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MEDICAL GRANDROUNDS. Caroline M. Armas, MD Medical Resident Moderator: Dr Benjamin Benitez. OBJECTIVES. To present a case of a 52 year old male, who came in due to epigastric pain To discuss a complication of Polycythemia Vera. Identifying data. NDG 52 year old, male Married Catholic
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MEDICAL GRANDROUNDS Caroline M. Armas, MD Medical Resident Moderator: Dr Benjamin Benitez
OBJECTIVES • To present a case of a 52 year old male, who came in due to epigastric pain • To discuss a complication of Polycythemia Vera
Identifying data • NDG • 52 year old, male • Married • Catholic • From Brgy. Valenzuela, Makati City • Admitted: October 16, 2010
History of present illness Admission
Review of systems • No fever, cough, colds • No chest pain, no difficulty of breathing • No dysuria, frequency, urgency
Past medical history • Post Cerebrovascular accident (2006) • Acid Peptic Disease on AlOH2 + MgOH2 as needed
Family history • No hypertension, diabetes, thyroid disorders • No history of cancer • Denies history of blood dyscrasia
PERSONAL AND SOCIAL HISTORY • Previous smoker – stopped 2006 • 14 pack-year (10sticks/day for 28years) • Occasional alcoholic beverage drinker • 1-2 bottles of beer , 1-2x/month
PHYSICAL EXAMINATION • Conscious, coherent, ambulatory, not in respiratory distress • BP 110/70 mmHg HR 72 bpm RR 19cpm T 36C • Ht 152cm Wt 81kg BMI 25.6 • Supple neck, no neck vein distention, • Symmetric chest expansion, clear breath sounds • Quiet precordium, normal rate, regular rhythm, apex beat at 5th ICS MCL, no murmurs
Physical examination • Flat abdomen, normoactive bowel sounds, soft, (+) direct tenderness on epigastric area • No edema; Full and equal pulses • Neurologic examination: unremarkable
Salient features • 52/M • Known case of Polycythemia vera • Post cerebrovascular accident – no residuals • (+) epigastric pain • (+) direct tenderness on epigastric area
Initial impression • Acid Peptic Disease • Acute pancreatitis • Polycythemia Vera • Post Cerebrovascular accident with no residual
COURSE IN THE WARD • 1st Hospital Day • CBC, Amylase and Lipase • Plain film of abdomen • CT of whole abdomen (plain) • Nothing per orem • Pantoprazole 40mg IV once daily • Octreotide 250mcg subcutaneous, followed by 750mcg IV drip • Referred to Hematology service
Course in the ward Plain CT scan of whole abdomen: Acute pancreatitis Minimal ascites Atherosclerotic disease of the abdominal aorta
Acute Pancreatitis • Most Common causes: Gallstones (30-60%) and Alcohol (15 to 30%) • Abdominal pain is the major symptom • Diagnosis: increased level of serum amylase • CT scan may confirm the clinical impression of acute pancreatitis even in the face of normal serum amylase levels
Polycythemia Vera • Is a stem cell disorder • Prominent feature: elevated absolute red blood cell count because of uncontrolled red blood cell production • Increased white blood cell and platelet production due to an abnormal clone of hematopoietic stem cells with increased sensitivity to different growth factors of maturation
COURSE IN THE WARD • 3rd Hospital day • Still with epigastric pain, grade 7/10 • Repeat CBC • Referred to Infectious Diseases service • Blood culture • Imipenem 250mg IV every 6 hours
COURSE IN THE WARD • 5th Hospital Day • (+) abdominal pain, grade 2/10 • CBC, CEA, AFP, CA 19-9 • Diet: General liquids • Hydroxyurea 500mg 2tabs 2x/day
Hydroxyurea • Is a nonalkylating agent that inhibits DNA synthesis and cell replication by blocking the enzyme ribonucleotide reductase resulting in a megaloblastic blood picture • Onset of action is rapid, usually 3-5 days of initiation of treatment and effect is short-lived once medication is stopped • Initial dose is 15mg/kg per day, taken in divided doses
COURSE IN THE WARD • 7th hospital day • (+) abdominal pain, grade 5/10 • CBC • CT of whole abdomen with IV contrast
COURSE IN THE WARD • CT of Whole Abdomen with IV contrast • Portal vein thrombosis extending to the SMV. • Minimal ascites which has slightly increased since the previous examination. • Interval increase in the size of the gallbladder likely reactive in nature. • Colonic diverticulosis • Atherosclerotic abdominal aorta. • Minimal right pleural effusion.
Thrombosis in polycythemiavera • Thrombosis is a frequent complication in persons with Polycythemiavera • Result from the disruption of hemostatic mechanisms because of increased level of red blood cells and an elevation of platelet count. • Significant risk factors for thrombosis • History of prior thrombosis • Age over 60 years old • Prolonged exposure to substantial degrees of thrombocytosis
Polycythemia Vera:The Natural History of 1213 Patients Followed for 20 Years • Retrospective cohort • Subjects: 1213 patients with polycythemia vera • 14% had thrombotic events before diagnosis of polycythemia vera; and 20% had a thrombotic event as presenting symptom
The Natural History of 1213 Patients Followed for 20 Years polycythemia vera • Follow-up: • Fatal thrombosis – arterial thrombosis (81%) and venous thrombosis (18%); • Nonfatal thrombosis: • Superficial thrombophlebitis (18.5%) • Deep Vein Thrombosis (17.5) • Myocardial infarction (14%) • Ischemic stroke (9.5%)
COURSE IN THE WARD • 7th hospital day • Blood C/S: no growth • Imipenem was discontinued • Referred to TCVS • Baseline PT, PTT • Heparin drip 10000 units to run for 24 hours
Heparin • Is an indirect thrombin inhibitor which complexes with antithrombin converting it from a slow to a rapid inactivator of thrombin. • Limitation: narrow therapeutic window of adequate anticoagualtion without bleeding. • Monitor response using aPTT • Therapeutic level for first 24hours: 1.5times the control • Maintenance: 1.5-2.5 times
COURSE IN THE WARD • 12th Hospital day • Therapeutic platelet reduction Repeat CBC
Phlebotomy • Mainstay of therapy of Polycythemia Vera • Objective is to remove excess cellular elements to improve the circulation of blood by lowering blood viscosity.
COURSE IN THE WARD • 14th hospital day • Minimal abdominal pain • Chest heaviness • ECG, cardiac enzymes referred to Cardiology service • 2D-Echo • Clopidogrel 75mg daily, Nicorandil 5mg 2x/day Trimetazidine 35mg 2x/day, Bisoprolol 2.5mg daily
COURSE IN THE WARD • 16th hospital day • Febrile episodes (Tmax 38C) • (+) Rales on left lower base • Chest Xray and CBC • Digoxin 0.125mg IV daily and Spironolactone 25mg daily