< Brief History >
A 55-year-old man suffered from intermittent abdominal pain
for one week. He is a HBV carrier and had been
well until 7 months ago when he suffered from left
upper quadrant pain and one episode of hematemesis
(about 400 ml fresh blood and blood clot).
Panendoscopy was performed at that time and showed gastric ulcer
without evidence malignancy. He was discharged after component therapy and proton
pump inhibitor use. However, intermittent abdominal pain over
epigastric area and left upper quadrant with fullness sensation
developed one week prior to this admission.
The pain occurred half an hour
after meals, persisted for about half an hour and was
relieved by stool passage. Black-colored stool was also noted recently. He
took some medication by himself without benefit in the
following days. The abdominal pain aggravated with
radiation to back on July 15, 2002 and he
was brought to the ER for further evaluation and
management.
On arrival, the temperature was
36℃, the pulse was 70 beats per minute, and the respiratory
rate was 16 per minute. The blood pressure was 120/70 mmHg. He
appeared acute ill-looking with knee-chest position because of
abdominal pain. The consciousness was clear. The conjuntivae
was pale and the sclerae was anicteric. There was neither
injected throat nor oral ulcer. The neck was supple without
lymphadenopathy or engorged jugular vein. The breath sounds
were clear. The heart sounds were regular without murmurs.
Inspection of the abdomen revealed superficial vein
engorgement. The auscultation revealed normoactive bowel
sounds. The abdomen was soft and flat. The liver was
impalpable but the spleen was enlarged with 4 finger breadth
below left costal margin. Epigastric and left upper quadrant
tenderness on deep palpation without rebound tenderness was
also noted. The rectal examination was normal except internal
hemorrhoids. There was no pitting edema over extremities.
< Laboratory Examination
>
1. Hemogram
|
WBC |
RBC |
Hb |
Hct |
MCV |
PLT |
Band |
Seg |
Eos |
Baso |
Mon |
Lym |
K/μL |
M/μL |
g/dL |
% |
FL |
K/μL |
% |
% |
% |
% |
% |
% |
July 15, 2002 |
18.55 |
5.12 |
11.5 |
35.1 |
68.6 |
1091 |
0 |
84 |
2 |
2 |
4 |
7 |
July 29, 2002 |
17.97 |
4.69 |
10.7 |
32.8 |
69.9 |
765 |
0 |
68 |
5 |
0 |
7 |
20 |
Aug. 1, 2002 |
13.37 |
4.85 |
11.1 |
34.6 |
71.3 |
941 |
0 |
72 |
4 |
3 |
5 |
16 |
Aug. 3, 2002 |
8.73 |
4.28 |
9.7 |
30.6 |
71.5 |
604 |
0 |
73 |
4 |
8 |
5 |
17
|
2. Blood chemistry
|
Albumin |
Globulin |
T-Bil |
D-Bil |
AST |
ALT |
ALP |
GGT |
LDH |
Glu AC |
g/dL |
g/dL |
mg/dL |
mg/dL |
U/L |
U/L |
U/L |
U/L |
U/L |
mg/dL |
July15, 2002 |
|
|
0.65 |
0.3 |
32 |
26 |
|
|
|
92 |
July 20, 2002 |
|
|
0.8 |
0.3 |
28 |
10 |
145 |
20 |
504 |
|
July 23, 2002 |
3.5 |
3.9 |
1.0 |
0.4 |
34 |
14 |
160 |
24 |
613 |
|
Aug.1 , 2002 |
3.8 |
4.0 |
0.6 |
0.3 |
41 |
20 |
165 |
29 |
650 |
|
|
BUN |
Cre |
UA |
Na |
K |
Ca |
TG |
T-CHO |
Amylase |
Lipase |
Mg/dL |
mg/dL |
mg/dL |
mmol/L |
mmol/L |
mmol/L |
mg/dL |
mg/dL |
U / L |
U / L |
July15, 002 |
27.8 |
1.59 |
|
137.5 |
4.59 |
|
|
|
115 |
260 |
July18,2002 |
|
|
|
|
|
|
|
|
69 |
125 |
July23,2002 |
19 |
0.8 |
3.4 |
135 |
4.9 |
2.25 |
81 |
118 |
|
|
Aug.1,2002 |
20 |
1.0 |
|
|
|
|
|
|
|
|
3. Stool exam ( O.B.): July
17,2002: +/-
;
July 24, 2002:
4+
4. Coagulation and DIC profile (4
unit of fresh frozen plasma have been transfused on July
19)
|
PT (sec) |
PTCont. (sec) |
PT INR |
PTT(sec) |
PTTCont. |
3P |
FDP |
D-Dimer |
Fibrinogen |
July 18, 2002 |
19.1 |
12.7 |
1.6 |
58.4 |
38.1 |
2+ |
10-20 |
1.69 |
|
July 23, 2002 |
16.5 |
10.8 |
1.4 |
54.8 |
35.1 |
Negative |
5-10 |
2.92 |
364 |
July 29, 2002 |
15.4 |
11.1 |
1.3 |
