Cutaneous T-cell lymphoma (CTCL) is a rare and difficult to diagnose malignancy of the lymphatic system. The disease is caused by an uncontrolled growth of T lymphocytes located in the lymphatic system of the skin. Patients with cutaneous T-cell lymphoma are few in number and therefore the disease and the living situation of this group of patients are rarely heard about. A study on the quality of life of Polish patients with CTCL aims to change this. This analysis is to open the eyes of the public to the most important needs of patients in the various spheres of their lives and their fight against illness.
The study 'Lymphomas we don't know. The problem we don't see', is the first study in Poland entirely dedicated to patients with CTCL, whose partners are Association of Friends of Lymphoma Patients "Snowdrop", Owl Eyes Lymphoma Support Association, Institute for Patients' Rights and Health Education and Federation of Polish Patients (FPP) and Orphan National Forum for Rare Disease Treatment, as well as key Scientific Societies, viz: Polish Lymphoma Research Group (PLGR), Medical University of Gdańsk (GUMed) and Polish Society of Dermatology (PTD). The study's ambassadors are experts specialising in the treatment of patients with cutaneous lymphoma: Prof. Dr. Małgorzata Sokołowska-Wojdyło, MD, PhD, and Hanna Ciepłuch, MD, PhD, both of whom are actively involved in co-creating the exploratory part of the study and will support it from the scientific side.
For years, patient organisations with the support of the medical community have been conducting numerous awareness campaigns on lymphomas, their types, epidemiology, diagnosis and treatment. From now on, these activities will be supplemented by education about a specific type of lymphoid malignancy, such as cutaneous lymphoma, with an emphasis on the most common T-cell lymphoma in this group, CTCL (Cutaneous T-cell Lymphoma). Outreach activities will begin with a survey of CTCL patients entitled: 'Lymphomas we don't know. The problem we don't see', which will be conducted in selected clinics located throughout Poland. The authors of the study will first conduct a series of in-depth interviews with both key subject matter experts specialising in the treatment of CTCL patients and patients. Based on the interviews and the adopted methodology, a patient survey will be created. The work will result in a report containing the most important findings, collecting not only a statistical description of the patients' situation, but also pointing out the unmet needs of the patients in various spheres of their lives. The document will be presented in early 2019.
There are few patients with CTCL in Poland. It is a difficult type of lymphoma to diagnose and few doctors specialise in treating these patients. Patient organisations also have very limited knowledge for this group of patients, so it is difficult to help them. I believe that the study we are working on and partnering with will allow us to understand the lives of patients with CTCL, to learn about their needs and problems, and for the public to learn more about this disease. We believe that the study will clearly define the unmet needs of this group of patients in Poland, contribute to starting a discussion about them, and thus improve the fate of patients with CTCL - said Maria Szuba, Chair of the Board of Directors of the Association of Friends of Lymphoma Patients 'Snowdrop'.
Knowledge of cutaneous lymphoma (CTCL) among doctors, patients and the public is low. We see a great need for education about this disease entity. As partners in the study, we will actively encourage patient participation through our news media. It is becoming necessary to develop a realistic and reliable picture of the lives of patients with this diagnosis in Poland - says oncologist Dr Elżbieta Wojciechowska-Lampka, President of the Owl Eyes Lymphoma Support Association.
The embarrassing symptoms of this disease, its concealment and fear of social marginalisation mean that awareness of this disease is very low. I believe that this study of the quality of life of patients with CTCL will contribute to a broader understanding of this disease and, above all, will show what problems CTCL patients face on a daily basis. Igor Grzesiak of the Institute for Patients' Rights and Social Education points out.
