< Brief history
>
A 51-year-old electrician was
referred to our hospital on May 14, 2005 with a two-week
history of intermittent high fever, rapidly progressive
jaundice and acute hepatomegaly. He was a habitual drinker but
was otherwise healthy. On April 28, 2005, he was exposed to
dust from bird nests when he repaired electric equipments and
circuitry at an abandoned factory. Three days later, high
fever and tea-colored urine developed. He took some
antipyretics but fever did not resolve. On May 11, he was
admitted to an outside hospital where laboratory tests
revealed a white cell count of 9.06 x 109/L, C-reactive protein 26.39 mg/dL,
serum aspartate aminotransferase 264 IU/L, serum alanine
aminotransferase 106 IU/L, total/direct bilirubin
(T-bil/d-bil) 5.6/4.5 mg/dL, alkaline phosphatase 490 IU/L,
and g -glutamyl-transferase 1054
IU/L. Physical examination revealed a non-tender liver
descending 2 cm below the costal margin. The abdominal
ultrasound showed hepatomegaly without an obvious focal lesion
or biliary tract dilatation. Ceftriaxone (1 g every 12 hours)
was given empirically, but spiking fever persisted. In the
following days, the jaundice rapidly progressed (T-bil/d-bil =
9.4/9.3 mg/dL), and the liver further enlarged
to 6 cm below the costal margin. The
urinalysis, chest X-ray, and the blood culture were negative.
Disseminated tuberculosis was suspected. However, antituberculous therapy was considered
too risky in the presence of rapidly progressive hepatic
failure. He was referred to our hospital.
At admission, initial temperature
was 39°C, the pulse rate was 100 beats per minute and regular,
and the respirations were 22 breaths per minute. The blood
pressure was 114/70 mmHg. The oxygen saturation was 96 percent
while he was breathing ambient air. His consciousness was
clear. The conjunctiva was not pale but the sclera was
markedly icteric. The pupils were isocoric and light reflex
was prompt. No oral ulcer or thrush was found. The neck was
supple without lymphadenopathy or jugular vein engorgement.
Spider angioma and a tattoo were noted over the anterior chest
wall. Breath sounds showed bilateral basal rales with
decreased breath sounds. Heart rhythm was regular without
audible murmurs. The abdomen was flat with engorged
superficial veins. There was no abdominal tenderness or
rebound tenderness. Shifting dullness was noted. The liver was
markedly enlarged, which was about 6cm below the costal
margin. There was mild pitting edema over legs without
petechia. No focal neurological sign was noted.
< Laboratory data
>
CBC/DC
Date |
RBC |
Hb |
Hct |
MCV |
MCH |
MCHC |
PLT |
WBC |
|
106/ul
|
g/dl |
% |
fL |
pg |
g/dl |
103/ul |
/ul |
940514 |
3.61 |
12.4 |
35.6 |
98.6 |
|
34.8 |
202 |
12430 |
940517 |
3.18 |
10.6 |
31.3 |
98.4 |
|
33.9 |
235 |
10160 |
940524 |
2.68 |
8.7 |
26.5 |
98.9 |
|
|
326 |
12030 |
940602 |
2.64 |
8.7 |
26.7 |
101.1 |
|
32.6 |
319 |
11460 |
940614 |
2.88 |
9.2 |
29.7 |
101.5 |
|
31 |
241 |
8670 |
Date |
Band |
Seg |
Eso |
Baso |
Mono |
Lymph |
940514 |
0 |
73 |
0 |
0 |
3 |
24 |
940517 |
1.1 |
88.2 |
0 |
0 |
1.1 |
9.6 |
940524 |
0 |
74.8 |
1.0 |
0.7 |
4.9 |
18.6 |
940602 |
13.5 |
67.7 |
0 |
0 |
11.5 |
7.3 |
940614 |
0 |
59 |
1.8 |
0.7 |
5.2 |
33.3 |
Biochemistry & Electrolytes
Date |
Alb/Glo |
Bil(T/D) |
BUN |
CRE |
GOT |
GPT |
ALKP |
GGT |
CRP |
|
g/dl |
mg/dl |
mg/dl |
mg/dl |
U/L |
U/L |
U/L |
U/L |
Mg/dl |
940514 |
2.53/- |
9.4/9.38 |
10.7 |
0.8 |
88 |
53 |
958 |
762 |
17.28 |
940516 |
|
10.50/7.78 |
15 |
0.7 |
75 |
37 |
813 |
506 |
17 |
940520 |
2.9/- |
10.04/7.38 |
|
|
84 |
32 |
934 |
492 |
11.36 |
940523 |
|
6.61/5.72 |
11.3 |
0.8 |
69 |
29 |
878 |
|
8.66 |
940602 |
3.04/4.1 |
3.24/2.23 |
|
|
71 |
32 |
379 |
430 |
2.54 |
940609 |
|
1.81/- |
8.5 |
0.5 |
34 |
29 |
508 |
251 |
0.59
|
Date |
CK |
LDH |
Na |
K |
Cl |
Ca |
P |
Mg |
|
m/dl |
U/L |
mM |
mM |
mM |
mM |
mM |
mM |
940514 |
39 |
911 |
132 |
4.5 |
109 |
1.81 |
1.04 |
|
940609 |
|
|
143 |
3.7 |
|
|
|
|
Urine
analysis
Date |
SG |
PH |
Protein |
Sugar |
Ketone |
Ob |
Bilirubin |
940514 |
1.020 |
6.5 |
+/- |
- |
+ |
- |
3+ |
Date |
WBC |
RBC |
Epi |
Cast |
Crystal |
Bac |
Urobil |
940514 |
0-1 |
0-1 |
0-1 |
- |
- |
- |
1.0 |
Coagulation
profile
Date |
PT (sec) |
INR |
PTT (sec) |
940514 |
16.1 |
1.3 |
43.5 |
Hepatitis
profile
