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Disseminated intravascular coagulation presenting as perioral haemorrhage
Disseminated intravascular coagulation presenting as perioral haemorrhage P. McLaughlin,
S. Chcn, J. G. Phillips
Depurtment of Oral and Mrrxillofacial Surgery, Glun Chyd Hospitul, Bodehvyddan, Chyd
SUMMARY. A case of disseminated intravascular coagulation presenting with lower lip haemorrhage is described. The coagulopathy was secondary to an undiagnosed prostatic adenocarcinoma. The management of such haemorrhage is outlined.
haemostasis. The patient also complained of worsening pain in his hip. After resuturing the lip, complete blood count, coagulation screen and pelvic radiographs were ordered. Initial results showed a normal blood picture, however, the prothrombin time (PT) was 24/15 s: the activated partial thromboplastin time (APPT) 59/46 s, the thrombin time (TT) IS/l5 s, and the fibrinogen level less than 0.1 g/l (normal 1.773.4 g/l). The pelvic radiographs showed a pathological fracture of the left pubic ramus. Further systemic questioning revealed nothing further of note, and specifically no urological symptoms. However, on digital rectal examination an irregularly enlarged prostate was found. A provisional diagnosis of DIC secondary to metastatic prostatic malignancy was made and the patient admitted to the hospital. Over the next 14 days a total of 21 units of fresh frozen plasma and 70 units of cryoprccipitate were required to bring the coagulopathy under control, and arrest recurrent haemorrhagc from the lip. During this time the patient’s haemoglobin level fell from 14.1 g/d1 to 10.3 gjdl. A prostatic biopsy confirmed adenocarcinoma. An isotope bone scan showed increased uptake in the pelvis and sternum consistent with metastatic disease. The patient underwent a transurcthral resection of the prostate, and was placed on long term therapy with the antigonadotrophin goserelin, on a monthly basis. He remains alive and well to date.
INTRODUCTION Disseminated intravascular coagulation (DIC) is a systemic disturbance of hacmostasis caused by an inappropriate production of thrombin. Thrombin catalyses the activation and consequent consumption of fibrinogen and other coagulant proteins as well as the production of fibrin thrombi. Following this is the secondary activation of the plasmin system which breaks down fibrin into its degradation products. Hence DIC is also termed a consumptive coagulopa. thy, or the defibrination syndrome. Clinical presentation of DIC is variable but is broadly related to whether circulatory obstruction or bleeding predominates and where the intravascular coagulation occurs. The spectrum ranges from asymptomatic chronic compensated DIG, through multisystem organ failure, to an acute fulminant bleeding diathesis. Both coagulation and fibrinolysis are activated by the same original trigger. Such triggers include direct action of thromboplastins from inflamed, necrotic or neoplastic cells, toxins of venomous animals: or indirect action of bacterial endotoxin, immune complexes, particulate agents, and lipids. The trigger may also arise from blood vessel cndothelium damaged either by physical agents or by ischaemia and acidosis as in hypovolaemic shock. A case is described of a patient with DlC presenting initially to a department of Oral and Maxillofdcial surgery.
