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The 45-year-old business man had
been a patient with chronic hepatitis B for more than 25
years, for which he had been regularly followed at our
outpatient clinic and liver cirrhosis was diagnosed 3 years
earlier before this admission. He had been taking lamivudine
since May 2006 for elevated AST and ALT levels. His AST and
ALT level declined gradually. He also had diabetes mellitus
and hypertension with regular medical control. He did not
smoke or drink alcohol.
Five months prior to admission, he
began to develop progressive abdominal distension and weight
gain from 82 Kg to 92 Kg. He did not have fever or abdominal
pain, nor did he take Chinese herb or other medications. His
symptoms worsened rapidly recently and he visited our
emergency room for help.
On examination, his consciousness
was clear, height was 175 cm and weight was 92 kg. The body
temperature was 36.8°C, pulse rate 112 beats per minute, the
respirations 22 per minutes and the blood pressure was 130/80
mmHg. The conjunctivae were mildly pale, sclerae were
anicteric and the pupils were isocoric with prompt light
reflex. The neck was supple without a goiter, palpable masses,
engorged jugular veins or lymphadenopathy. Chest, heart, and
back were normal. The liver span was 8 cm and shifting
dullness was found on palpation. The extremities were freely
movable without edema. Neurological examinations were
unremarkable.
A chest X-ray
(Fig.1)
showed distended abdomen and bilateral lungs were poorly
expanded. An abdominal sonography (Fig.2
) showed liver cirrhosis with
patent portal vein, mild splenomegaly and massive ascites. He
was admitted for further evaluation
[Lab]
CBC/DC
| |
WBC
K/ul
|
RBC
M/ul |
HB
g/dL |
HCT
% |
MCV
fL
|
MCH
pg |
PLT
K/ul |
Seg
% |
Eos
% |
Baso
% |
Mono
% |
Lym
% |
|
2006/1/26 |
5.0 |
3.65 |
12.9 |
36.7 |
100.5 |
35.1 |
135 |
- |
- |
- |
- |
- |
|
2007/3/26 |
5.9 |
2.47 |
8.8 |
25.7 |
104 |
35.6 |
124 |
61.7 |
1.4 |
0.3 |
14.9 |
21.7 |
BCS + e-
| |
BUN
mg/dl |
Cre
mg/dl |
Na
mmol/L |
K
mmol/L |
Ca
mmol/L |
P
mg/dL |
AST
U/l |
ALT
U/l |
T-Bil
mg/dl
|
D-Bil
mg/dl
|
ALP
U/l |
GGT
U/l |
|
2006/10/26 |
15.3 |
1.1 |
136 |
4.2 |
2.31 |
- |
74 |
79 |
1.2 |
0.5 |
139 |
- |
|
2007/3/26 |
49.5 |
2.0 |
133 |
4.6 |
2.12 |
3.3 |
33 |
26 |
1.08 |
0.23 |
86 |
33 |
|
|
Alb
g/dl |
Glo
g/dl |
LDH
U/l |
Glucose
mg/dl
|
CHO
mg/dl |
TG
mg/dl
|
|
2006/10/26 |
3.0 |
5.3 |
494 |
134 |
- |
- |
|
2007/3/26 |
3.2 |
3.7 |
740 |
98 |
144 |
79 |
Tumor markers
|
|
CA19-9
U/ml
|
CEA
ng/ml
|
AFP
ng/ml |
PSA
ng/ml
|
|
2007/3/26 |
29.1 |
1.05 |
7.52 |
0.356
|
Coagulation
| |
PT
g/dl |
PT Cont
g/dl
|
INR
U/l |
PTT
mg/dl |
PTT Cont
mg/dl
|
|
2006/10/26 |
17.6 |
11.3 |
1.6 |
|
|
|
2007/3/26 |
17.5 |
11.7 |
1.5 |
38 |
37.6 |
Urine analysis
| |
Appear. |
Sp.
Gr. |
pH |
Protein |
Glu. |
Ketone |
O.B. |
Uro. |
Bil. |
Nitrite |
WBC |
RBC |
Epi. |
Cast |
|
|
* |
* |
* |
mg/dL |
g/dL |
* |
* |
EU/dL |
* |
* |
/HPF |
/HPF |
/HPF |
/LPF |
| 2007/3/26 |
Y;C |
1.019 |
5.0 |
100 |
- |
- |
- |
0.1 |
- |
- |
2-3 |
1-2 |
1-2 |
- |
Ascites analysis
| |
Appear. |
Sp.
