 1000 concentration) given IM in the antero-lateral thigh every 5 to 10 minutes as necessary is recommended for pediatric patients. Alternatively, an EpiPen Jr 0.15 mL can be consid-ered in patients weighing 10 to 25 kg.

200

A 54-year-old male with poorly controlled hypertension and cor-onary artery disease on daily aspirin presents with a swollen first metatarsophalangeal (MTP) joint on the right foot. You make the diagnosis of gout. Which of the following is the most appropriate initial treatment for this patient with a presumptive diagnosis of gout?

a.     Naproxen 550 mg twice daily

b.     Naproxen 550 mg twice daily coupled with daily proton pump inhibitor (PPI) therapy     

c.     Colchicine     

d.     Allopurinol and colchicine     

e.     Febuxostat and naproxen


Answer: b.
This patient is moderate risk for gastrointestinal (GI) injury due to history of poorly controlled hypertension and daily aspirin use; thus an oral nonsteroidal antiinflammatory drug (NSAID) coupled with daily PPI use is reasonable. If the patient had no cardiac or GI risk factors, use of NSAID alone would be appropriate. Colchicine is an alternative therapy, although given its side effect profile, another NSAID is generally preferred. Allopurinol and febuxostat are medica-tions that may be used to prevent gout attacks but should generally not be started during an acute flair and are not typically initiated in the ED setting.      

300

 ? A 36-year-old female patient is receiving a unit of packed red blood cells to treat her chronic anemia from end-stage renal disease when she develops a temperature of 38.2°C. The rest of her vital signs are normal. Which of the following is the best next step in manage-ment?    

a.? Administer acetaminophen 

b. Given a bolus of 1 L of NS    

c.Start broad spectrum antibiotics   

d.Stop the blood transfusion

Answer: D.


 The first step in any suspected transfusion reaction is to stop the blood transfusion. If after investigation and brief observation this appears to be a non-hemolytic febrile transfusion reaction, you may administer acetaminophen and resume the transaction. Fluids resuscitation is the mainstay of treatment for hemolytic transfusion reactions, which can present as a fever. However, as is the case with any transfusion reaction, the first step is stopping the blood transfu-sion. Antibiotics are indicated in bacterial culture positive transfusion reactions. Typically patients will experience other vital signs abnormal-ities, such as tachycardia and hypotension, when having one of these reactions.



300

What is the Discharge criteria in patient with febrile neutropenia ? (3 out of 5)

small fraction of patients may be safely treated with enteral antibiotics in the outpatient setting.

Discharge criteria : 

These patients should

 (1) meet the low-risk criteria of a MASCC score of 21 or less; 

(2) have no evidence of pneumonia, line infection, cellulitis, or organ failure;

 (3) have reliable daily follow-up with their oncologist.

(4) demonstrate clinical stability during observation in the ED for 4 hours or longer.

 (5) carry low suspicion of infection with a drug resistant organism. 

Prior to discharge, an initial dose of parenteral antibiotics should be given in the ED, reliable follow-up and access to the outpatient antibiotic regimen must be ensured, and discharge should be coordinated with the patient’s oncologist.

 Abx of choice in such a patient : 

300

A 53-year-old woman presents reporting severe acute neck pain at the base of her skull, intermittent occipital headaches, bilateral paresthesias in her arms, and vertigo when she looks down. She has a longstanding history of progressively worsening symmetric painful swelling in her joints. Her vital signs are temperature 98.7°F (37.1°C), blood pressure 129/70 mm Hg, heart rate 93 bpm, respiratory rate 17/min, and oxygen saturation 100%. Which condition is the most likely underlying etiology of her symptoms?

A.Giant cell arteritis

B.Polyarteritis nodosa

C.Rheumatoid arthritis

D.Transverse myelitis

Rheumatoid arthritis is a chronic systemic inflammatory disorder that has a predilection for the atlantoaxial joint. Instability and subluxation of the atlantoaxial joint may be spontaneous or related to minor injury. Many patients with atlantoaxial instability have no symptoms. Flexion and extension of the cervical spine are contraindicated in patients with rheumatoid arthritis and may precipitate symptoms of acute cervical myelopathy. Symptoms suspicious for instability or subluxation include severe cervical pain that radiates occipitally, upper limb paresthesias and vertigo, and other signs of vertebrobasilar ischemia. Additional complications of rheumatoid arthritis may include inflammatory manifestations throughout the body such as constitutional symptoms, symmetric inflammatory polyarthritis, skin lesions, interstitial lung disease, inflammatory cardiac conditions, vascular disease, hematologic manifestations, and Sjögren syndrome. Cervical CT scan may reveal fracture or subluxation, but MRI is required to assess the extent of spinal cord involvement in symptomatic patients.