DISCUSSION DIC is not uncommonly seen in obstetric and surgical practice, and the association of coagulopathies with carcinoma of the prostate is well recognised.’ There are few reports of DIC complicating oral or maxillofacial surgical procedures, yet prostatic carcinoma is common in the elderly male population, with around 8000 new cases presenting annually in the UK. Yurthcrmore, of all cases found at post mortem only 30% will have caused symptoms during life.2 A study of patients with chronic DIC found carcinoma of the prostate as the underlying cause in 25%~~ Prostatic cancer cells probably initiate DlC by expressing a cell surface protease that directly activates the extrinsic coagulation pathway.4 A case of fatal haemorrhage after dental extraction due to DIC secondary to an undiagnosed prostatic
Case report A 62-year-old male attended the Accident and Emergency department of Ysbyty Gwynedd Hospital, Bangor having fallen from a step ladder. He complained of a painful left hip and had sustained a 2 cm laceration to his lower lip. Clinical examination revealed superficial bruising only, and he was referred to the oral and maxillofacial unit for suturing of his lower lip. This was accomplished using local anaesthetic and the patient was discharged. The patient returned 24 h later with continued bleeding from the lip wound. Re-exploration failed to find a discrete vessel but considerable difficulty was encountered achieving 94
Disseminated
carcinoma has been reported.’ Catrambone and Pfcffer describe significant haemorrhage after biopsy of a prostatic tumour that had metastasised to the mandibular condyle.” A single case of DIC secondary to abruptio placenta, which resulted in massive facial haemorrhage following a road traflic accident has also been reported.’ Although there are a wide range of causes of DIG, treatment follows a structured approach. Where possiblc the trigger event is eliminated, using, for example, antibiotics in septicacmia, or blood volume replacement in shock. Hormonal manipulation has been successful in reversing DTC in metastatic prostate carcinoma.’ DIC is more likely to occur in patients who are in shock or compromised such that tissue perfusion and oxygenation arc reduced. Correction of blood volume, cardiac output, blood pressure and haemoglobin levels, may prevent DIC from becoming fulminant in such situations. The main modality of treatment is the replacement of consumed clotting factors. Fresh frozen plasma (FFP) contains all coagulation factors and their inhibitors. Cryoprecipitatc is a factor concentrate including fibrinogen, von Willibrand’s factor and factor XIII. Bleeding due to DIC is treated initially with infusion of 1 litre of FFP and 10 units of cryoprecipitate. Close clinical and laboratory monitoring is carried out because further infusions may bc required every 4 h or less to control haemorrhage. Platelet transfusions are needed in severe thrombocytopenia or when the platelet count is falling rapidly. The inhibition of proteolytic pathways using heparin is controversial: and is only generally used in those patients with DIC demonstrating microvascular thrombosis, such as peripheral gangrene.’
intravascular
coagulation
presentin
as perioral
hacmorrhage
95
history and examination, coupled with a low threshold for coagulation screening, is advisable. References 1. Grimon D. Turnbull D, Lohmann RC. Carcinoma of the prostate presenting as acute disscminatcd intravascular coaalation. CMAJ 1986: 135: 775-776. 2. Cusvhicri A, Giles GR, Moossa AR. Essential Surgical Practice. 2nd cd. Bristol: Wright, 1988: 1377-1378. coagulation; clinical 3. Straub PW. Chronic intravascular spectrum and diagnostic criteria, with special emphasis on metabolism. distribution and localisation of I”‘-fibrinogen. Acta Mcd Stand 1971: suppl 526: I 79. 4. Colman RW. Rubin NR. Disseminated intravascular coagulation due to malignancy. Semin Oncol 1990; 17: 172- 186. 5. McKechnie J. Prostatic carcinoma prcscnting as a haemorrhagic diathesis after dental extraction. Br Dent J 1989; 166: 295 296. RJ, Pfelfer KC. Significant postoperative 6. Catrdmbone hacmorrhage following biopsy of a prostatic tumour metastatic to the mandibular condyle. J Oral Maxillofac Surg 1990; 48: 858. 861. 7. Samman X. Disseminated intravascular coagulation and facial injury. Brit J Oral Maxillofac Surg 1984; 22: 295-300. 8. Goldberg SL, Fenstcr HN, Perler Z ef al. Disseminated intravascular coagulation in carcinoma of the prostate: role of estrogen therapy. Crology 1983: 22: I30 132. intravascular 9. Kesteven P. Saunders P. Disscmiuated coagulation. Care of the Critically 111;9: 22-27.
