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When the lesion is localized, it is generally curable. However, long-term survival for children with advanced disease older than 18 months of age is poor despite aggressive multimodal therapy (intensive chemotherapy, surgery, radiation therapy, stem cell transplant, differentiation agent isotretinoin also called 13-"cis"-retinoic acid, and frequently immunotherapy with anti-GD2 monoclonal antibody therapy).
Biologic and genetic characteristics have been identified, which, when added to classic clinical staging, has allowed patient assignment to risk groups for planning treatment intensity. These criteria include the age of the patient, extent of disease spread, microscopic appearance, and genetic features including DNA ploidy and N-myc oncogene amplification (N-myc regulates microRNAs), into low, intermediate, and high risk disease. A recent biology study (COG ANBL00B1) analyzed 2687 neuroblastoma patients and the spectrum of risk assignment was determined: 37% of neuroblastoma cases are low risk, 18% are intermediate risk, and 45% are high risk. (There is some evidence that the high- and low-risk types are caused by different mechanisms, and are not merely two different degrees of expression of the same mechanism.)
The therapies for these different risk categories are very different.
- Low-risk disease can frequently be observed without any treatment at all or cured with surgery alone.
- Intermediate-risk disease is treated with surgery and chemotherapy.
- High-risk neuroblastoma is treated with intensive chemotherapy, surgery, radiation therapy, bone marrow / hematopoietic stem cell transplantation, biological-based therapy with 13-"cis"-retinoic acid (isotretinoin or Accutane) and antibody therapy usually administered with the cytokines GM-CSF and IL-2.
With current treatments, patients with low and intermediate risk disease have an excellent prognosis with cure rates above 90% for low risk and 70–90% for intermediate risk. In contrast, therapy for high-risk neuroblastoma the past two decades resulted in cures only about 30% of the time. The addition of antibody therapy has raised survival rates for high-risk disease significantly. In March 2009 an early analysis of a Children's Oncology Group (COG) study with 226 high-risk patients showed that two years after stem cell transplant 66% of the group randomized to receive ch14.18 antibody with GM-CSF and IL-2 were alive and disease-free compared to only 46% in the group that did not receive the antibody. The randomization was stopped so all patients enrolling on the trial will receive the antibody therapy.
Chemotherapy agents used in combination have been found to be effective against neuroblastoma. Agents commonly used in induction and for stem cell transplant conditioning are platinum compounds (cisplatin, carboplatin), alkylating agents (cyclophosphamide, ifosfamide, melphalan), topoisomerase II inhibitor (etoposide), anthracycline antibiotics (doxorubicin) and vinca alkaloids (vincristine). Some newer regimens include topoisomerase I inhibitors (topotecan and irinotecan) in induction which have been found to be effective against recurrent disease.
Chemotherapy with topotecan and cyclophosphamide is frequently used in refractory setting and after relapse.
Mycosis fungoides can be treated in a variety of ways. Common treatments include simple sunlight, ultraviolet light (mainly NB-UVB 312 nm), topical steroids, topical and systemic chemotherapies, local superficial radiotherapy, the histone deacetylase inhibitor vorinostat, total skin electron radiation, photopheresis and systemic therapies (e.g. interferons, retinoids, rexinoids) or biological therapies. Treatments are often used in combination.
In the “Stanford technique” of Total skin electron therapy the patient stands about 10 meters from a radiation source, with a large acrylic sheet in between to scatter the electrons across a broad area. Then the patient carefully assumes six different positions. In severe cases that progress to Sézary disease
Stanford University has been pioneering low-dose radiation (1/3 of the standard), followed by stem-cell transplantation without chemo, as a potential cure with promising results.
In 2010, the U.S. Food and Drug Administration granted orphan drug designation for naloxone lotion, a topical opioid receptor competitive antagonist used as a treatment for pruritus in cutaneous T-cell lymphoma.
Experimental treatments include Resimmune or A-dmDT390-bisFv(UCHT1) which is an anti-T cell immunotoxin in a Phase II clinical trial.
Mogamulizumab (KW-0761) had a phase 3 clinical trial for Relapsed/Refractory CTCL (including mycosis fungoides). After preliminary results on mycosis fungoides in 2017 the US FDA granted it a priority review for CTCL.
A limbal nodule is any nodular lesion at the limbus (junction of the cornea and sclera) of the eye.
The differential diagnosis for a limbal nodule can include:
- Pinguecula
- Early Pterygium
- Foreign body / foreign body granuloma
- Phlycten, an inflamed nodule of lymphoid tissue
- Episcleritis
- Scleritis
- Granuloma
- Limbal dermoid, a kind of choristoma (NB: in other organs "dermoid" can refer to a teratoma)
- malignant melanoma
The main goal of treatment is to remove the cause of the phlegmonous process in order to achieve effective treatment and prevention of recidives.
If the patient's condition is mild and signs of inflammatory process are present without signs of infiltrates, then conservative treatment with antibiotics is sufficient.
If the patient's condition is severe, however, immediate operation is usually necessary with application of drainage system. All of these are done under general anaesthesia. During operation, the cavity or place of phlegmonous process are washed with antiseptic, antibiotic solutions and proteolyic ferments.
In post-operative period, patients are treated with intravenous antibiotics, haemosorbtion, vitaminotherapy. Additionally, the use of i/v or i/m antistaphylococci γ-globulin or anatoxin can be taken as immunotherapy.
During operation of phlegmon dissection at any location, it is important:
1. to avoid spreading of pus during operation;
2. to take into account the cosmetic value of the operating site, especially when treating phlegmmonous process of the face; and
3. to avoid damaging nerves.
If infested, animals should be removed from the flock and all wool in and around roughly a 1 cm or larger radius of the discoloured area clipped. The area is treated with insecticide to kill the maggots. Soothing cream can also be applied to skin grazes or lesions caused by the maggots. Clippings should be collected after removal and placed in a maggot-proof bag and left closed in the sun, to ensure that no other animal is infected.
There are several preventative measures which are used to reduce the occurrence of flystrike in sheep flocks, these include:
- Controlling intestinal parasites to prevent scours and a suitable surface for flystrike
- Scheduled shearing and crutching
- Removing the tails of lambs at weaning
- Mulesing
- Preventative chemical treatments before fly infestation risk is high
- Breeding for traits that reduce the likelihood of infestation
- Removing or avoiding large maunure heaps or other sites attractive to the flies
- Using fly traps near the flock to attract and kill any local flies, helping to minimise the local populations. NB: Traps often emit a pungent smell and are best placed away from human activity.
None of these measures completely stop the occurrence of fly strike in sheep, and regular treatment is still necessary.
Systemic features of infection such as increased body temperature (up to 38-40 °C), general fatigue, chills, sweatings, headache, loss of appetite).
Inflammatory signs – dolor (localized pain), calor (increase local tissue temperature), rubor (skin redness/hyperemia), tumor (either clear or non-clear bordered tissue swelling), functio laesa (diminish affected function).
NB: severity of patient condition with phlegmons is directly proportional to the degree of intoxication level i.e. the more severe the condition, the higher the degree of intoxication level.
A noninfectious occurrence of phlegmon can be found in the acute pancreatitis of Systemic Lupus Erythematosus. The immunosuppressive aspects of this disease and the immunosuppressive medications used to treat it blunt each of the signs of infection.