300

A 57-year-old deceased donor kidney transplant recipient is sent to the ED from her primary care physician's office 3 months postop-eratively because routine blood work revealed a creatinine of 3.45 (baseline creatinine 1.0). Which of the following medications may be responsible for her acute kidney injury?     

a. Albuterol     

b.Tacrolimus     

c.Pancrelipase    

 d. Prednisone

 Answer: B. Tacrolimus is associated with dose-dependent nephrotox-icity. A serum level of tacrolimus should be obtained in any solid organ transplant recipient maintained on this immunosuppressive agent pre-senting with acute kidney injury. It is important to recognize that tac-rolimus can both cause and be affected by renal insufficiency, so it may not have been the initial trigger but may have ultimately worsened the renal injury. Alternatively, a new drug (fluconazole, azithromycin, etc.) may have been initiated which resulted in higher serum concentrations of tacrolimus and resultant renal injury.

300

A 42-year-old male presents with pain, warmth, and swelling over his posterior elbow.The patient reports frequently having to lean on his elbow while performing electrical work as part of his job. The patient has full range of motion but flexion and extension of the elbow results in increased pain. Fluid is subsequently aspirated from the affected area. Which of the following is true?

A. Empiric antibiotic therapy should target gram-negative organisms

B. Olecrnon bursitis is caused by hematogenous spread

C. Septic bursitis may be present if the bursal white blood cell count is >10,000 per mm

D. Treatment with antibiotics alone is usually successful

E. The bursa should be incised, drained, and packed


Answer C. This patient has olecranon bursitis. Olecranon bursitis most commonly occurs because of repetitive microtrauma caused by leaning or rubbing of the elbow. Hematogenous spread affects deeper bursae. Although most cases of olecranon bursitis are sterile inflammatory reactions, septic bursitis may account for as many as 33% of olecranon bursitis cases. Furthermore, because of the superficial location, septic bursitis is most common in the olecranon and prepatellar bursae, and rarely occurs elsewhere. Predisposing factors to septic bursitis include patients who are immunocompromised because of diabetes, renal insufficiency, and cancer as well as patients with anatomic abnormalities of the joint spaces and surrounding structures such as patients with gouty or rheumatoid arthritis (both of which may involve the bursa). Aspiration of the bursa is the only reliable means available to help differentiate between septic and sterile bursitis. In contrast to the higher number of leukocytes in septic arthritis, septic bursitis may be present when the WBC count is only 10,000 per mm 3 or lower (one study used 2,000 as a threshold). However, the average cell count in septic bursitis exceeds 60,000. Gram stain is positive in only 50% of cases, and Staphylococcus aureus is the most common responsible organism.

400

A 21-year-old man presents with easy fatigue and lack of energy. During a recent clinic visit, he was found to be anemic with a hemoglobin of 8 g/dL, mean corpuscular hemoglobin concentra-tion (MCHC) of 25%, mean corpuscular volume (MCV) of 61 fL, and a peripheral smear remarkable for target cells and basophilic stippling. Serum iron levels were normal. What is the most likely explanation for his anemia? 

 a.Iron deficiency     

b.Porphyria     

c. Sideroblastic anemia     

d. Thalassemia

Answer: d.

 Hypochromic, microcytic anemias with normal serum iron imply thalassemia (a defect in globin chain production). Both alpha- and beta-thalassemia manifest as a hypochromic microcytic anemia with target cells and basophilic stippling. The microcytosis is generally more severe than with iron-deficiency anemia. Sideroblastic anemia generally presents with elevated serum iron.


400

 72-year-old woman with chronic myelogenous leukemia and heart failure with an ejection fraction of 30% is brought to the ED by her adult child for altered mental status. The patient reports blurred vision, headache, and fatigue. On exam, she appears confused. Labs reveal WBC 157,000/mm3, hemoglobin 8 g/dL, and platelets 78,000/mm3. Her peripheral smear is seen above. What is the best next step in the treatment of this patient?

