Spontaneous intramuscular hemorrhage in cancer-associated dermatomyositis: a case and literature review

Abstract

Background Spontaneous intramuscular hemorrhage (SIH) is a rare but life-threatening complication of dermatomyositis (DM). The pathogenetic mechanism and management of intramuscular hematoma in these patients remains unclear. Here we discuss a case of recurrent hemorrhage in a patient with cancer-associated DM, and review the relevant literature for timely diagnosis and treatment. Case presentation A 53-year-old male patient presented with rashes, muscle weakness, and dysphagia and was diagnosed with DM. During treatment, he developed SIH of the arm and right psoas major muscle successively. MRI showed extensive edema of the right shoulder girdle muscle and muscle groups of the upper arm. During the second SIH, a CT scan showed new-onset hematoma formation in the right psoas major muscle. The detection of D-dimer, thrombin-antithrombin III complex (TAT), plasmin-α2-plasmininhibitor complex (PIC) and tissue plasminogen activator-inhibitor complex (t-PAIC) indicated predominant hyperfibrinolysis over thrombosis. Blood transfusion and supportive treatment were immediately performed, and the hematoma did not expand. However, his abdominal distension was not relieved after active treatment. Further electronic gastroscopy discovered gastric sinus ulcers, and histopathology of the biopsy confirmed signet-ring cell carcinoma. Conclusions Although patients with cancer-associated DM have an increased risk of thrombosis, prophylactic anticoagulation therapy needs deliberate consideration. It is important to monitor the coagulation parameters dynamically during anticoagulation therapy. Especially when the level of D-dimer is high, and it is uncertain whether the patient is in a state of thrombosis or hyperfibrinolysis, the detection of TAT, PIC, t-PAIC can help to determine whether to initiate anticoagulation therapy.

Evaluation of the frequency and risk factors of spontaneous intramuscular hemorrhage associated with dermatomyositis

“…Publications containing non-DM inflammatory myositis cases, cases without spontaneous bleeding, poster presentations, publications containing under-18 cases, and articles not written in English were excluded from analysis. Including 23 cases that met the inclusion criteria and our own case, total of 24 SIH patients were analyzed [5][6][7][8][9][10][11][12][13][14][15][16][17][18]. We compared the data of these patients with our 41 DM patients without SIH.…”

“…From a diagnostic point of view, the most commonly used diagnostic method in patients was CT (87.5%). Magnetic resonance imaging (MRI) and USG were also used [5][6][7][8][9][10][11][12][13][14][15][16][17][18]. When looking at the literature, angiography is used for diagnostic purposes, especially in patients with severe bleeding [5,14,16].…”

“…Magnetic resonance imaging (MRI) and USG were also used [5][6][7][8][9][10][11][12][13][14][15][16][17][18]. When looking at the literature, angiography is used for diagnostic purposes, especially in patients with severe bleeding [5,14,16]. In patients with subscriber hemorrhage, angiography, both diagnostic and embolization-wise, may be a suitable option [5,14].…”

“…Pneumomediastinum is a rare but still known complication in the course of DM, while DM-related spontaneous intramuscular bleeding (SIH) is very rarely defined and is not very well known among rheumatologists. So far only case reports have been identified in the literature [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]. The frequency and risk factors of DM-related SIH are unknown.…”

“…Detailed hemorrhage-related characteristics of patients with spontaneous intramuscular hemorrhage (together with our patient and cases in the literature)[5][6][7][8][9][10][11][12][13][14][15][16][17][18] …”