A 53-year-old male patient complained weakness and myalgia with rashes for a half month. Skin rashes spread from face, scalp, chest, back to hips, with itches and increased after sunshine. The generalized fatigue and weakness deteriorated, accompanied with dyspnea, dysmasesis, and dysphagia. Physical examination showed muscle strength testing of neck flexors and proximal upper and lower limbs was 3/5. Erythematous patches over the scalp, minimal heliotrope rash, fuscous rashes over his chest, back and hips was discovered during skin exam. He had a history of perianal abscess surgery 5 years ago, gastrointestinal polyp resection 3 months ago, and left hip replacement due to avascular necrosis of femoral head one month ago.
Laboratory tests showed minor positive ANA 1:100 speckled pattern, myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs), extractable nuclear antigen (ENA), and anti-neutrophil cytoplasmic antibodies (ANCAs) were all negative. Other laboratory findings included extremely elevated creatine kinase 8875 U/L (ref ≤ 190), with increased ALT 125 U/L, AST 413 U/L, LDH 940 U/L, ferritin 2573.8 ug/L (ref 30-400), CEA 10.70 ng/ml (ref ≤5.0), IL-6 8.41 pg/ml (ref 0.1-2.9) and decreased C3 0.73 g/L (ref 0.8-1.8). His hemoglobin (HB) (136 g/L) and platelet count were normal. Basic coagulation testing revealed elevated D Dimer 5.91 ug/ml FEU (ref <0.5) with normal PT, APTT, fibrinogen. Magnetic resonance imaging (MRI) detected diffuse edema in bilateral thigh muscle groups and intermuscular fascia, and subcutaneous soft tissue consistent with inflammatory myopathy (Fig. 1). Electroneuromyography (EMG) demonstrated electrophysiological features of multiple myogenic damage. Positron emission tomography-computed tomography (PET-CT) was performed given the unusually high level of CA19-9 (400.60 U/ml) and CEA (10.7 ng/ml). PET-CT revealed the muscle metabolism was increased (both upper extremities, right pectoral muscles, bilateral scapular muscles were the most prominent and swollen), and the retroperitoneal lymph nodes increased in size and metabolism. Based on these findings, he was diagnosed as DM.
On admission, he was started on pulse methylprednisolone 200mg per day for 5 days, since he was treated with prednisone 40 mg per day without improvement before admission, plasmapheresis for one time, and prophylactic low molecular weight heparin (LMWH) 3200 IU per day. On day 3, he presented severe pain in the right arm. Swollen right arm and hand was found with purple bruises. He also felt pain on both shoulders and right chest. Ultrasound showed the muscle fibers in the right upper extremity were swollen and liquid dark areas were seen locally (consider hematoma). MRI showed extensive edema of the right shoulder girdle muscle and muscle groups of the upper arm; Edema of subcutaneous soft tissue of shoulder and upper arm (Fig. 2a, 2b). APTT was slightly prolonged (50.2 s), with decreased fibrinogen (1.33 g/L), and HB dropped from 136 g/L to 82 g/L, platelet decreased from 125 × 109/L to 67 × 109/L (Table 1). Emergent angiography confirmed a small branch of the brachial artery bleeding (Fig. 2c). Since embolism therapy and surgery was impropriate in that situation, a conservative pressure dressing treatment was performed (Additional file 1). LMWH was stopped and transfusion with red blood cells and fresh frozen plasma was supplied with hemostatic therapy. The bleeding was controlled after therapy. He remained stable in 10 days. Due to critical dysphagia and weakness, pulse IVIG 20 g per day for 5 days was given. Considering increased D-dimer (10.76 μg/ml) and prolonged recumbency increased the risk of VET, so prophylactic LMWH was started again. The weakness wasn’t worse, while CK level decreased to 1831 U/L. However, 4 days later, he complained of abdominal distention, gradually right thigh pain. The MRI scan showed hematoma formation in the right psoas major muscle, and CT scan showed bilateral swelling of the iliopsoas muscle and new-onset hematoma formation in the right psoas major muscle (Fig. 3). Hemostatic drug was immediately administrated, together with blood transfusion and supportive treatment, the hematoma did not expand.
