We are the combination of four hospitals: the General Hospital, the Children’s Hospital, the Women’s Hospital and the Traumatology, Rehabilitation and Burns Hospital. We are part of the Vall d’Hebron Barcelona Hospital Campus: a world-leading health park where healthcare plays a crucial role.
Patients are the centre and the core of our system. We are professionals committed to quality care and our organizational structure breaks down the traditional boundaries between departments and professional groups, with an exclusive model of knowledge areas.
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The commitment of Vall d'Hebron University Hospital to innovation allows us to be at the forefront of medicine, providing first class care adapted to the changing needs of each patient.
Myelofibrosis is included within the group of chronic myeloproliferative disorders. It may appear de novo (primary) or following polycythaemia vera or essential thrombocythaemia.
It is characterised by bone marrow fibrosis, progressive defect in blood cell production and marked presence of constitutional symptoms, as well as an enlarged spleen and liver that attempt to compensate for the production of red blood cells.
Approximately one third of patients have no symptoms. Diagnosis is made by studying alterations in control analyses.
The symptoms appear gradually, with marked tiredness, night time sweating, fever, loss of muscle mass, loss of appetite and abdominal pain being the most common. They are not exclusive symptoms of this disease, so if they are present, it is advisable to consult your GP who will refer you to the corresponding haematology department if they suspect a diagnosis of myelofibrosis.
It is a disease that can remain stable for a long time, or present in very symptomatic forms.
It is considered a rare disease, with a low incidence of 5-7 cases per million inhabitants per year. It mainly affects people aged 60-70 years.
Diagnosis begins with the study of the aforementioned symptoms, of alterations in the physical examination (such as an increase in spleen size) or of alterations in the analysis such as anaemia, decreased white blood cells and platelets, among others.
A bone marrow biopsy can be performed for diagnosis, which, together with the analysis, will allow the determination of risk factors for the progression of the disease, which will guide treatment.
Treatment is determined by the risk of disease progression, patient characteristics and the presence of symptoms.
The only curative treatment for the moment is blood stem cell transplant, which can be offered to a small group of patients, as it usually presents in older patients who are not candidates for this type of treatment.
Therefore, the main goal of treatment today is to control symptoms and prevent complications. Drugs such as erythropoietin, danazol and others, including blood transfusions, are used to control symptoms related with anaemia.
To control symptoms or increased spleen size, hydroxyurea and ruxolitinib are mainly used.
Clinical trials exist that seek to improve current treatment. Consult your haematologist to find out which are available.
It is usually monitored with analyzes.
Acute leukaemia involves abnormal cell growth in the haematopoietic system characterised by significant proliferation and accumulation of immature cells, firstly in the bone marrow and subsequently in the blood, with a great degree of clinical and biological heterogeneity. Acute leukaemias are clonal proliferations (tumour cells that originate from a single initial cell and accumulate various genetic mutations that result in development of the disease) of altered blood stem cells. In normal circumstances, multipotent stem cells give rise to haematopoietic cells, which give rise to blood cells via a process of cell proliferation and differentiation mediated by the cell’s own mechanisms and by the surrounding tissues. Under normal conditions, blood cells migrate to the blood and tissues and are indispensable for the body to function correctly.
In acute leukaemias, the accumulation of different genetic and molecular alterations gives rise to the progressive accumulation of these cells, which substitute normal blood cells in a process known as "hiatus leukemicus", whereby progenitor cells (blasts) do not mature and accumulate in the bone marrow and peripheral blood. The symptomatology may be very mild and non-specific initially, resulting mainly from the lack of blood cells and sometimes from tissue infiltration. These are very serious diseases that require chemotherapy treatment to control them and often a transplant of bone marrow progenitor cells.
In many cases there are at first no major symptoms. Any symptoms there are mainly derive from the lack of blood cells and include tiredness, bleeding, infections and on rare occasions lack of appetite, bone pain, breathing difficulty or neurological symptoms. A physical examination may reveal palpitations, bruises, bleeding from mucus membranes, fever, infiltration of gums or other organs (skin, spleen, liver, etc.).