57.1 |
35.7 |
(Negative) |
(<10) |
(<0.5) |
(214 -474)
|
Protein S Ag : Total : 80 (80-180 ) |
Protein S Ag :Free : 45 ( 70-180 ) |
Protein S:Fun : 69 (68-155 ) |
Protein C: Fun: 78 ( 70-192 ) |
Antithrombin III: Ag: 97 ( ≧85 ) |
Antithrombin III: Fun: 79 ( ≧85
) |
< Course and Treatment
>
Leukocytosis, thrombocytosis, and slightly elevated lipase
level was noted. Acute pancreatitis was suspected initially,
so NPO, fluid resuscitation and pain control were given. His
pain relieved and follow-up lipase level decreased. Abdominal
echo revealed: 1. obliterated portal vein , 2. marked
splenomegaly , 3. dilated left intrahepatic duct, and 4.
cavernous transformation at hepatic hilum. (Fig.1)
Mesenteric ischemia has been suspected but abdominal CT showed
no evidence of thrombosis of main trunk of superior mesenteric
artery. Color Doppler ultrasound revealed obliteration of
portal vein with cavernous transformation. (Fig.2) MR
angiography revealed enhanced fibrotic band like lesion
surrounding extrahepatic portal vein. Minimal vessels could be
traced including splenic vein and superior mesenteric vein.
(Fig.3)
Thrombosis of splenic vein and superior mesenteric vein was
impressed. Panendoscopy showed varices over esophagus, stomach
and duodenum related to portal hypertension. (Fig 4
) Beta-blockers with propranolol were given for prophylaxis of
variceal bleeding. Tumor markers including alpha-fetoprotein, CEA, CA19-9 and
PSA have been checked and were all within normal limit. Due to the
persistent thrombocytosis without infection signs, bone marrow
studies with aspiration and biopsy was performed on July 26. The pathology
was compatible with chronic myeloproliferative disorder. Homocysteine level
,protein C, protein S, anti-thrombin III, anti-cardiolipin antibody
and anti-phospholipid antibody were checked and all were within
normal limit. Essential thrombocythemia was favored based on
the clinical and pathological findings. Therefore, hydroxyurea
was prescribed for control of platelet count. His abdominal pain
improved gradually and he was discharged on August
3, 2002 and was followed up at
hematologic outpatient clinic. Warfarin was added for the possible risk
of recurrent thrombosis since August
30.
< Case Analysis >
This is a case of essential thromocythemia ( ET ) , who
presented with symptoms and signs of portal vein thrombosis,
including splenomegaly and variecs bleeding. Essential
thrombocythemia may be asymptomatic ( 57 % ) and may present
with bleeding, arterial or venous thrombosis, or some
vasomotor symptoms ( headache, dizziness , erythromelalgia).
The treatment of ET is based on risk stratification, and the
high risk group refer to those are older than 60 years of age
or with a history of thrombosis or extreme thrombocytosis.
Treatment is necessary only in those with high risk and
several drugs have been showed to be effective. Hydroxyurea
reduced the thrombotic event from 45 % to 9 % but carries the
risk of leukemia. Anagrelide inhibit megakaryocyte maturation
in bone marrow and control thrombosis in more than 90 % of
patients. Aspirin is indicated in those with vasomotor
symptoms, transient ischemic accident or unstable angina. In
pregnant female patients, interferon α can be considered. In
patients with acute portal portal vein thrombosis, intravenous
heparization followed by oral anticoagulation recannalize more
than 80 % patients. In patients with chronic portal vein
thrombosis, treatment aims at control of portal hypertension
with endoscopic follow-up and propranol use. Chronic
anticoagulation also has been used to be helpful in reducing
the risk of splanchnic venous
infarction. |