- IgM-anti-HAV: negative
- HBsAg (EIA): negative
- Anti-hepatitis C virus: positive
- HCV RNA: undetectable
- IgM-anti-hepatitis E virus:
negative
Immunological studies
- Anti-nuclear antibody: 1:40
- RA factor (Nephelometry):<20
Serology test
- S.T.S (VDRL): negative, TPHA:
positive
- Leptospirosis: negative
- Anti-HIV: positive; Western Blot:
negative
- Proteus: OX19: 1:20 (negative)
- Proteus: OX2: 1:20 (negative)
- Proteus: OXK: 1:20 (negative)
- S. typhi: H: 1:20 (negative)
- S. typhi: O: 1:20 (negative)
- S. paratyphi A: 1:20 (negative)
- S. paratyphi B: 1:20 (negative)
- Q fever
檢驗項目 |
收件日期 |
發病日期 |
採檢日期 |
病日 |
檢體種類 |
IgG titer |
IgM titer |
判定 |
phase I |
phase II |
phase I |
phase II |
Q fever |
94/05/31 |
94/05/16 |
94/05/17 |
2 |
血清 |
2560 |
5120 |
160 |
>320 |
Q熱陽性 |
Q fever |
94/06/01 |
94/05/16 |
94/05/31 |
16 |
血清 |
2560 |
>5120* |
160 |
>320 |
Q熱陽性 |
Q fever |
94/07/19 |
94/05/16 |
94/07/15 |
61 |
血清 |
2560 |
20480 |
640 |
1280 |
Q熱陽性 |
*備註:雖未做到 end-point,但若以相同之螢光強度之稀釋倍數來看,phsae II之 IgG titer
有4倍以上之上升 (約640=5120)
Culture Negative results of
bacteria, mycobacterial, or fungal cultures of blood, pleural
effusion, bone marrow, liver tissue and
stool
< Image
>
CXR (5/14): bilateral blunting CP
angles; atelectasis of the right lower lung (Fig.1
) Chest, abdomen CT (5/14):
bilateral pleural effusions and atelectasis of bilateral lower
lungs; hepatosplenomegaly; no biliary tract dilation or
LAP
< Pathology >
Bone marrow (940516) (Fig.2 ) Diagnosis:
hypocellular marrow MICROSCOPIC FINDING There is
interstitial infiltration of plasma cells accounting for about
10 % of mononucler cells. Neither dysplasia, nor evidence of
excess of blast or myelofibrosis is noted. No evidence of
lymphoma/metastasis is noted. Serous degeneration is observed.
Focal lipogranuloma is seen. No acid-fast bacillus or PAS (+)
fungus is found. A reactive marrow is considered.
Liver biopsy (940519) (Fig.3)
(Fig.
4)
Diagnosis: granulomatous inflammation Microscopically,
it shows hepatic tissue with granulomatous inflammation mainly
in portal areas and mild hepatocytic damage. No multinuclear
giant cells, eosinophils, or doughnut granuloma is found.
Focal macrovasicular steatosis and Mallory bodies are seen,
which is compatible with an active drinker clinically.
< Course and treatment
>
After admission, cefotaxime (1 g
every six hours) and doxycycline (100 mg twice daily) were
given empirically. After coagulopathy (15.9 seconds, INR 1.32)
was corrected by infusion of fresh frozen plasma, transjugular
liver biopsy and bone marrow biopsy were performed. There were
multiple small fibrinoid-ring granulomas in liver parenchyma
and bone marrow. No acid-fast bacilli or periodic
acid-Schiff-positive fungi were found. Whole body computed
tomography demonstrated ascites and a significantly enlarged
liver without evidence of tumor or lymphadenopathy. Multiple
blood cultures revealed no growth. Anti-hepatitis C antibody
was positive. However, the ribonucleic acid of hepatitis C
virus was undetectable. Widal test, Weil-Felix test,
anti-nuclear antibody, serological tests for hepatitis A, B, E
virus, HIV, Leptospirosis, Cryptococcus, and
toxoplasmosis were non-reactive. Doxycycline therapy was
continued, and the jaundice and fever gradually improved over
a two-week period.
Two weeks after admission, high
levels of anti-Coxiella burnetii phase II IgM
(>1:320) and phase II IgG (1: 5120) were reported, and a
four-fold elevation of phase II IgG was noted in the paired
serum collected two weeks later. Echocardiography did not show
significant findings. The eventual diagnosis was acute Q fever
with granulomatous hepatitis and bone marrow involvement.
Doxycycline therapy was continued, and hydroxychloroquine (200
mg twice daily) was added to the regimen. After six weeks of
treatment, total bilirubin level decreased to 1.56 mg/dL, and
liver size decreased to 2 cm below the right costal margin. He
received regular follow-up. Doxycycline and hydroxychloroquine
were continued. There was no fever. Because of his frequent
drinking, serum aspartate aminotransferase was around 50 IU/L,
serum alanine aminotransferase was around 25 IU/L, alkaline
phosphatase level was around 350 IU/L and g-glutamyl-transferase
was around 800 IU/L. No hyperbilirubinemia was noted during
follow up.
|