Question of the Week # 433

433) A 27 year old woman with no significant past medical history presents to your office after she sustained an accidental cut from a clean, new kitchen knife. She has no significant family history. She does not smoke or drink. She enjoys hanging out in beaches over the weekends and sun tanning. She however, noticed that she tans very quickly when compared to her friends. She received Tetanus immunization as a child and her last tetanus booster was 1 year ago. She also reports she recently visited her gynecologist office at which time some routine labs were done. She has the copies of her lab reports with her which reveal:
WBC : 10k/µl

HGB: 15gm%

Platelets : 300k/µl

Iron : 150 mcg/dL

TIBC: 200 mcg/dl

Transferrin saturation : 75%

Serum Ferritin : 220 ( N= 12 to 200 ng/ml)

Further investigations revealed homozygos state for C282Y mutation. On examination, her skin is golden tan in color. She has a  1cm cut on her palm which is clean. The wound is cleaned with soap and water.

Which of the following is the most appropriate management?

A) Instruct her to increase Vitamin C intake to promote wound healing

B) Administer Tetanus Immunoglobulin

C) Administer Tetanus Toxoid

D) Instruct her to avoid contact with sea water until the wound is healed

E) Instruct her to increase Red Meat Intake

 

 

Question of the Week # 432

432) A 62-year-old man presents with painless, progressive enlargement in the left side of his neck over the past 3 months. He denies any fever, night sweats or chills. He denies any pain, dysphagia, cough, hemoptysis, chestpain, shortness of breath, history of trauma or dental infection. His past medical history is significant for  100 pack-year history of smoking and daily alcohol abuse. On examination, he is afebrile. He has a large 5 cm , hard, non-tender and matted lymphadenopathy on the left lateral aspect of his neck . He has no other lymph node enlargement. No rash. Reminder of the exam including oral cavity examination is normal. There are no obvious tongue, pharyngeal or tonsillar lesions.  HIV and VDRL are negative. A CT scan of the chest, abdomen and pelvis does not reveal any lymphadenopathy or obvious mass lesions. Which of the following is the most appropriate next step in management?

A) Open biopsy of the neck mass

B) Pan-endoscopy

C)  Fine Needle Aspiration Cytology ( FNAC)

D) Test for HPV ( Human Papilloma Virus) and EBV ( Ebstein Barr Virus)

E) Prescribe antibiotic therapy and re-evaluate in 4 weeks

Question of the Week # 431

431) A 25 year old G2P1L1 at 32 weeks gestation presents with right leg swelling and pain progressively increasing over the past two days. Her previous pregnancy was uneventful except for uncomplicated C-section and she has a 2 year old healthy male child. Past medical history is negative for any significant health issues. There is no family history of clots or cancer.  Physical examination is consistent with 32 week gestation. Right lower extremity is swollen about 3cm more in calf-circumference when compared to the left. There is tenderness in right calf. A Doppler ultrasound reveals thrombosis in superficial femoral vein. Factor V leiden mutation, Lupus anticoagulant, anti-cardioloipin antibodies, prothrombin gene mutation are negative. Anti-thombin III is within normal limits. She is scheduled for C-section in 6 weeks. Which of the following is the most appropriate Statement concerning her management?

A)     Since this is not deep vein thrombosis, start her on LMWH ( Low Molecular Weight Heparin) at prophylactic doses

B)      Start warfarin and continue for total 6 months

C)      Start LMWH, discontinue 2 days prior to C-section, start unfractionated heparin followed by discontinuation 4 hrs prior to C-section and then start LMWH 12 hrs after surgery followed by Warfarin for 6 months.

D)     Start LMWH, discontinue 2 days prior to C-section, start unfractionated heparin followed by discontinuation 4 hrs prior to C-section and then start LMWH 12 hrs after surgery followed by Warfarin for 6 weeks post-partum

E)      Start LMWH now through delivery followed by warfarin for 6 months post-partum.

Question of the Week # 430

430 ) A 70  year-old man is evaluated in your office for fatigue and unsteady gait. His symptoms started 2 months and progressively increasing. He also reports numbness and paresthesias in his bilateral lower extremities. His other issues include ill-fitting dentures for which he used dental adhesives for past 2 years. His past medical history is also significant for gastric bypass surgery approximately 8 years ago for recurrent peptic ulcer disease. He receives Vitamin B12 Subcutaneously every 3 to 6 months. On presentation, he is confined to a wheelchair. Neurological examination revealed markedly decreased vibratory sensation and proprioception in the lower extremities bilaterally. Both pin-prick and light touch sensations were moderately reduced below the knees. Muscle power, bulk, tone, and reflexes were normal. Rest of his physical examination is normal. Laboratory investigations reveal hemoglobin = 8.5 g/dL, MCV= 74 fL, MCHC = 33.6gm/dl, leukopenia with a WBC =1.9k/µl and absolute neutrophil count = 475 cells/µL. His platelet count, iron studies, folate and syphilis serologies were within normal limits.  Vitamin B12 was 900pg/ml ( N = 180 to 400pg/ml) . Serum Zinc level is  600µg/dl  ( Normal is 70-150 µg/dl).

