caroline m. armas, md medical resident moderator: dr benjamin benitez
TRANSCRIPT
Caroline M. Armas, MDMedical 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 features52/MKnown case of Polycythemia veraPost cerebrovascular accident – no
residuals(+) epigastric pain(+) direct tenderness on epigastric area
Initial impressionAcid Peptic DiseaseAcute pancreatitisPolycythemia VeraPost Cerebrovascular accident with no
residual
COURSE IN THE WARD1st Hospital Day
CBC, Amylase and LipasePlain film of abdomenCT of whole abdomen (plain)Nothing per oremPantoprazole 40mg IV once dailyOctreotide 250mcg subcutaneous,
followed by 750mcg IV dripReferred to Hematology service
COURSE IN THE WARDCBCHemoglobin 10.7Hematocrit 33.5WBC 11.67Segmenters 63Lymphocytes 22Eosinophils 2Basophils 2Monocytes 11Platelet count 1267000MCV 84.4MCH 27MCHC 31.9RDW 17
Amylase 48
Lipase 33.6
PFA October 16 2010
Course in the wardPlain CT scan of whole abdomen:
Acute pancreatitisMinimal ascitesAtherosclerotic disease of the abdominal aorta
Acute PancreatitisMost Common causes: Gallstones (30-60%)
and Alcohol (15 to 30%)Abdominal pain is the major symptomDiagnosis: increased level of serum amylaseCT scan may confirm the clinical impression
of acute pancreatitis even in the face of normal serum amylase levels
Polycythemia VeraIs a stem cell disorderProminent 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 WARD3rd Hospital day
Still with epigastric pain, grade 7/10Repeat CBCReferred to Infectious Diseases service
Blood culture Imipenem 250mg IV every 6 hours
COURSE IN THE WARDCBC 3rd HD
Hemoglobin 10.7 10.9
Hematocrit 33.5 34.6
WBC 11.67 14.91
Segmenters 63 52
Lymphocytes 22 27
Eosinophils 2 3
Basophils 2 2
Monocytes 11 16
Platelet count 1267000 1342000
MCV 84.4 85.6
MCH 27 27
MCHC 31.9 31.5
RDW 17 17.1
COURSE IN THE WARD5th Hospital Day
(+) abdominal pain, grade 2/10 CBC, CEA, AFP, CA 19-9Diet: General liquidsHydroxyurea 500mg 2tabs 2x/day
COURSE IN THE WARDCBC 3rd HD 5th HD
Hemoglobin 10.7 10.9 11
Hematocrit 33.5 34.6 35.4
WBC 11.67 14.91 14.84
Segmenters 63 52 55
Lymphocytes 22 27 18
Eosinophils 2 3 13
Basophils 2 2 2
Monocytes 11 16 12
Platelet count 1267000 1342000 978000
MCV 84.4 85.6 86.1
MCH 27 27 26.8
MCHC 31.9 31.5 31.1
RDW 17 17.1 17
5th HD
AFP (8.6) 1.41
CA19-9 (0-39) 4.81
CEA (0-5.5) 0.92
HydroxyureaIs 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 WARD7th hospital day
(+) abdominal pain, grade 5/10CBCCT of whole abdomen with IV contrast
COURSE IN THE WARDCBC 3rd HD 5th HD 7th HDHemoglobin 10.7 10.9 11 11.3
Hematocrit 33.5 34.6 35.4 36.4WBC 11.67 14.91 14.84 13.17
Segmenters 63 52 55 56Lymphocytes 22 27 18 27Eosinophils 2 3 13 8
Basophils 2 2 2 Monocytes 11 16 12 9Platelet count 1267000 1342000 978000 934000
MCV 84.4 85.6 86.1 86.7
MCH 27 27 26.8 26.9MCHC 31.9 31.5 31.1 31RDW 17 17.1 17 17.4
COURSE IN THE WARDCT 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 diverticulosisAtherosclerotic abdominal aorta.Minimal right pleural effusion.
Thrombosis in polycythemia veraThrombosis is a frequent complication in
persons with Polycythemia veraResult from the disruption of hemostatic
mechanisms because of increased level of red blood cells and an elevation of platelet count.