Lymphomas we don't know
Lymphomas are tumours of the lymphatic system, generally taking a form localised to the lymph nodes, with often very good treatment outcomes. Cutaneous T-cell lymphoma (CTCL), is 'atypical' in that it originally arises in the skin (rather than in the lymph nodes) and, although it often has a relatively slow course, complete cure is rarely possible. CTCL usually takes the form of patches or plaques on the skin, but often progresses to a nodular form as the disease progresses. In advanced stages, it contributes to involvement of the lymph nodes as well as other organs. Cutaneous T-cell lymphoma leads to skin ulcers, intractable itching of the skin, and pain, which significantly reduce the quality and comfort of life. CTCL is a rare disease, with an incidence of 2.2 patients per 10,000 people (the incidence threshold for a rare disease adopted by the European Medicines Agency is 5 patients per 10,000 people). According to data held by Prof. Małgorzata Sokołowska-Wojdyło, MD, PhD, from the Department of Dermatology, Venereology and Allergology, GUM, there are approximately 2,000 patients with CTCL in Poland. In turn, NFZ data indicate that around 60-70 people are treated each year in the drug programme for CTCL. A large proportion of patients are still of working age at the time of diagnosis - about half of patients are diagnosed under the age of 55-65 years (depending on the CTCL subtype). Patients are an extremely small group who need support in many areas of their lives. Therefore, in order to draw attention to the needs of CTCL patients, a study will be set up to determine what impact the disease has on daily functioning and to help patients come to the forefront of public awareness.
A problem we cannot see
As Prof Sokołowska-Wojdyło points out, establishing the diagnosis of CTCL can be difficult because cutaneous lymphoma can be confused with other common skin diseases. Often the symptoms of the neoplasm can be similar to the skin lesions of psoriasis or eczema. The most common cutaneous lymphoma, mycosis fungoides, may be heralded by pruritus, as well as erythematous and infiltrative lesions occupying areas not exposed to sunlight, and sometimes erythroderma, i.e. generalised inflammation of the skin (the skin is red almost all over the body). Most lymphomas originating in the skin are characterised by a long-term course. Some progress gradually, leading to painful tumours with breakdown and, in later stages, involvement of lymph nodes and internal organs - points out the expert, who specialises in the treatment of patients with cutaneous lymphoma from the Department of Dermatology, Venereology and Allergology in Gdansk.
When skin lesions occur, a dermatologist should be consulted to rule out or confirm a diagnosis of lymphoma. In the majority of cases of CTCL, the diagnosis is established by a dermatologist and a pathomorphologist, as, in addition to the clinical picture, the basic investigation for the diagnosis of CTCL is the histopathological evaluation of a skin section, in the case of enlarged lymph nodes, also the evaluation of a lymph node or a fragment of a - Professor Sokolowska-Wojdylo points out.
Initially I went to various dermatologists because of pimples and pustules on my body. My first suspicion was psoriasis. After a series of tests, I was diagnosed with psoriasis and was treated for this. Danuta Sarnek, a patient with CTCL, admits. It wasn't until six months later that I had a deep section taken of the lesions appearing on my skin and was diagnosed with T-cell lymphoma, mycosis fungoides to be exact.
Non-specific and difficult-to-diagnose skin lesions, but also inappropriate therapies, are an additional burden that patients have to face. Therefore, the involvement of a specialised, multidisciplinary team is crucial for rapid diagnosis and implementation of optimal therapeutic management.
– It is worth making the public aware of the noticeable changes in the patient's life in terms of emotional, professional, family and social spheres. The disease affects the patient's quality of life. The key is rapid diagnosis and the initiation of appropriate therapy, the choice of which can also affect the patient's daily life, not only because of the potential side effects of the drugs, but also because of the need for frequent visits to the doctor or phototherapy treatments - e.g. 3-4 times a week for several months). Multidisciplinary medical care (dermatologist, oncologist, haematologist - often the patient goes to two of them at the same time) is important. The choice of treatment method depends on availability (e.g. fast electron beam irradiation can only be performed in a few cities in Poland). It is important to have access to therapies that give long-lasting responses. Because of the impact of the disease on all aspects of life, when choosing a method of therapy at a given point in time, the patient's needs - family, workload and other obligations - must also be taken into account as far as possible. This can be afforded in the early stages of the disease, due to its chronic course. - Professor Sokołowska-Wojdyło points out.
Patients with CTCL can live a normal life, fulfil themselves professionally and pursue their passions. All they need is individualised therapy and to organise their lives in such a way that the disease does not interfere with their daily functioning.