Case study: Folliculotropic mycosis fungoides скачать в хорошем качестве

Case study: Folliculotropic mycosis fungoides

A case study presentation by Prof. Miles Prince. Full transcript My name is Miles Prince. I am a hematologist and I work at the Peter MacCallum Cancer Centre in Australia. I am going to talk through a very interesting and difficult case study with you today. I recently treated a patient who was referred to me because he had a difficult-to-manage a form of cutaneous T-cell lymphoma (CTCL) called folliculotropic mycosis fungoides (MF). Folliculotropic MF is challenging because it is an aggressive variant of MF. Of particular interest is that this patient is relatively young, and well below the average age of onset of MF, which is 70 years. This 51-year-old man first presented in January 2013. He had a 1-2 year history of large, scaly annular plaques, which began predominantly on his cheeks and arms, and he had a typical pattern of follicular tropism. He had treatment with topical and intralesional steroids followed by narrow band UVB and oral prednisone; but unfortunately did not experience a prolonged response. The rash continued to worsen, and by March 2013 when he first presented to us, a course of subcutaneous interferon-α (IFN-α) at a standard dose of 1.5 IU t.i.d was recommended. This is a relatively low dose, and patients are encouraged to increase to 2 or 3 million units a day. The patient received IFN-α treatment for approximately nine months and responded well. One of the major problems that he had, however, was mood changes, which are a common side effect of this type of medication. The mood changes were quite significant and despite his best efforts, the patient had to discontinue the therapy. By January 2014, approximately a year after his initial presentation, the patient was clearly progressing and symptomatic, and was therefore started on 25 mg/week of methotrexate. There was some disease stabilisation initially, but the patient's condition slowly progressed. When the patient presented he had plaque-stage disease predominantly on his upper limbs, but approximately 12% of his body surface area (BSA) on his lower limbs were affected as well. By definition he was stage 1B folliculotropic MF. As this is often a histopathological diagnosis, the pathology was reviewed and it showed classic features of epidermotropism and, importantly, no evidence of transformation. There was, however, a clear folliculotropic element. Some photo micrographs of the histopathology have been provided. The pictures of his face show the predominant areas of follicultropism, with areas of alopecia and raised areas around the follicles. There were definite areas of alopecia, but this is difficult to see in the photographs. There are typical patterns of small atypical lymphocytes, with no evidence of transformation. There is a good picture [inset] of the hair follicles and adnexa with some high magnification views of these atypical cells. The disease was not difficult to diagnose. The patient presented with CD3 and CD4 positivity, which are a classic sign of CTCL. Of particular interest was that the patient was also CD25 positive. This was significant due to the potential therapeutic options available, such as the fusion toxin denileukin diftitox. There was relatively low-level CD30 positivity, with only approximately 10--15% of cells having strong CD30+ intensity. It was clear that the patient was not responding to the treatment available. This case was unusual, as mentioned previously, in that this was a relatively young man. He was only 51 years old and was seriously considering allogeneic transplantation, which is something that is rarely considered in CTCL. Folliculotropic MF is an intermediate disease ranging from low-grade to more aggressive. Allogeneic transplant was a serious consideration. For the full transcript, please visit www,lymphomahub.com.