The Authors Philip 4IcLoughlin, FRCS, FDSRCS Registrar Simon Chen, BDS Senior House Oficcr John G. Phillips, MI3 RS, FDSRCS, FFDRCSI Consultant Department of Oral and Maxillofacial Surgery Glan Clwyd Hospital Bodelwyddan Rhyl Clwyd
CONCLUSION
Correspondence and requests for offprints to Mr Philip McLaughlin, Maxillofacial Unit, Queen Victoria Hospital, Road. East Grinstead, West Sussex, RH19 3DZ
The reported cast highlights a significant potential for DIC in the elderly male population. A full clinical
Paper received I I March Accepted 21 June 1993
1993
Holtye
S. Chcn, J. G. Phillips
Depurtment of Oral and Mrrxillofacial Surgery, Glun Chyd Hospitul, Bodehvyddan, Chyd
SUMMARY. A case of disseminated intravascular coagulation presenting with lower lip haemorrhage is described. The coagulopathy was secondary to an undiagnosed prostatic adenocarcinoma. The management of such haemorrhage is outlined.
haemostasis. The patient also complained of worsening pain in his hip. After resuturing the lip, complete blood count, coagulation screen and pelvic radiographs were ordered. Initial results showed a normal blood picture, however, the prothrombin time (PT) was 24/15 s: the activated partial thromboplastin time (APPT) 59/46 s, the thrombin time (TT) IS/l5 s, and the fibrinogen level less than 0.1 g/l (normal 1.773.4 g/l). The pelvic radiographs showed a pathological fracture of the left pubic ramus. Further systemic questioning revealed nothing further of note, and specifically no urological symptoms. However, on digital rectal examination an irregularly enlarged prostate was found. A provisional diagnosis of DIC secondary to metastatic prostatic malignancy was made and the patient admitted to the hospital. Over the next 14 days a total of 21 units of fresh frozen plasma and 70 units of cryoprccipitate were required to bring the coagulopathy under control, and arrest recurrent haemorrhagc from the lip. During this time the patient’s haemoglobin level fell from 14.1 g/d1 to 10.3 gjdl. A prostatic biopsy confirmed adenocarcinoma. An isotope bone scan showed increased uptake in the pelvis and sternum consistent with metastatic disease. The patient underwent a transurcthral resection of the prostate, and was placed on long term therapy with the antigonadotrophin goserelin, on a monthly basis. He remains alive and well to date.
INTRODUCTION Disseminated intravascular coagulation (DIC) is a systemic disturbance of hacmostasis caused by an inappropriate production of thrombin. Thrombin catalyses the activation and consequent consumption of fibrinogen and other coagulant proteins as well as the production of fibrin thrombi. Following this is the secondary activation of the plasmin system which breaks down fibrin into its degradation products. Hence DIC is also termed a consumptive coagulopa. thy, or the defibrination syndrome. Clinical presentation of DIC is variable but is broadly related to whether circulatory obstruction or bleeding predominates and where the intravascular coagulation occurs. The spectrum ranges from asymptomatic chronic compensated DIG, through multisystem organ failure, to an acute fulminant bleeding diathesis. Both coagulation and fibrinolysis are activated by the same original trigger. Such triggers include direct action of thromboplastins from inflamed, necrotic or neoplastic cells, toxins of venomous animals: or indirect action of bacterial endotoxin, immune complexes, particulate agents, and lipids. The trigger may also arise from blood vessel cndothelium damaged either by physical agents or by ischaemia and acidosis as in hypovolaemic shock. A case is described of a patient with DlC presenting initially to a department of Oral and Maxillofdcial surgery.
