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A paraneoplastic syndrome is a phenomenon or complex of symptoms that can occur with malignancy, without this being the result of tumour cells in the affected area. In this article, we describe the following paraneoplastic syndromes: thrombophlebitis migrans, clubbing, pemphigus, acanthosis nigricans, blue fingers, dermatomyositis, and myasthenia gravis.

A 61-year-old woman presented with a sudden marked swelling and blue-red discolouration of her entire left leg. The diagnosis phlegmasia cerulea dolens was made. This is an extreme case of lower-extremity deep venous thrombosis that can cause critical limb ischaemia and possible limb loss.

The revised Dutch College of General Practitioners (Nederlands Huisartsen Genootschap [NHG]) guideline 'Deep-vein thrombosis and pulmonary embolus' includes recommendations for the treatment of patients with deep-vein thrombosis (DVT) and thrombophlebitis, and for the exclusion of pulmonary embolism (PE). The general practitioner (GP) can exclude the presence of DVT or PE in some patients by using a decision rule and a D-dimer test. When using D-dimer test as a point-of-care test, meticulous care is essential during the test procedure and storage of blood. The GP can treat many patients with DVT; the NHG guideline does not advise use of direct oral anticoagulants. In the case of an isolated DVT in the calf, treatment with anticoagulants or ultrasound follow-up can be chosen in consultation with the patient or on the basis of regional agreements. In the case of patients with superficial thrombophlebitis, a wait-and-see approach is usually sufficient.

The revised Dutch College of General Practitioners (Nederlands Huisartsen Genootschap [NHG]) guideline 'Deep-vein thrombosis and pulmonary embolus' includes recommendations for the treatment of patients with deep-vein thrombosis (DVT) and thrombophlebitis, and for the exclusion of pulmonary embolism (PE). The general practitioner (GP) can exclude the presence of DVT or PE in some patients by using a decision rule and a D-dimer test. When using D-dimer test as a point-of-care test, meticulous care is essential during the test procedure and storage of blood. The GP can treat many patients with DVT; the NHG guideline does not advise use of direct oral anticoagulants. In the case of an isolated DVT in the calf, treatment with anticoagulants or ultrasound follow-up can be chosen in consultation with the patient or on the basis of regional agreements. In the case of patients with superficial thrombophlebitis, a wait-and-see approach is usually sufficient

De herziene NHG-standaard 'Diepe veneuze trombose en longembolie' bevat aanbevelingen voor de diagnostiek en behandeling van patiënten met diepe veneuze trombose (DVT) en tromboflebitis en voor het uitsluiten van een longembolie.De huisarts kan aanwezigheid van een DVT of longembolie uitsluiten bij een deel van de patiënten door gebruik te maken van een beslisregel en een D-dimeerbepaling.Voor gebruik van een D-dimeerbepaling als sneltest ('point-of-care test') zijn zorgvuldigheid rond de testafname en het bewaren essentieel.De huisarts kan veel patiënten met een DVT zelf behandelen; de NHG-standaard adviseert het gebruik van directe orale anticoagulantia niet.Bij een geïsoleerde kuitvenetrombose wordt in overleg met de patiënt of op grond van regionale afspraken voor behandeling met antistolling of echografische follow-up gekozen.Bij patiënten met oppervlakkige tromboflebitis kan meestal worden volstaan met een afwachtend beleid.

Lemierre's syndrome, a thrombophlebitis of the internal jugular vein, is a rare disorder, usually caused by the microorganism Fusobacterium necrophorum. Throat ache and swelling of the neck are often the first symptoms. Without adequate treatment, Lemierre's syndrome may result in thrombosis of the internal jugular vein and metastatic lung abscesses, with a mortality rate of 18%. On the basis of 2 cases, Lemierre's syndrome is described here. In cases where Lemierre's syndrome is suspected, hospitalization often follows, with the administration of intravenous antibiotics and drainage of the abscesses. One should be on the alert for Lemierre's syndrome when a patient is presented with swelling in the neck following an oropharyngeal infection.