CT image showed a lamellar slightly dense shadow of the right psoas major muscle mass.
Table 1 Characteristics of laboratory examinations
|
Parameter
|
Day1
|
Day4
|
Day8
|
Day16
|
Day18
|
Day22
|
Reference value range
|
White cell count as × 109/L
|
5.64
|
9.34
|
8.11
|
7.05
|
8.32
|
3.92
|
(3.5-9.5)
|
Platelets as × 109/L
|
125
|
67
|
80
|
111
|
98
|
225
|
(125-350)
|
Hemoglobin in g/L
|
136
|
82
|
103
|
90
|
78
|
91
|
(130-175)
|
Creatine kinase in U/L
|
8875
|
8270
|
10800
|
2448
|
1831
|
1702
|
(≤ 190)
|
ALT in U/L
|
125
|
140
|
119
|
72
|
56
|
41
|
(≤ 41)
|
AST in U/L
|
413
|
345
|
377
|
164
|
133
|
144
|
(≤ 41)
|
LDH in U/L
|
940
|
839
|
892
|
631
|
599
|
663
|
(135-225)
|
Ferritin in ug/L
|
2573.8
|
1941
|
1648.9
|
1756.8
|
1831
|
1618.2
|
(30-400)
|
myoglobin
|
1200
|
1200
|
1200
|
676
|
886.9
|
521.7
|
(≤ 154.9)
|
Urea nitrogen in mmol/L
|
11.37
|
7.7
|
7.1
|
9.1
|
12.9
|
5.7
|
(3.1-8.0)
|
Creatinine in μmol/L
|
73
|
63
|
60
|
77
|
73
|
47
|
(59-104)
|
EGFR in mL/min/1.73 m2
|
100.7
|
107
|
109.1
|
98.5
|
100.7
|
120.6
|
(> 90)
|
Fibrinogen in g/L
|
3.01
|
1.33
|
2.07
|
3.25
|
3.13
|
2.5
|
(2-4)
|
D-Dimer in μg/mL FEU
|
5.91
|
1.33
|
7.53
|
10.76
|
7.44
|
9.33
|
(< 0.5)
|
TT s
|
20.4
|
37.2
|
18
|
19.8
|
18.2
|
16.5
|
(14-19)
|
PT s
|
12.5
|
12.8
|
13.1
|
13.3
|
12.5
|
13.3
|
(11.5-14.5)
|
APTT s
|
37.4
|
50.2
|
38.5
|
50.1
|
42.6
|
38.8
|
(29-42)
|
In addition to the coagulation routine, the patient's coagulation factors, protein C and protein S were also tested (Additional file 1). Furthermore, special tests about bleeding were carried out, include coagulation and fibrinolysis. Fibrin degradation products (FDPs) elevated to 5.5 μg/ml, with increased thrombin-antithrombin III complex (TAT) 10.4 ng/ml (ref < 4), plasmin-α2-plasmininhibitor complex (PIC) 3.16 μg/ml (ref < 0.8) and tissue type plasminogen activator inhibitor complex (t-PAIC) 41.9 ng/ml (ref < 17). These results indicated that he has hyperfibrinolysis, perhaps progress to disseminated intravascular hemolytic (DIC). Fortunately, he was rescued after transfusion and hemostatic therapy with termination of anticoagulant.
After active treatment, abdominal distension has not been relieved. Electronic gastroscopy found gastric sinus ulcer, histopathology of biopsy showed signet-ring cell carcinoma (Fig. 4). We rechecked CA19-9 > 1000 U/ml and CEA 24.84 ng/ml. Gastric signet-ring cell carcinoma was diagnosed, and he started chemotherapy (paclitaxel 300 mg).