Asian Cardiovasc Thorac Ann 2006;14:517-519
© 2006 Asia Publishing EXchange Ltd
Disseminated Intravascular Coagulopathy Caused by Intracardiac Mesothelioma
Tao Jin, MD,
Hai-Yong Wang, MD1,
Yi-Ming Ni, MD
Department of Cardiothoracic Surgery
1 Department of Oncology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China
For reprint information contact: Yiming Ni, MD Tel/fax: 86 571 8723 6645 Email: Ni_yiming{at}hotmail.com, Department of Cardiothoracic Surgery, The First Affiliated Hospital, College of Medicine, Zhejiang University, 79 Qingchun Road, Hangzhou, Zhejiang 310003, China.
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ABSTRACT
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We report a case of intracardiac mesothelioma complicated by chronic disseminated intravascular coagulopathy in a 50-year-old woman. Her symptoms were completely relieved by emergency resection of the tumor. Primary resection of the intracardiac mesothelioma is adequate treatment for this complicated surgical problem.
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INTRODUCTION
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Chronic disseminated intravascular coagulopathy (DIC) is an acquired syndrome representing a hypercoagulable state, hemorrhagic symptoms, and multiple organ failure. The clinical relevance of this syndrome is complicated as there is no established way of diagnosing DIC and it is difficult to distinguish whether the clinical features are attributable to the underlying disease or DIC.1 A wide spectrum of severity is seen, from chronic subclinical DIC with slow depletion of coagulation factors, to the acute fulminant presentation seen more often in the surgical setting.2 In this case, the diagnosis was challenging because the hematologic complication overwhelmed the clinical presentation.
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CASE REPORT
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A 50-year-old woman complained of palpitations and dyspnea on moderate exertion of one year duration. Five months before admission, she noticed petechial hemorrhage and widespread purpura, and the symptoms had become aggravated in the previous 20 days. She had undergone a hysterectomy for hysteromyoma 17 years before. Physical examination revealed widespread petechial hemorrhages, most of which were located on her back, buttocks, and all four limbs. A grade III/VI ejection systolic murmur was heard at the pulmonary auscultatory area. Her platelet level was 123K·µL–1.3 Red and white blood cell counts were within the normal ranges, and infection was ruled out because repeated bacterial cultures of blood and urine were negative. She had no history of significant liver disease, and her liver function tests were normal. Coagulation studies revealed: prothrombin time 17.7 sec (normal, 11.0–14.5 sec), activated partial thromboplastin time 59.8 sec (normal, 28.0–40.0 sec), thrombin time 18.2 sec (normal, 14.0–21.0 sec), and fibrinogen 1.4 g·L–1 (normal, 2.0–4.0 g·L–1). Serum chemistry showed elevated alkaline phosphatase and total bilirubin. Bone marrow aspiration revealed increased megakaryocytes in the hypocellular marrow. A diagnosis of DIC was made. Initial echocardiography demonstrated a 4.4 x 3.8 cm mass mimicking a myxoma in the enlarged right atrium, with its lower part extending 7 cm into the inferior vena cava. Coronal and horizontal magnetic resonance images showed a spherical hyperintense mass in the right atrium (Figure 1
). The patient was given a transfusion of 600 mL of fresh frozen plasma after admission.
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Figure 1. Magnetic resonance images showing a spherical hyperintense mass in the right atrium, extending to the inferior vena cava.
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On the 6th day after admission, she complained of chills and had a moderate fever. Her dyspnea quickly deteriorated. Coagulation studies showed: prothrombin time 27 sec, activated partial thromboplastin time 64.5 sec, fibrinogen 1.4 g·L–1. She underwent emergency surgical resection of the atrial tumor after preoperative treatment with prothrombin and fresh frozen plasma. A clamshell incision on the right 4th costa was performed to expose the tumor. Just before instituting extracorporeal circulation, the activated coagulation time was 160 sec, which was prolonged to > 999 sec after giving 100 mg heparin. Cardiopulmonary bypass was established by cannulation of the right femoral artery, femoral vein, and superior vena cava. The peduncle of the tumor originated from the confluence of the inferior vena cava and right atrium, and the diameter was approximately 5 cm. The tumor was solid, well enveloped, 12 cm in length, with the widest segment in the right atrium, and the diameter was 5 cm, so there was still 7 cm of tumor extending into the inferior vena cava (Figure 2). The tumor was successfully removed along with the peduncle and parts of the right atrial wall. Cardiopulmonary bypass lasted for 66 min. After neutralization with 130 mg protamine, the activated clotting time was reduced to 124 sec on stopping cardiopulmonary bypass. During the operation, 4 units of packed red cells, 4 units of fresh frozen plasma, and 15 units of platelets were transfused. Total drainage of blood from the left thoracic cavity and the mediastinum amounted to no more than 450 mL in the next 48 hours. Postoperatively, blood tests showed decreasing hypofibrinogenemia and falling D-dimer fibrin degradation products, and these continued to improve without requiring further blood products.
