Until relatively recently, major surgery in patients with acute leukaemia was usually avoided because the mortality was assumed to be prohibitively high. For example, in two series of leukaemic patients in 1964 and 1968, 14 of 148 patients and 12 of 259 patients developed complications requiring surgery. Of these 26 patients, four underwent surgery and three of them died. The remaining 22 patients, who were managed conservatively, all died.

Improved remission with chemotherapy rates mean that elective surgical procedures, which formerly would not have been considered, may now be undertaken just as they would in healthy patients. Thus, the major surgical challenge remains the acute leukaemic patient requiring an emergency operation.

The demand for surgery in these patients is increasing for two reasons. First, as a result of improved chemotherapy and medical management of leukaemia, complications requiring surgery are becoming more frequent. The true incidence is unknown but several studies report a surgically remediable complication rate of 5 to 10 per cent. Second, advances in supportive techniques have facilitated a more aggressive approach in dealing with complications requiring surgery. Moreover, improved imaging has increased the accuracy of preoperative diagnosis and reduced the occurrence of unnecessary laparotomy for a possible acute abdomen.

Finally, recent advances in management increasingly require surgical procedures for diagnosis, venous access, and therapeutics. In Oxford, for example, a cut-down insertion of a Hickman line is commonly practised in certain patients requiring chemotherapy.

Leukaemic patients are susceptible to several conditions and complications, an awareness of which will enhance early diagnosis and appropriate management. In addition to these specific problems, leukaemic patients are susceptible to the same surgical problems as the general population. These conditions may also demand urgent surgery and Table 1 588 is by no means an exhaustive classification of indications for surgery.

These patients are predisposed to necrotizing enteric lesions, which may occur anywhere between the oesophagus and the rectum. These lesions are usually the result of leukaemic infiltration and may manifest as ulcers, mucosal haemorrhage and infarction, and mechanical obstruction. Necrosis and perforation can ensue after secondary bacterial invasion or intensive chemotherapy. Opportunistic infection with Candida and other organisms including Mycobacterium tuberculosis may occur.

The association between anorectal disease and acute leukaemia is well recognized. It manifests as ulcers, abscesses, fissures, and fistulae. Rectal ulcers, due to infiltration, with or without associated abscesses are uncommon, but are said to be distinctive complications of this disease. Anorectal infections may be characterized by rapid local spread of inflammation, and early surgery has been associated with uncontrolled, massive sloughing of the perianal tissues with fatal outcome. The safest approach in these patients is to administer high-dose antibiotics when the picture is one of inflammatory induration, while a small well-placed incision should be used when there is an obviously fluctuant abscess. Wide excision of inflamed tissues should be avoided.

Infiltration of organs outside the gastrointestinal tract occurs most commonly in the spleen and occasionally the prostate. Prostatectomy may be necessary to relieve bladder outlet obstruction, but should be postponed until the patient is in remission. Splenectomy may be indicated for several reasons, including painful enlargement, hypersplenism, or spontaneous rupture. These complications most commonly occur in patients with one of the chronic leukaemias.

The acute leukaemic patient presents several technical difficulties to the surgeon, and accepted surgical principles do not necessarily apply. Poor quality white blood cells and immunosuppression, induced by chemotherapy and steroids, predispose to infection and abscess formation. Thrombocytopenia, abnormal platelet function, and clotting disorders increase the risks of perioperative haemorrhage. Disseminated intravascular coagulation remains an almost absolute contradiction to surgery, except in a critically ill patient with poor venous access (often a child), for whom a cut-down insertion of a Hickman line into the inferior vena cava via the saphenous vein can be safely performed.

Symptoms and signs of serious pathology, for example peritonitis, are frequently mild or non-specific because of the inability of these patients to mount an adequate host response to infection. The host response is further suppressed by steroid therapy. This suppression is reflected by an inappropriately low-grade temperature, white-cell count, and levels of inflammatory markers such as C-reactive protein or erythrocyte sedimentation rate. In addition, signs suggestive of an acute abdomen, such as abdominal pain, nausea, vomiting, and even malaena are common in leukaemic patients and are frequent symptoms of toxic chemotherapy. In 20 cases of appendicitis reported in the literature before 1970, the diagnosis was suspected prior to death in only 12 patients.

Major surgery has been successfully performed in acute leukaemic patients. Successful thoracotomy, common bile duct drainage, colectomy, hysterectomy, prostatectomy, and caesarian section have all been reported. However the decision to undertake major surgery should always be influenced by both the patient's general condition and his or her prognosis. Even though abdominal complications may be recognized, surgical intervention may not be possible because of the precarious condition of the patient.

As far as possible, only one surgical firm manages leukaemic patients requiring surgery in Oxford. This accumulated experience has allowed the development of an expertise and familiarity with these patients and the problems specific to them. Management is further facilitated by consideration of the following points.

A high index of suspicion is required when assessing these patients because of their minimal signs and symptoms. Computed tomography and magnetic resonance imaging should be readily used when the diagnosis is in doubt. Surgery must not be undertaken without the availability of the necessary blood product support. Emergency surgery can usually be postponed overnight to ensure a full blood transfusion and haematological laboratory service. Emergency surgery, even when a relatively simple procedure such as appendicectomy, should be performed by an experienced surgeon and not delegated to a junior. Aggressive antibiotic cover is used and the clinical microbiologists are notified and involved in management at an early stage. When platelet support is anticipated, it should be started after induction of anaesthesia and continued for several hours after surgery. However if splenectomy is being performed in the face of hypersplenism, for example in myelofibrosis, platelet infusion should only begin after the spleen is removed to avoid wasteful sequestration of platelets in the spleen. Surgical incisions must be planned to give generous exposure, especially when there is some diagnostic doubt. Muscle cutting incisions should be avoided if possible. Long midline incisions are generally suitable. Meticulous haemostasis must be obtained during surgery, and when bleeding has been a problem, suction drains are advisable to facilitate postoperative monitoring. Postoperative management should be in an intensive care or high dependency unit where accurate monitoring is possible. A high index of suspicion for haemorrhagic complications is essential and a policy of early relook laparotomy, before the onset of coagulopathies, is advised.

Problems with preoperative diagnosis are common and early imaging is advised. An awareness of the conditions and complications to which these patients are predisposed will aid diagnosis. With platelet support, meticulous surgical technique, and careful postoperative management, major surgery is usually feasible in leukaemic patients. Thus, the decision to operate on these patients will be based increasingly on surgical indications.

Rasmussen BL, Freeman JS. Major surgery in leukemia. Am J Surg 1975; 130: 647–51.

Bishop JF, Schiffer CA, Aisner J, Matthews JP, Wiernik PH. Surgery in acute leukemia: a review of 167 operations in thrombocytopenic patients. Am J Hematol 1987; 26: 147–55.

Spiers ASD. Surgery in management of patients with leukaemia. Br Med J 1973; 3: 528–32.

Sedhev MK, Dowling MD, Seal SH, Stearns MW. Perianal and anorectal complications in leukemia. Cancer 1973; 31: 149–52.