Significant risk factors for thrombosisHistory of prior thrombosisAge over 60 years oldProlonged exposure to substantial degrees
of thrombocytosis
Polycythemia Vera:The Natural History of 1213 Patients Followed for 20 YearsRetrospective cohortSubjects: 1213 patients with polycythemia
vera14% 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 veraFollow-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 WARD7th hospital day
Blood C/S: no growthImipenem was discontinued
Referred to TCVSBaseline PT, PTTHeparin drip 10000 units to run for 24
hours
HeparinIs 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 aPTTTherapeutic level for first 24hours:
1.5times the controlMaintenance: 1.5-2.5 times
COURSE IN THE WARD
COURSE IN THE WARD12th Hospital day
Therapeutic platelet reduction Repeat CBC
COURSE IN THE WARDCBC 3rd HD 5th HD 7th HD 13th HD
Hemoglobin 10.7 10.9 11 11.3 10.7
Hematocrit 33.5 34.6 35.4 36.4 34.4
WBC 11.67 14.91 14.84 13.17 8.54
Segmenters 63 52 55 56 55
Lymphocytes 22 27 18 27 24
Eosinophils 2 3 13 8 5
Basophils 2 2 2
Monocytes 11 16 12 9 6
Platelet count 1267000 1342000 978000 934000 623000
MCV 84.4 85.6 86.1 86.7 85.4
MCH 27 27 26.8 26.9 26.6
MCHC 31.9 31.5 31.1 31 31.1
RDW 17 17.1 17 17.4 17.7
PhlebotomyMainstay of therapy of Polycythemia VeraObjective is to remove excess cellular
elements to improve the circulation of blood by lowering blood viscosity.
COURSE IN THE WARD14th hospital day
Minimal abdominal painChest heavinessECG, cardiac enzymes referred to
Cardiology service2D-EchoClopidogrel 75mg daily, Nicorandil
5mg 2x/day Trimetazidine 35mg 2x/day, Bisoprolol 2.5mg daily
COURSE IN THE WARD
ECG Probable old inferior wall MINonspecific ST-Twave changes
2D-Echo Interventricular septal hypertrophy with hypokinetic posterior and inferior walls from mid to apex. Mildly depressed left ventricular systolic function with EF of 52%. Mild mitral tricuspid and pumonic regurgitation. Normal pulmonary artery pressure. Doppler evidence of impaired LV diastolic dysfunction.
TCPK 73
Trop I 0.3
CPK-MB 1.5
COURSE IN THE WARD16th hospital day
Febrile episodes (Tmax 38C)(+) Rales on left lower baseChest Xray and CBCDigoxin 0.125mg IV daily and
Spironolactone 25mg daily
CHEST X-ray October 31, 2010
COURSE IN THE WARDCBC 3rd HD 5th HD 7th HD 13th HD 16th HD
Hemoglobin 10.7 10.9 11 11.3 10.7 10.3
Hematocrit 33.5 34.6 35.4 36.4 34.4 32.9
WBC 11.67 14.91 14.84 13.17 8.54 9.16
Segmenters 63 52 55 56 55 70Lymphocytes 22 27 18 27 24 22
Eosinophils 2 3 13 8 5 1
Basophils 2 2 2 1
Monocytes 11 16 12 9 6 6Platelet count 1267000 1342000 978000 934000 623000 415000
MCV 84.4 85.6 86.1 86.7 85.4 86.1
MCH 27 27 26.8 26.9 26.6 27
MCHC 31.9 31.5 31.1 31 31.1 31.3
RDW 17 17.1 17 17.4 17.7 18
COURSE IN THE WARD20th Hospital day
Still with febrile episode (Tmax 37.9C)(+) cough productive of yellowish
phlegmMoxifloxacin 400mg once daily(-) abdominal pain Octreotide was
discontinued
COURSE IN THE WARD22nd Hospital day
Repeat Chest Xray Referred to Pulmonology serviceMoxifloxacin shifted to Piperacillin
Tazobactan 4.5g IV every 8 hoursHeparin was titrated and eventually
consumedWarfarin initially 5mg/tab daily
CHEST X-ray November 4, 2010
COURSE IN THE WARD27th Hospital day
Repeat PT showed INR 4.08 – Warfarin was discontinued
Afebrile with decreased episode of coughing
Repeat Chest Xray
CHEST X-ray November 9, 2010
COURSE IN THE WARD29th hospital day:
AfebrileDecrease episodes of coughingNo abdominal pain and with good
appetiteRepeat PT – INR 3.55Given last dose of antibiotics and was
discharged the following day.
FINAL DIAGNOSISAcute PancreatitisPortal Vein ThrombosisNon ST Elevation MIHospital Acquired PneumoniaPolycythemia VeraPost Cerebrovascular Accident with no
residual
FURTHER OUTPATIENT CAREUse of Myelosupressive therapy plus
phlebotomies with the intent of normalizing erythrocyte and platelet counts
Proven thrombotic complications warrant the use of long term anti-coagulation with warfarin.