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.