Transcript Polycythemia Vera by Caroline Armas

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
• Diagnosed with Polycythemia Vera (2006)
• Rx: Hydroxyurea (Litalir); phlebotomy as needed
• Epigastric pain, grade 10/10, nonradiating
• Rx: Ranitidine, Pantoprazole and
2 weeks Aluminum/Magnesium
• Persistence of abdominal pain
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
CBC
Hemoglobin
Hematocrit
WBC
Segmenters
Lymphocytes
Eosinophils
Basophils
Monocytes
Platelet count
MCV
MCH
MCHC
RDW
10.7
33.5
11.67
63
22
2
2
11
1267000
84.4
27
31.9
17
Amylase
48
Lipase
33.6
PFA October 16 2010
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
CBC
Hemoglobin
Hematocrit
WBC
Segmenters
Lymphocytes
Eosinophils
Basophils
Monocytes
Platelet count
MCV
MCH
MCHC
RDW
10.7
33.5
11.67
63
22
2
2
11
1267000
84.4
27
31.9
17
3rd HD
10.9
34.6
14.91
52
27
3
2
16
1342000
85.6
27
31.5
17.1
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
COURSE IN THE WARD
CBC
Hemoglobin
Hematocrit
WBC
Segmenters
Lymphocytes
Eosinophils
Basophils
Monocytes
Platelet count
MCV
MCH
MCHC
RDW
10.7
33.5
11.67
63
22
2
2
11
1267000
84.4
27
31.9
17
3rd HD
10.9
34.6
14.91
52
27
3
2
16
1342000
85.6
27
31.5
17.1
5th HD
11
35.4
14.84
55
18
13
2
12
978000
86.1
26.8
31.1
17
5th HD
AFP (8.6)
1.41
CA19-9 (0-39)
4.81
CEA (0-5.5)
0.92
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
CBC
3WARD
HD
5
COURSE
IN
THE
Hemoglobin
10.7
10.9
rd
Hematocrit
WBC
Segmenters
Lymphocytes
Eosinophils
Basophils
Monocytes
Platelet
count
MCV
MCH
MCHC
RDW
th
33.5
11.67
63
22
2
2
11
34.6
14.91
52
27
3
2
16
HD
11
35.4
14.84
55
18
13
2
12
1267000
84.4
27
31.9
17
1342000
85.6
27
31.5
17.1
978000
86.1
26.8
31.1
17
7th HD
11.3
36.4
13.17
56
27
8
934000
86.7
26.9
31
17.4
9
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 polycythemia vera
 Thrombosis is a frequent complication in persons with
Polycythemia vera
 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
Platelet count
1600000
1400000
1200000
1000000
800000
Platelet count
600000
400000
200000
0
Day 1
Day2
Day4
Day 5
Day 6
Day 8
Day 10
Day 11
COURSE IN THE WARD
 12th Hospital day
 Therapeutic platelet reduction  Repeat CBC
COURSE IN THE WARD
CBC
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
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
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
31.9
31.5
31.1
31
31.1
17
17.1
17
17.4
17.7
WBC
Platelet count
MCHC
RDW
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
ECG
Probable old inferior wall MI
Nonspecific 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
Trop I
CPK-MB
73
0.3
1.5
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
CHEST X-ray October 31, 2010
COURSE IN THE WARD
CBC
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
11.67
14.91
14.84
13.17
8.54
9.16
Segmenters
63
52
55
56
55
70
Lymphocytes
22
27
18
27
24
22
Eosinophils
2
3
13
8
5
1
Basophils
2
2
2
Monocytes
Platelet
count
11
16
12
9
6
6
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
31.9
31.5
31.1
31
31.1
31.3
17
17.1
17
17.4
17.7
18
WBC
MCHC
RDW
1
COURSE IN THE WARD
 20th Hospital day
 Still with febrile episode (Tmax 37.9C)
 (+) cough productive of yellowish phlegm
 Moxifloxacin 400mg once daily
 (-) abdominal pain  Octreotide was
discontinued
COURSE IN THE WARD
 22nd Hospital day
 Repeat Chest Xray
 Referred to Pulmonology service
 Moxifloxacin shifted to Piperacillin Tazobactan
4.5g IV every 8 hours
 Heparin was titrated and eventually consumed
 Warfarin initially 5mg/tab daily
CHEST X-ray November 4, 2010
COURSE IN THE WARD
 27th 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 WARD
 29th hospital day:
 Afebrile
 Decrease episodes of coughing
 No abdominal pain and with good appetite
 Repeat PT – INR 3.55
 Given last dose of antibiotics and was
discharged the following day.
FINAL DIAGNOSIS
 Acute Pancreatitis
 Portal Vein Thrombosis
 Non ST Elevation MI
 Hospital Acquired Pneumonia
 Polycythemia Vera
 Post Cerebrovascular Accident with no residual
FURTHER OUTPATIENT CARE
 Use 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.