A.Fluid resuscitation

B.Hemodialysis

C.Leukapheresis

D.Red blood cell transfusion


Correct Answer ( C )

This patient has hyperviscosity syndrome. The syndrome is characterized by sludging and leads to decreased perfusion secondaryto increased products (paraproteins, RBCs, and WBCs) in the bloodstream. Most commonly, this is caused by a malignancy that results in hyperproduction of a cell line. In this case, the patient has a blast crisis in a previously indolent leukemia and is now experiencing sludging in the microcirculation, which has caused local ischemia. The presenting symptoms can be subtle and most commonly include altered mental status, fatigue, and headache. White blood cell counts > 100,000/mm3, or leukocrits > 10% are usually found. The Initial treatment is usually aggressive fluid resuscitation or phlebotomy and replacement with intravenous fluids. However, in patients who may not be able to tolerate large volumes of fluid, leukapheresis is the primary treatment when there is a blast transformation, as seen in this patient. When the hyperviscosity is due to a dysproteinemia, such as multiple myeloma or Waldenstrom macroglobulinemia, the treatment is plasmapheresis

400

A 72-year-old male with history of gout presents to the emergency department (ED) with worsening knee pain and swelling. He states this pain is worse than previous gout episodes. A diagnostic arthro-centesis is performed. Which of the following synovial fluid results would be most consistent with a diagnosis of septic arthritis?    

 a.Gram stain negative, positive crystals (needle-shaped), synovial white blood cell (WBC) count 25K, polymorphonuclear (PMN) 70%, turbid appearance   

b.Gram stain negative, negative crystals, synovial WBC count 12K, red blood cell (RBC) 1K, fat globules in aspirate    

c. Gram stain negative, positive crystals (needle-shaped), synovial WBC count 40K, PMN 92%, turbid appearance   

d. Gram stain negative, positive crystals (rhomboid-shaped), syno-vial WBC count 35K, PMN 60%

Answer: c.

A synovial fluid sample with PMN greater than 90% is pre-sumed to be consistent with septic arthritis regardless of total synovial WBC count. Answer choices a and d may represent septic arthritis in certain scenarios, but neither is diagnostic. These represent acute gout and pseudogout, respectively. Answer b is representative of a lipo-hemarthrosis consistent with intra-articular fracture. A positive Gram stain would also be diagnostic of septic arthritis, although approxi-mately 50% of cases of septic arthritis are associated with a negative Gram stain.

400

A 65-year-old woman presents to the emergency department with 2 weeks of decreased urine volume. Her medical history is notable for a kidney transplant 3 months ago. She stopped taking all her medications last week because they make her nauseous. Her vital signs on arrival are T 38.5°C, HR 120 bpm, RR 18/min, BP 190/110 mm Hg, and SpO2 98% on room air. Her lab work shows a doubling of her baseline serum creatinine, and her urinalysis is notable for 3+ proteinuria and > 182 WBC/hpf. Which of the following best describes the pathogenesis of this patient’s disease process?

A.Fibrosis and glomerulosclerosis

B.Infection secondary to immunosuppression

C.Lymphocytic infiltration of graft tissue

D.Massive capillary thrombosis

Correct Answer ( C )

The patient is presenting with symptoms of acute rejection. This diagnosis is suggested by rising serum creatinine, worsening proteinuria, and worsening hypertension in a patient who is < 1 year post-transplant, especially if she is off immunosuppressant medications. In patients who are not taking their immunosuppressants, graft pain, fever, malaise, and decreased urine output can occur. Most patients with acute rejection are asymptomatic, and the diagnosis is made through routine laboratory surveillance. Laboratory findings consistent with acute rejection include a rise in serum creatinine, pyuria, and new or worsening proteinuria. Biopsy with histological analysis is needed for a definitive diagnosis. In acute rejection, the histological analysis will show lymphocytic infiltration of the transplanted tissue. Patients with evidence of allograft rejection should be admitted, and early communication with the patient’s transplant surgeon regarding the initiation of antirejection medications should be considered.


400

Name of this finding ? With 2 Ddx 

 Raynaud phenomenon

systemic lupus erythematosus 

Rhumatoid Arthritis 

scleroderma

Buerger Disease (Thromboangiitis Obliterans)

500

A 35-year-old male with a history of sickle cell disease presents with acute onset of fever, malaise, fatigue, and lightheadedness. Physical examination demonstrates a tachycardic patient with pale conjunctivae. You suspect aplastic crisis and draw a complete blood count with reticulocyte count. You review his old records and note that the patient’s baseline hemoglobin level is 8 g/dL. Which of the following laboratory abnormalities is most consistent with an aplastic crisis?