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Figure 2. Photomicroscopy of the right atrial tumor. The shuttle-shaped tumor cells were arranged in line with obvious atypia. Hematoxylin and eosin stain, original magnification x 400.
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The tumor weighed 135 gm. Grossly, it was firm and white-gray in color. Microscopically, there were cystic and tubular structures lined with single to double sheets of cuboid tumor cells (Figure 2
). The tumor cells stained positively with human bone marrow endothelial cell-1 antibody (Figure 3). A pathologic diagnosis of primary atrial and inferior vena caval malignant mesothelioma was made from these findings. As no laboratory or clinical findings showed that the tumor was related to the pleura or the pericardium, it was concluded that it was a primary atrial mesothelioma. The patient was discharged home on the 8th postoperative day, with no petechial hemorrhage or purpura on the body, and normal coagulation studies. Her coagulation results remained normal for more than 6 months and there was no recurrence of the tumor for 22 months until she died of bone metastasis and pathologic fracture as well as dyscrasia.
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Figure 3. Photomicrograph of a section of the atrial tumor showed diffuse cytoplasmic positivity with human bone marrow cell-1 antibody (original magnification x 400).
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DISCUSSION
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Malignant mesothelioma occurs most frequently in the pleura (88.8%), peritoneum (9.6%), or both (0.6%), but it has also been found in the pericardium (0.7%) and tunica vaginalis testis (0.2%).3 Mesotheliomas of the heart have been reported before but they represent < 5% of all malignant cardiac neoplasms, and DIC has also been reported with mesothelioma.4,5 A solid neoplasm has been shown to markedly alter the coagulation pathways and trigger any variety of DIC and its associated syndromes. Most tumors probably initiate DIC by expression of tissue factor on their cell surfaces, which in conjunction with factors VII–IX, can activate the intrinsic pathway proteins and the extrinsic pathway.2 The pathogenesis of coagulopathies induced by mesothelioma may be related to the release of endothelin, thromboplastin, and other attractant factors from the tumor tissue, with subsequent clot formation. Moreover, the tumor tissue may activate platelets directly, causing thrombocytopenia and shortening of the platelet survival time. The chronic deposition of platelets and fibrin on the tumor surface and concurrent fibrinolysis may be responsible for the consumption coagulopathy.1
Our patient’s clinical features of DIC deteriorated quickly after admission and were relieved shortly after resection of the tumor. The partial obstruction of intracardiac blood flow and subsequent clot formation might have exacerbated DIC. In these rare cases of DIC truly caused by atrial mesothelioma, operative resection may provide a cure. Diagnosis should prompt thorough investigation and close collaboration between surgeon and hematologist during the perioperative period. Fresh frozen plasma or prothrombin are good choices for perioperative treatment. The role of heparin in DIC conditions associated with hemorrhage remains more controversial. Since the formation of thrombin results in depletion of coagulation proteins and platelets, and subsequent bleeding, interruption of this process by inhibiting thrombin seems appropriate. However, heparin is a potent anticoagulant itself, and can cause bleeding.
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REFERENCES
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- Colman RW, Rubin RN. Disseminated intravascular coagulation due to malignancy. Semin Oncol 1990;17:172–86.[Medline]
- Hillerdal G. Malignant mesothelioma 1982: review of 4710 published cases [Review]. Br J Dis Chest 1983;77:321–43.[Medline]
- Hollins J, Lowman A, Assey ME. Primary pericardial mesothelioma: diagnostic and therapeutic challenges in management. South Med J 1988;81:537–8.[Medline]
- Liu L, Wang Z, Zhang X, Bu H, Qin Z. Pleural malignant mesothelioma complicated with disseminated intravascular coagulation. Hua Xi Yi Ke Da Xue Xue Bao 1993;24:426–8.[Medline]
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ABSTRACT
INTRODUCTION