DISCUSSION DIC is not uncommonly seen in obstetric and surgical practice, and the association of coagulopathies with carcinoma of the prostate is well recognised.’ There are few reports of DIC complicating oral or maxillofacial surgical procedures, yet prostatic carcinoma is common in the elderly male population, with around 8000 new cases presenting annually in the UK. Yurthcrmore, of all cases found at post mortem only 30% will have caused symptoms during life.2 A study of patients with chronic DIC found carcinoma of the prostate as the underlying cause in 25%~~ Prostatic cancer cells probably initiate DlC by expressing a cell surface protease that directly activates the extrinsic coagulation pathway.4 A case of fatal haemorrhage after dental extraction due to DIC secondary to an undiagnosed prostatic
Case report A 62-year-old male attended the Accident and Emergency department of Ysbyty Gwynedd Hospital, Bangor having fallen from a step ladder. He complained of a painful left hip and had sustained a 2 cm laceration to his lower lip. Clinical examination revealed superficial bruising only, and he was referred to the oral and maxillofacial unit for suturing of his lower lip. This was accomplished using local anaesthetic and the patient was discharged. The patient returned 24 h later with continued bleeding from the lip wound. Re-exploration failed to find a discrete vessel but considerable difficulty was encountered achieving 94
Disseminated
carcinoma has been reported.’ Catrambone and Pfcffer describe significant haemorrhage after biopsy of a prostatic tumour that had metastasised to the mandibular condyle.” A single case of DIC secondary to abruptio placenta, which resulted in massive facial haemorrhage following a road traflic accident has also been reported.’ Although there are a wide range of causes of DIG, treatment follows a structured approach. Where possiblc the trigger event is eliminated, using, for example, antibiotics in septicacmia, or blood volume replacement in shock. Hormonal manipulation has been successful in reversing DTC in metastatic prostate carcinoma.’ DIC is more likely to occur in patients who are in shock or compromised such that tissue perfusion and oxygenation arc reduced. Correction of blood volume, cardiac output, blood pressure and haemoglobin levels, may prevent DIC from becoming fulminant in such situations. The main modality of treatment is the replacement of consumed clotting factors. Fresh frozen plasma (FFP) contains all coagulation factors and their inhibitors. Cryoprecipitatc is a factor concentrate including fibrinogen, von Willibrand’s factor and factor XIII. Bleeding due to DIC is treated initially with infusion of 1 litre of FFP and 10 units of cryoprecipitate. Close clinical and laboratory monitoring is carried out because further infusions may bc required every 4 h or less to control haemorrhage. Platelet transfusions are needed in severe thrombocytopenia or when the platelet count is falling rapidly. The inhibition of proteolytic pathways using heparin is controversial: and is only generally used in those patients with DIC demonstrating microvascular thrombosis, such as peripheral gangrene.’
intravascular
coagulation
presentin
as perioral
hacmorrhage
95
history and examination, coupled with a low threshold for coagulation screening, is advisable. References 1. Grimon D. Turnbull D, Lohmann RC. Carcinoma of the prostate presenting as acute disscminatcd intravascular coaalation. CMAJ 1986: 135: 775-776. 2. Cusvhicri A, Giles GR, Moossa AR. Essential Surgical Practice. 2nd cd. Bristol: Wright, 1988: 1377-1378. coagulation; clinical 3. Straub PW. Chronic intravascular spectrum and diagnostic criteria, with special emphasis on metabolism. distribution and localisation of I”‘-fibrinogen. Acta Mcd Stand 1971: suppl 526: I 79. 4. Colman RW. Rubin NR. Disseminated intravascular coagulation due to malignancy. Semin Oncol 1990; 17: 172- 186. 5. McKechnie J. Prostatic carcinoma prcscnting as a haemorrhagic diathesis after dental extraction. Br Dent J 1989; 166: 295 296. RJ, Pfelfer KC. Significant postoperative 6. Catrdmbone hacmorrhage following biopsy of a prostatic tumour metastatic to the mandibular condyle. J Oral Maxillofac Surg 1990; 48: 858. 861. 7. Samman X. Disseminated intravascular coagulation and facial injury. Brit J Oral Maxillofac Surg 1984; 22: 295-300. 8. Goldberg SL, Fenstcr HN, Perler Z ef al. Disseminated intravascular coagulation in carcinoma of the prostate: role of estrogen therapy. Crology 1983: 22: I30 132. intravascular 9. Kesteven P. Saunders P. Disscmiuated coagulation. Care of the Critically 111;9: 22-27.
The Authors Philip 4IcLoughlin, FRCS, FDSRCS Registrar Simon Chen, BDS Senior House Oficcr John G. Phillips, MI3 RS, FDSRCS, FFDRCSI Consultant Department of Oral and Maxillofacial Surgery Glan Clwyd Hospital Bodelwyddan Rhyl Clwyd
CONCLUSION
Correspondence and requests for offprints to Mr Philip McLaughlin, Maxillofacial Unit, Queen Victoria Hospital, Road. East Grinstead, West Sussex, RH19 3DZ
The reported cast highlights a significant potential for DIC in the elderly male population. A full clinical
Paper received I I March Accepted 21 June 1993
1993
Holtye