A. Hemoglobin 8 g/dL, reticulocyte count 6%

B. Hemoglobin 8 g/dL, reticulocyte count 1%

C. Hemoglobin 6 g/dL, reticulocyte count 6%

D. Hemoglobin 6 g/dL, reticulocyte count 1%

E. Hemoglobin 4 g/dL, reticulocyte count 6%

Answer D. Sickle cell disease is a hemoglobinopathy causing sickling of red blood cells with any systemic stress, which results in diffuse microinfarctions. Sickle cell trait is present in approximately 10% of all African Americans, and sickle cell disease is primarily a disease of this population. Symptoms involve multiple organ systems and result in specific acute crises—vaso-occlusive, acute chest syndrome, splenic sequestration, and aplastic. Aplastic crises are characterized by the acute onset of worsening anemia combined with bone marrow failure. Laboratory abnormalities demonstrate a drop of hemoglobin of 2 g/dL from stable levels and an inadequate reticulocyte response (<2%) from the bone marrow to this sudden anemia. Aplastic crises are usually postinfectious and are responsible for 5% of all deaths in sickle cell patients.

500

List 5 causes of fever in oncology patient ? 

1. Infection* (only identified in 1⁄3 of patients)

2. DVT / Pulmonary embolus

3. Chemotherapy or medication effect

4. Direct tumor burden effect

5. Transfusion reaction

500

Which of the following symptoms is least likely to be seen in a patient presenting with giant cell arteritis?     a.? Amaurosis fugax 

b.Constitutional symptoms   

c.Dysphagia     

d. Jaw claudication    

e.Scalp tenderness

Answer: c.
Patients presenting with giant cell arteritis (GCA) com-monly have constitutional symptoms. Other presenting symptoms include headache and scalp tenderness. Jaw claudication is relatively specific for GCA, and visual symptoms (e.g., amaurosis fugax) may herald impending blindness from the disease if not treated. Dysphagia is not a presenting feature of GCA.

500

Which of the following is true regarding anaphylaxis?

A. Bee stings are the most common cause.

B. Exercise may trigger anaphylaxis.

C. Anaphylaxis usually occurs upon first exposure to an allergen.

D. Anaphylaxis is a type IV hypersensitivity reaction.

E. The risk of anaphylaxis is greatest in the very young and elderly.

Answer B. 

Foods are the single most common cause of anaphylaxis although up to one-third of causes are unknown. Exercise accounts for 7% of anaphylaxis cases. Anaphylaxis occurs as an immediate type I hypersensitivity reaction mediated by immunoglobulin E (IgE) antibodies. It requires prior sensitization to the allergen in order to develop allergen-specific IgE antibodies. Subsequent exposure to the allergen allows mast cell and basophil degranulation leading to the subsequent anaphylactic response. Anaphylactoid reactions are immediate type I hypersensitivity reactions that do not require prior allergen sensitization and which clinically mimic anaphylaxis. Anaphylactoid reactions (e.g.,response to iodinated radiographic contrast material) typically require a larger dose of the offending agent, but the clinical management is identical to anaphylaxis. Patients at the extremes of age are less likely to have anaphylaxis, probably because of less mature immune responses

500

A 5-year-old boy is brought to the ED due to abdominal pain. For the past week, he has had intermittent abdominal pain without a clear pattern in onset or remission and intermittent roving joint pains. Prior to the onset of these symptoms, he had a bilateral lower extremity macular and erythematous rash that developed into several small palpable areas of purpura with surrounding patchy nonblanching erythema and purplish color. Which of the following is most likely to be abnormal upon further evaluation?

A.Coagulation studies

B.Complete blood count

C.Ophthalmologic examination

D.Urinalysis



Answer D 

IgA vasculitis is most common between the ages of 3 and 15 years with a male predominance. It results from IgA deposition of unknown etiology in tissues with resulting cytotoxic immune system activity. The most common set of guidelines used to direct further investigation was published in 2005 by the European Alliance of Associations for Rheumatology (EULAR). The presence of purpurais mandatory for diagnosis, and one of the following must be present: abdominal pain, acute arthralgias, or signs of kidney involvement on urinalysis. These criteria are sensitive but not specific. 


Further testing and expert consultation are required for patients meeting these guidelines. IgA vasculitis does not cause significant alterations in platelet counts, platelet function, coagulation factor levels, or coagulation factor activity. The most common laboratory abnormalities are microscopic hematuria and proteinuria due to IgA deposition within the nephrons and resulting kidney injury. In pediatric patients, the diagnosis is clinical. A skin or kidney biopsy is required in adult patients to rule out other more common causes of small vessel vasculitis. Treatment of IgA vasculitis is typically supportive. Most episodes self-resolve and do not recur. Monitoring for recovery of kidney function is important in patients with significant kidney involvement.

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