Quick tip: National guidance requires all patients with suspected neutropenic sepsis to have broad spectrum antibiotics within 1 hour
Neutropenic sepsis develops in patients on chemotherapy or with bone marrow failure due to intrinsic haematological disease such as myelodysplastic syndrome or acute leukaemia, or due to immune neutropenia. Urgent identification and treatment is critical as each hour delay in antibiotic therapy increases mortality. Centres offering chemotherapy will have an emergency line for their patients for 'acute oncology service' review on oncology or haematology wards but patients can still present to GP, walk-in centres or accident and emergency departments therefore vigilance is critical.
Ongoing support of the patient involves regular review, involvement of critical care if necessary and discussion with microbiology if atypical organisms suspected or the patient is not responding to first line antibiotics. This discussion may involve consideration of atypical organisms such as fungal chest/sinus diseases and pneumocystis jiruvecii for example.
Quick Tip; Venesection performed with a tourniquet in place can give an artificially elevated calcium
Hypercalcaemia can be a consequence of myeloma, some lymphomas and leukamias, and non-haematological malignancies. Presenting symptoms include;
Priorities of management are;
Quick Tip; In any case of suspected malignant spinal cord compression immediate steroid therapy before imaging is required to minimise permanent neurological injury
Cord compression is an emergency that needs rapid action to prevent permanent neurological injury. Causes are most commonly solid organ malignancy metastases and myeloma, although it can also be caused by lymphoma or rarely patients with leukaemic infiltration of the cord can present as a cord compression syndrome.
Short term management includes discussion with neurosurgery if a compressive lesion is found on MRI, depending on the appropriateness for the patient. Radiotherapy may be considered. Management of the underlying malignant cause is the definitive treatment.
Quick Tip: Prompt rasburicase and fluid therapy are the definitive management for renal failure induced by TLS
Tumour lysis syndrome (TLS) is a combination of clinical and laboratory features that develop in cancer patients receiving chemotherapy or rarely spontaneously in fast growing tumours. It is most commonly seen with so-called 'liquid' tumours such as leukaemia or lymphoma which are particularly sensitive to chemotherapy due to a rapid proliferative rate among other reasons, and the syndrome develops when the contents of these malignant cells are released into the circulation. The syndrome can be life-threatening due to gross electrolyte disturbance and potentially severe renal impairment.
Diagnostic criteria as per Cairo and Bishop criteria are;
2 or more of
Uric acid > 476 micromol/L or 25% increase from baseline
Potassium > 6mmol/L or 25% increase from baseline
Phosphate (Adults) > 1.45mmol/L or 25% increase from baseline
Phosphate (Children) > 2.1mmol/L or 25% increase from baseline
Calcium <1.75mmol/L or 25% decrease from baseline
1 needed for diagnosis of clinical TLS
Creatinine > 1.5 upper limit normal
The priority with tumour lysis is prevention by patient risk assessment before chemotherapy and management with fluids, allopurinol or rasburicase depending on the relative risk of occurrence. There will however be patients who do develop TLS spontaneously with a rapidly proliferative malignancy or who develop it even with prophylaxis. In those patients management should be;
Quick tip; DIC will not resolve unless the underlying trigger is managed
Disseminated intravascular coagulation is a condition of gross over-activation of the coagulation pathway causing increased risk of clot formation and subsequent bleeding risk from consumption of clotting factors and platelets. This is most commonly triggered by trauma, infection, malignancy, severe inflammation such as pancreatitis and obstetric complications. A DIC-like syndrome can also be caused by acute promyelocytic leukaemia (APL), a subtype of acute leukaemia. DIC should be managed by the team caring for the patient in conjunction with haematology
Quick Tip; Not all elements of the classic 'pentad' are required to diagnose TTP
Thrombotic thrombocytopenic purpura is a form of microangiopathic haemolytic anaemia (MAHA) which is a syndrome of intravascular haemolysis of small vessels associated with end organ damage and significant morbidity and mortality. It is not a common condition but especially for haematologists, awareness and vigilance are critical as appropriate management will limit morbidity and mortality.
TTP arises either due to an inherited defect of, or acquired antibody to the ADAMTS13 enzyme which is used to breakdown large von Willebrand factor (vWF) multimers. Because of this large vWF complexes form in small vessels, cleaving red cells and causing haemolysis. Acquired causes include;
Due to this haemolysis and associated inflammatory response there is associated end organ damage. The classic pentad of Thrombotic thrombocytopenic purpura are;
Haematological investigations may show;
Diagnosis is made from a combination of the clinical features and seeing schistocytes (red cell fragments) on a blood film
Treatment needs removal of the precipitating cause and plasma exchange. This is the only definitive management of the condition and must be guided by haematology advice.
Quick tip: Baseline haemoglobin is often lower than reference range therefore check previous results so as not to transfuse inappropriately
Sickle cell anaemia manifests in patients who are homozygous for the sickle gene HbS or have a compound sickling disorder of a heterozygous HbS with another haemoglobinopathy such as beta thalassaemia. Clinical manifestation of the disease is variable between individuals therefore a thorough history of the patient's disease, treatment and frequency of crises is important for management. A painful sickle cell crisis (aka vaso-occlusive crisis), in the most basic terms, occurs when a trigger leads to local or systemic hypoxia. This leads the abnormal red cells to 'sickle' and these can occlude small vessels leading to infarction, pain or other complications.
Commonest causes for painful sickle crises;
Other presentations of sickle cell disease;
Discuss with local haematology team for additional and ongoing management.
Links: NICE CKS