Which of the following is most appropriate at this time?

A) Check Methyl Malonic Acid Level

B) Start Penicillamine

C)  Bone Marrow Biopsy

D) CT scan of the Head

E) Discontinue dental adhesives and check Copper levels

Interpreting Mixing Studies – Hematology Highyield Concept I

Approaching Prolonged PTT and understanding Mixing Studies : 

 

Archer USMLE Step 3

ARCHER HEMATOLOGY REVIEW

Question :  On a mixing study, the PTT corrects initially but gets prolonged again after incubation for 2 hours. What is this condition?

This description of mixing studies is consistent with presence of a temperature- and time-dependent anti-VIII inhibitor. It just means that the inhibitor is a warm reacting IgG antibody that requires one or two hour’s incubation at 37°C to be detected. If the PTT corrects initially but prolongs again after incubation (meaning if thePTT is at least 15% longer than the normal reagent plasma’s incubated PTT ) –> anti-VIII should be suspected. This can be confirmed by obtaining a Factor VIII level which will be low in case of presence of inhibitor. Further coinfirmation of Factor VIII inhibitor can be obtained by Bethesda Titer. In such cases, you should also carefully review patient’s prior bleeding history – because patients with significant inhibitor will have prior bleeding history. 

 

Discussion : 

 

All about Mixing Studies :  

 Mixing studies involve mixing patient’s plasma with equal amount of normal plasma. If the PTT immediately corrects ( that is if mixture yields within 10% of normal value) – it indicates Factor Deficiency ( example : VIII, IX, XI, XII) . If the PTT does not correct – it means an inhibitor is present. Such an inhibitor is most likely to be Lupus anticoagulant (LA) if there is no bleeding history. Factor VIII inhibitor is also possible but such presence of factor VIII inhibitor would be associated with significant history of bleeding which will help you differentiate from LA . If the PTT corrects initially but prolongs again after 1 to 2 hr incubation, it means a Factor VIII inhibitor is present.

 

Approaching LA diagnosis :

After you know that there is possible LA on mixing studies, you should proceed with confirmatory tests for LA. At least Two test systems are necessary to confirm LA – to avoid false negative tests because LAs are so heterogeneous . PTT based tests can be used. Dilute Russell Viper Venom tests are sensitive and quite specific for LAs. So, Lack of correction in mixing studies of either abnormal test is presumptive evidence for LA. These results can be further followed with neutralization studies using high phospholipid reagents. Correction by the neutralizing reagents confirms the presence of LAC.Remember that Lupus anticoagulants are partof a family of antibodies called antiphospholipid antibodies. These are also detected using a series of immunoassays:· Anticardiolipin IgG, IgM, orIgA antibody· Anti-b2 glycoprotein I IgGor IgM antibody. 

 

Approaching Factor VIII inhibitor Diagnosis:

Once the mixing study corrects initially but prolongs again after 1 to 2 hour incubation such a result is more consistent with Factor VIII inhibitor ( acquired Factor VIII inhibitor). You should obtain a careful history regarding previous bleeding from the patient. Alternatively, acquired factor VIII inhibitors can develop transiently after pregnancy and/ or major surgeries and such patients will not have any history of bleeding. Also, obtain a Factor VIII level which will be low in case of presence of inhibitor. Further confirmation and levels of of Factor VIII inhibitor can be obtained by Bethesda Titer. If these patients have high Factor VIII titer and have significant bleeding, you can not treat them with Factor VIII replacement because remember the inhibitor is an antibody and giving factor viii may further induce antibody response and can be threatening. If significant titers of Factor VIII inhibitor are present and if the patient is bleeding , you must use FEBA ( Factor EIGHT BYPASS ACTIVITY) or Factor VII to treat this patient’s acute bleeding .  

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Question of the Week # 429

429) A 72-year-old man presents for an out patient follow up after being discharged from the hospital two days ago. He was admitted for a transient ischemic attack on the right side of his body which resolved spontaneously. He was worked up and subsequently discharged. Review of his in-patient investigations reveal a carotid doppler which revealed 100% stenosis of the left carotid artery and a 40% stenosis of the right carotid artery. He smoked about 1 pack per day for past several years. His past medical history includes chronic atrial fibrillation for which he has been on Aspirin alone. During the last admission, he was started on Coumadin as well. Today, his INR is therapeutic at 2.5 . His fasting lipid profile shows a calculated LDL cholesterol of 60mg%.  On examination, there are no neurological deficits. His blood pressure is 110/70 mm Hg. In addition to recommending smoking cessation and lifestyle changes along with continuation of coumadin, which of the following is most appropriate at this time?

