After an interesting day on resus with a syndrome I’d never heard of, I thought now would be a good time to research this condition and revise my knowledge of some of the other issues that this particular patient presented with.
The underlying diagnosis with this patient was leukaemia. As there is a previous blog post about this we will skip to the issue of why he presented to the emergency department. Neutropenic sepsis is a complication of those undergoing chemotherapy. Chemotherapy causes a reduction in the number of neutrophils, a form of white blood cell that is heavily involved in fighting off infection. Neutropenic sepsis is defined as a temperature higher than 38 degrees Celsius in a patient with a neutrophil count of <0.5 x10^9/L. Therefore, when there is a low number of neutrophils the patient is much more susceptible to infections that usually the immune system would easily fight off. Patients that have a reduced number of neutrophils are instructed to measure their temperature and to present to hospital if this is raised, due to the fact simple infections can now be fatal. If a patient has undergone chemotherapy within the last 6 weeks and is unwell, always consider neutropenic sepsis.
Sepsis causes leaky capillaries, where fluid escapes the vasculature into the extracellular fluid. This is due to the innate immune response and can cause severe hypotension. This response is a physiological reaction to infection, which is supposed to remain localised to enable an increased blood flow to the area and therefore an increased white blood cell delivery. However, sepsis has been shown to develop when the innate immune response becomes amplified and unregulated. This causes an imbalance of inflammatory response causing systemic vasodilation and increased capillary permeability. Patients can often present with a crashing blood pressure and this needs severe fluid resuscitation to correct the hypotension.
When it comes to managing sepsis, the Sepsis 6 is used. I like to think of this as take 3 and give 3 to help remember the whole management:
- Blood cultures
- Urine output
- Broad spectrum antibiotics (until the organism and sensitivities has been established)
The condition that the patient also had was sweet’s syndrome, or febrile neutrophilic dermatosis. This is a skin disease is characterised by acute, tender, erythematous plaques, nodes, pseudo vesicles and sometimes blisters, usually only on the head, legs and arms, the trunk is rarely involved. Fever and joint pain often accompany this. In this case Sweet’s syndrome was malignancy associated however it can be drug induced or idiopathic. This condition is treated with granulocyte colony-stimulating factor (G-CSF) which is also a treatment for acute myeloid leukaemia. This lesions often occur in patients with neutrophilia however can appear in neutropenic when being treated with G-CSF as it induces stem cell proliferation.