Mondor's disease is a rare benign condition characterized by thrombophlebitis of superficial veins of the chest and the breast. Typically, there is a spontaneous onset of tenderness and redness due to a cord-like induration on the lateral thoracic wall. It is a self-limiting disease that should be distinguished from breast cancer. In general, the combination of clinical symptoms and mammography confirms the diagnosis.

We reviewed the literature for the association between superficial vein thrombosis (SVT) and venous thromboembolism (VTE) and for evidence of treatment of SVT with low-molecular-weight heparin (LMWH). There is some evidence for an association between SVT and VTE. This association seems stronger for proximal SVT as compared with distal SVT. In general practice, the absolute risk of VTE with or after SVT is low, approximately 3%. There is evidence that treatment of SVT with LMWH may have a beneficial effect on its course. NSAIDs have a similar effect. There is indirect evidence that LMWH is effective in the prevention of VTE, if the treatment is continued for more than 4 weeks. In case of a proximal SVT, we suggest an ultrasound examination be carried out and LMWH treatment given if the diagnosis is confirmed; in other cases, NSAIDs can be considered.

In a 53-year-old man with acute sigmoid diverticulitis abdominal CT revealed septic thrombophlebitis with gas configurations in the inferior mesenteric vein.

A 35-year-old woman had a palpable painful cord under the left breast with dimpling in the breast, due to Mondor's disease.

Two male patients aged 55 and 77 years, respectively, presented to the casualty department with fever, chills and right abdominal upper quadrant tenderness. They also had hyperbilirubinaemia. Based on CT scan findings and blood cultures yielding Bacteroides fragilis, a diagnosis of pylephlebitis (septic thrombophlebitis of the mesenteric veins and/or the portal vein) was made. This is a condition with a mortality rate of 10-70%. Primary sources such as diverticulitis are often seen in patients with pylephlebitis, in which bacteria are drained by the mesenteric veins and cause a thrombus in the portal system. In the two patients no primary focus was detected. They were treated with intravenous antibiotic therapy followed by oral antibiotics, and were discharged in good health. Pylephlebitis can be complicated by liver abscesses. Treatment consists of broad-spectrum antibiotics which are adjusted based on the blood cultures results. The duration of treatment is between two and six weeks, depending on the presence of liver abscesses. In patients with abscesses that cannot be drained, longer treatment may be indicated.

An 18-year-old man was admitted because of high fever, a sore throat and dyspnoea. Additional investigations revealed tonsillitis, lung abscesses and thrombophlebitis of the internal jugular vein. Lemierre's syndrome was diagnosed.

A 19-year-old man was admitted with fever, haematuria, general malaise, pain on the left side of the pelvis and a full feeling in the left lower abdominal quadrant of the abdomen. His history mentioned a varices operation. During admission he developed an acute abdomen and deep vein thrombosis in both legs. CT of the abdomen showed a pathologic mass and absence of the vena cava inferior. Laparotomy revealed extensive thrombosis in widened veins. Clinical improvement occurred following adequate thrombolysis. Congenital absence of the vena cava inferior results from aberrant development during organogenesis. Patients are typically asymptomatic, but may present with venous insufficiency at a young age and deep vein thrombosis. Absence of the vena cava inferior can be accompanied by other congenital abnormalities such as dextrocardia and congenital heart diseases. Diagnosis is made using CT or MRI. Treatment consists of lifelong anticoagulant therapy and, if necessary, surgery.

In a 35-year old man with deep venous thrombosis liver metastases of an adenocarcinoma were observed. The primary tumour was not found. The patient had pigmentations on the lips and hamartomatous polyposis of the intestine. These findings indicate Peutz-Jeghers syndrome. In this syndrome there is an increased risk of, notably, gastrointestinal malignancy at an early age. Recent investigations have shown that Peutz-Jeghers syndrome is associated with a mutation in the STK11 gene on chromosome 19.