A) Carotid Endarterectomy of Right Carotid Artery

B) Carotid Stenting of left carotid artery

C)  Carotid Endarterectomy of Left Carotid Artery

D) Carotid Magnetic Resonance Angiography

E) No other intervention indicated

Question of the Week # 428

428) A 32-year-old male boxer presents to the emergency room after sustaining an injury during a local boxing match.  He was punched by the opponent over his right ear about six hours ago . He complains of pain and swelling over his right ear. He has no hearing deficit or tinnitus or headaches or blurred vision. He denies nausea or vomiting. Past medical history is unremarkable. On examination, his vitals are stable. His right ear is red, warm and swollen with a medium sized anterior auricular swelling which is tender to palpation. There is no facial swelling. If untreated, which of the following would be most likely course of this injury?

A) Spontaneous Resolution

B) Hearing Deficit

C) Permanent ear disfigurement

D) Otitis Externa

E) Mastoiditis

Question of the Week # 427

427) A 32-year-old male boxer presents to the emergency room after sustaining an injury during a local boxing match.  He was punched by the opponent over his right ear about six hours ago . He complains of pain and swelling over his right ear. He has no hearing deficit or tinnitus or headaches or blurred vision. He denies nausea or vomiting. Past medical history is unremarkable. On examination, his vitals are stable. His right ear is red, warm and swollen with a medium sized anterior auricular swelling which is tender to palpation. There is no facial swelling. Which of the following is the most appropriate management?

A) Compressive dressing

B) Needle aspiration of the Hematoma

C) Cold compresses, analgesics and antibiotics

D) Incision and Drainage

E) Observe and await  spontaneous hematoma resolution

Question of the Week # 426

426) A 70-year-old man with history of atrial fibrillation and metallic mitral valve presents to the out patient clinic for follow up. He has been taking warfarin for the past 5 years. His INR has been in therapeutic range between 3.0 to 4.0. However, over the last one month his INR has been in the range of  1.5 to 2.0 . His other medical problems include moderate osteoarthritis and mild cognitive dysfunction. He started taking Glucosamine supplements for “preserving his joints “and Ginseng to slow “ageing of his brain”  about two months ago. He has also been started on Aspirin by his cardiologist about 1 month ago. On examination, he is afebrile and vitals are stable. Abdominal examination is benign. A repeat INR is still sub-therapeutic at 1.5. Which of the following is the most appropriate management?

A) Increase Warfarin dose by 20%

B) Stop Glucosamine and repeat INR in 1 week

C) Continue Warfarin at same dose and repeat INR in 1 week

D) Stop Ginseng and repeat INR in 1 week

E) Stop Aspirin, increase Warfarin dose and repeat INR in 1 week

Question of the Week # 425

425) A 80 day old african american woman with advanced dementia is admitted to the intensive care unit for overwhelming sepsis secondary to a urinary tract infection. At presentation she was hypoxemic and was subsequently, intubated and placed on a ventilator. Blood and urine cultures are obtained and she is started on antibiotics A chest x-ray post-intubation shows endotracheal tube in place and bilateral diffuse alveolar infiltrates. On examination, his pulse is 110/min, Blood pressure is 110/80 mm Hg and respiratory rate is 18/min with the patient on Assist-Control mode on the ventilator. Pulse oximetry reveals 88% on Fio2 90%. Chest examination reveals diffuse crackles. Cardiac examination reveals tachycardia, there is no S3 gallop. Brain natriuretic peptide level is 50 pg/ml . A 2D echocardiogram shows normal left ventricular function with an ejection fraction of 60%. His ventilator settings are as follows : Assist-Control mode; Fio2 ( Fraction of inspired oxygen) : 90%, Respiratory rate : 18/minute, Tidal Volume : 500 ml, PEEP ( Positive End expiatory Pressure) : 5 cm H20 . Arterial blood gas analysis reveals ( on Fio2 70%): pH : 7.36, pCo2 : 45 mmHg, pO2 : 55 mm Hg, Bicarbonate : 23 Meq/L

Which of the following is indicated at this time?

A) Intravenous Furosemide

B) Swan-Ganz Catheter Placement

C) Increase PEEP by 3 cm H20

D) Increase Tidal Volume to 650 ml

E) Increase Fio2 to 100%

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