Gr. |
Rivalta
test
|
Protein |
Alb |
TG |
CHO |
AFS |
Gram's |
RBC |
WBC |
Sediment |
|
|
* |
* |
* |
g/dL |
g/dL |
m/dL |
m/dL |
* |
* |
/ul |
/ul |
* |
|
2007/3/27 |
Bloody |
1.025 |
- |
4.3 |
1.5 |
100 |
54 |
- |
- |
100000 |
3500 |
Abnormal
cells
|
Pleural effusion analysis
| |
Appear. |
Sp.
Gr. |
Rivalta
test
|
Protein |
Glu |
TG |
LDH |
RBC |
WBC |
Sediment |
|
|
* |
* |
* |
g/dL |
m/dL |
m/dL |
U/ |
/ul |
/ul |
* |
|
2007/4/16 |
Y;T |
1.029 |
+ |
5.4 |
4 |
282 |
7736 |
10000 |
4000 |
Abnormal
cells |
Hepatitis
Profiles
HBsAg (+), Anti-HBs antibody (-), HBeAg
(-), Anti-HBe antibody (+), Anti-HBc antibody (+), Anti-HCV
(-)
[Course and
Treatment]
The
paracentesis showed bloody ascites (Fig.3)
and abnormal cells were found in the ascites (Fig.4
). The tumor markers,
including CEA、CA19-9、AFP、PSA, were all within normal limits.
The upper gastroinsteinal endoscopy and colonoscopy both
disclosed negative results. An abdominal MRI showed cirrhotic
change of the liver, splenomegaly and ascites. There was no
vascular obstruction or hepatic tumor. The cytology of the
ascites specimen reported presence of lymphoma cells. The
computed tomography from the neck to the pelvic region did not
show lymph node enlargement and mass lesions. Bone marrow
study was also normal. Surface marker study of the ascites
specimen was positive for CD138, which was restricted to
plasma cell of hematologic malignancy. Chromosome study of the
ascites specimen showed hyperploid, which confirmed the
existence of malignancy. The human herpes virus 8 of ascites
was positive and primary effusion lymphoma was diagnosed. He
was transferred to hematology ward for further
chemotherapy.
[Discussion]
肝硬化患者腹水增加通常需考慮以下幾點：(1)、鹽份或水份攝取過多，(2)、藥物順應性不佳，(3)、肝功能惡化，(4)、肝門靜脈栓塞，(5)、肝癌，(6)、感染。我們的病人經由病史及症狀並未發現鹽份或水份攝取過多、藥物順應性不佳的情形。而肝功能、血液學、及尿液、胸部X光可以初步排除感染及肝失償的可能。超音波檢查並未發現肝門靜脈栓塞或肝癌。因此我們必須考慮其他原因造成的腹水，如惡性腫瘤、腎臟疾病、心衰竭、胰臟炎等。
肝硬化患者的腹水檢查十分重要，經由腹水的顏色、蛋白量、紅血球、白血球及細胞型態分析，將有助於腹水形成原因的探討(Table
1.
)。一般肝硬化及腎臟疾病造成的腹水中蛋白質通常少25
g/L，而腫瘤、腹膜炎腹水中蛋白質較多；一般腹水中紅血球少有超過10000/uL，而腫瘤造成的腹水有20%的可能紅血球超過10000/uL。
原發性積液淋巴瘤(Primary effusion
lymphoma,
PEL)是一種罕見的淋巴瘤，第一例於1989年在AIDS患者上發現。回顧文獻報導，原發性積液淋巴瘤患者多為HIV感染者，少數為器官移植後的患者及老人。目前primary
effusion
lymphoma在WHO的分類為B細胞淋巴瘤的一種。不同於其他淋巴瘤多以腫瘤或淋巴結腫大表現，其特徵是含有淋巴瘤的體液在體腔內(如腹腔、胸腔、心包膜腔)異常聚積，因此也被稱為body
cavity-based lymphoma (BCBL)。患者的症狀也因為腫瘤細胞在不同體腔而有喘、腹脹等不同表現。
Primary effusion
lymphoma的細胞型態介於immunoblastic plasmcytoid lymphoma and
anaplastic large cell lymphoma之間，具有中度或高的nucleus/cytoplasm
(N/C) ratio，細胞核可以見到分裂複製。Primary effusion lymphoma的患者多有HHV-8
(Human herpes virus–8)感染，其成因可能和HHV-8感染相關。
Primary effusion
lymphoma的預後極差，目前治療方法尚未有定論。在AIDS患者中，有學者發現若使用高效能抗病毒藥物(highly
active antiretroviral therapy;
HAART)治療HIV感染，可能改善PEL患者的存活率。此外亦有學者使用傳統化學治療(如CHOP、EP、high dose
MTX)或嘗試interferon、anti-CD20來治療。但是無論治療與否，平均存活率多小於六個月。
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Case Discussion
繼續教育考題