To evaluate a standard protocol for ambulant treatment of patients with deep venous thrombosis (DVT) in a leg with low molecular weight heparins (LMWH).Prospective.A protocol was established within a regional context which provided for all patients in or around Veghel, the Netherlands, with demonstrated DVT in the period 1 March 1997-31 July 1999 to receive ambulant treatment with LMWH and oral anticoagulants. After diagnosing the treatment was started in the emergency department of the Sint Joseph Hospital in Veghel, the subsequent LMWH injectons were administered by the general practitioner in his practice.Of the 130 patients with demonstrated DVT, six were hospitalized because of pulmonary embolism, carcinoma or pregnancy, while 124 (95%) were eligible for ambulant treatment (56 males with a median age of 57 years (range 16-98) and 68 females with a median age of 51 years (18-89). During the acute period of LMWH treatment, none of the 124 patients developed a recurrence of thrombosis, pulmonary embolism or major haemorrhage. During 3 months' follow-up one patient developed a thrombosis in the other leg, this patient died after 2 months from disseminated carcinoma of the colon.Ambulant treatment of patients with DVT of the leg with LMWH in a daily practice setting is feasible and can be carried out effectively and safely in 95% of the patients in a regional organization.

A 55-year-old man presented with deep venous thrombosis of his left leg. Laboratory evaluation disclosed macrocytic anaemia and leukocytosis. In the clinical decision process it was not verified whether all symptoms fitted in with the presumptive diagnosis of pernicious anaemia and stomach carcinoma. The ultimate diagnosis was acute lymphocytic leukaemia. A proper evaluation of a peripheral blood smear would have led to this diagnosis earlier.

Two men, aged 71 and 56 years, with pacemakers, developed the superior vena cava syndrome one and five years, respectively, after infection of the pacemaker pocket. They had been treated with antibiotics and partial removal of the foreign bodies. The conditions of both included occlusion of the superior vena cava and of both subclavian veins. The symptoms disappeared after removal of the total pacemaker system and venous reconstruction. The possibility of a superior vena cava syndrome occurring is increased if other complications have occurred previously, particularly infection. Prevention and treatment comprise on the one hand prevention and treatment of the infection (which is not always obvious) and on the other, earliest possible detection of thromboembolisms.

A 67-year-old man was admitted because of suspected deep venous thrombosis of the right leg. At sonography, CT of the abdomen and aortography it became obvious that compression of the right iliac vein by an abdominal aortic aneurysm was responsible for the deep venous thrombosis. This has only been reported before twice. In the case of deep venous thrombosis or oedema of the legs an abdominal aortic aneurysm needs to be excluded.

Evaluation of clinical management in patients suspected of pulmonary embolism as compared with the Dutch consensus for diagnosing pulmonary embolism.Retrospective.Medical Centre Alkmaar.Retrospective analysis of clinical management in patients subjected to ventilation-perfusion scintigraphy on suspicion of pulmonary embolism.In 55 (24%) of the 225 patients included a high-probability scan was found, in 68 (30%) a non-high-probability scan and in 102 (45%) a normal scan. The clinical management in all patients with a normal or high-probability scan was according to the consensus. In the 68 patients with a non-high-probability scan the consensus was completely followed in 16%, partially in 18% and not at all in 66%.Clinical management of patients with a non-high-probability scan in the vast majority of cases was not according to the consensus. The consensus was followed in all patients with a normal or high-probability scan.

Ascending phlebothrombosis of the lower extremities can have serious consequences if not identified at an early stage. In the Academic Hospital of the Free University, Amsterdam, 18 patients with this disease were seen in the period 1980-1991. Treatment should be aimed at preventing progression of the disease to the deep venous system. This may necessitate ligation and dissection of the V. saphena magna in the groin. Usually conservative treatment will do, provided regular follow-up is performed to see whether progression has taken place to the hiatus saphenus or the popliteal fossa. As the disease has usually progressed further than the clinical signs indicate, duplex scanning should be used in case of doubt. Progression to the deep system should immediately be treated surgically.

In a consensus meeting on the prophylaxis of deep venous thrombosis guidelines were given - especially for the Dutch situation - concerning the use of mechanical measures (elastic stockings, intermittent pneumatic compression) and of antithrombotic medication (low doses of heparin, coumarin derivatives, dextrans) in various groups of surgical and non-surgical patients.