T-cell lymphomas: a guide for the patient
BY.
Dr.
FRANCESCA BONELLO
Medical Surgeon Specializing in Hematology
Receiving a diagnosis of T lymphoma can generate questions, fears, and a strong sense of loss. In this guide, we want to accompany you with clear and reliable information to help you understand what T lymphoma is, what the treatment options are, and what aspects are important to know in order to approach the journey with greater awareness and peace of mind. Getting informed is the first step in feeling like an active participant in care choices.
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What are T-cell Lymphomas
T-cell lymphomas are a group of blood cancers, or hematologic tumors, that originate from T lymphocytes, a type of white blood cell that under normal conditions is responsible for recognizing and fighting infection. T-cell lymphomas are part of Non-Hodgkin ly mphomas along with B-cell lymphomas, but unlike the latter, they are much rarer and diagnosis is often more complex.
Are they rare tumors?
Yes, T lymphomas are rare cancers; in fact, about 1-2 new cases are diagnosed per 100,000 population each year, although in some geographical areas, such as South America and parts of Asia, they are more common. T-cell lymphomas make up about 7-10% of all Non-Hodgkin’s lymphomas. Precisely because of their rarity, they often require diagnosis and clinical management in specialized centers.
What are the most common symptoms?
- Symptoms may vary depending on the specific type of lymphoma but the most common are:
- Enlargement of lymph nodes the different places on the body, for example, neck, armpits, groin
- Persistent fever, often in the evening or night hours, persisting even after antibiotic therapy
- Sweats often at night
- Unintentional weight loss, without having reduced food intake
- Marked fatigue
- There are also T-cell lymphomas with exclusively cutaneous involvement (such as mycosis fungoides), which in that case are manifested by skin lesions of different appearance that can arise at any body site and tend to grow to change over time without regressing.
How do you arrive at the diagnosis?
In the presence of one or more of these symptoms, the first step in arriving at a diagnosis is to see your doctor for a general examination and possible blood tests or ultrasound of enlarged lymph nodes. To make the diagnosis, a biopsy of the suspected lymph node or organ (e.g., skin) must be taken for histological examination.
Histologic diagnosis of T lymphoma can be complex, so it is always a good idea to perform the examination in specialized centers experienced in recognizing these diseases.
Biopsy allows identification of the T lymphoma subtype and molecular tests that help in the choice of treatment.
After diagnosis, staging is performed, that is, theextent of the disease is defined by total body CT scan, PET scan, and by bone marrow biopsy (because lymphoma cells can also be located in the bone marrow).
Are there different types of T lymphoma?
- Absolutely, there are many subtypes of T-cell lymphoma, each with different clinical behavior and characteristics (e.g. greater or lesser aggressiveness). In some cases different subtypes require different treatment approaches, so it is very important to identify them.
- The most frequent subtype is peripheral T-cell lymphoma not otherwise specified, followed by angioimmunoblastic T lymphoma and anaplastic T lymphoma.
- In some geographical areas such as Asia, however, extra-nodal T-NK lymphomas are more common.
- Then there are cutaneous T-cell lymphomas such as mycosis fungoides, which accounts for about 60% of all cutaneous lymphomas and has a usually indolent course and is often managed in specialized dermatology centers.
What are the treatment options?
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Therapy depends on the subtype of T lymphoma, the stage or extent of the disease, and the patient’s general condition and age .
In general, for systemic forms, the standard approach is a multi-drug combination chemotherapy, which can be followed by transplantation of autologous (i.e., the patient’s own) hematopoietic stem cells .
A combination of chemotherapy and immunotherapy, with monoclonal antibodies directed against specific targets present on the tumor cell, is planned for some subtypes. Sometimes radiation therapy can also be combined, but this is not a standard approach. - The choice of therapy is made by a multidisciplinary team. For cases that relapse or do not respond to first-line therapy, there are several biologic drugs available with varying efficacy, and, if the patient’s condition permits, allogeneic (i.e., donor) stem cell transplantation may be used. The relapsed forms are often more aggressive and whenever possible it is always good to consider inclusion in clinical trials with experimental drugs.
Can T lymphoma be cured from?
Yes, T lymphoma can be cured. Some subtypes have less aggressiveness and are more easily cured by first-line therapy, while other subtypes have more aggressiveness and tend to recur more frequently. Outcomes have improved in recent years thanks to the introduction of new drugs and the personalization of treatment.
Are there any new therapies coming?
Yes, research is very active in this field. Suffice it to say that until a few years ago for a subtype of T lymphoma called anaplastic large cell T lymphoma, only chemotherapy was used, whereas today it is possible to add to chemotherapy a monoclonal antibody conjugate called brentuximab-vedotin, which has greatly improved remission and cure rates.
Can people participate in clinical trials at INOC?
Yes, some clinical trials for T lymphomas are active and soon to be activated at INOC – Istituto Nazionale Oncologico Candiolo, especially for relapsed or nonresponsive forms of first-line therapy.