The average age for acute leukaemia is generally 67 years, but it can affect people of any age. Acute leukaemia is the most common cause of abnormal cell growth in children, with lymphoid leukaemia being the most common. Myeloid leukaemia is more common in the adult population.
A suspected diagnosis is reached in a number of ways, including clinical history, physical examination and a blood test. The diagnosis is confirmed using bone marrow aspiration in which we study neoplastic cells (blasts) under a microscope, as well as conducting multiparametric flow cytometry, cytogenetic analysis and molecular biology tests.
Based on chemotherapy. New drugs are currently being developed, such as immunotherapy or treatment against specific biological alterations (personalised treatment). If not contraindicated, a transplant of haematopoietic progenitor cells may be required once the response has been reached. Therapeutic strategies are adapted on the one hand to the patient’s situation (age, concomitant diseases, etc.) and on the other hand to the biological characteristics of the disease.
Full blood test and bone marrow aspiration.
Unfortunately, there is no way of preventing acute leukaemia from developing. The mechanisms that lead to a person developing this disease are not exactly known. We do know some factors that may be related, such as chemotherapy or radiotherapy in the past or exposure to certain toxins. A predisposition in some congenital diseases has also been observed, as well as cases where there is a family history of the disease.
The most common form is chronic myeloid leukaemia and this sheet refers exclusively to this form of leukaemia. It is a form of abnormal cell growth (neoplasia) that originates in immature multipotent blood cells (stem cells) and gives rise to red blood cells, platelets and white blood cells.
It is characterised by cellular proliferation of white blood cells with cytogenetic alteration that consists of the appearance of an abnormal chromosome known as the Philadelphia chromosome. The Philadelphia chromosome is also seen in other processes, such as acute lymphoblastic leukaemia, and diagnostic differentiation is required to distinguish which process is occurring. Chronic myeloid leukaemia may also involve red blood cell and platelet disorders, and is characterised by its chronicity. This disease serves to illustrate the revolution that has taken place in the last few years with targeted therapies in abnormal blood cell growth.
These are not very specific: Usually marked by tiredness, anaemia, loss of appetite.
This disease has a low incidence (1-1.5/100,000 pop./yr) and low mortality (more than 90% survival in the last 5 years). It mostly affects middle-aged people (40-50 years), although it can occur at any age.
A physical examination often reveals spleen growth. Blood tests show a very significant increase in white blood cells and bone marrow aspiration should be conducted to confirm. Genetic and molecular biology tests are also conducted.
The treatment most used is tyrosine-kinase inhibitors. It is an oral, chronic and relatively well tolerated treatment, including Imatinib: it is the first drug that was described, and the most commonly used. Side effects include submalar skin pigmentation (aesthetic problems), nausea and vomiting, which are often mild.
As there is some resistance to this drug, new drugs need to be developed to stop the other proliferation pathways of the affected cells.
Physical examination, blood test, bone marrow aspiration.
A tumour is an abnormal growth of tissue. In the case of orbital tumours, this growth is located in the tissues around the eye, which may be muscles, bones, fat, the lacrimal gland, nerves and blood vessels. They are rare tumours of several different types that may appear at any age. Orbital tumours may be benign or malignant. Benign tumours may cause pain due to compressing or displacing the different structures in the eye socket. Malignant tumours, on the other hand, as well as spreading to neighbouring tissue, may produce metastasis in other unconnected organs or lymphatic nodules.
The most common symptom is a protrusion of the eyeball out of its socket, known as “exophthalmos”. However it can also cause loss of vision due to compression of the optic nerve, double vision, pain and can limit the movement of the eyeball.
In some cases, tumours may be present in the eye socket for an entire lifetime with no symptoms.
It is hard to know the exact number of people affected by orbital tumours as it is a rare kind of tumour that includes several variants.
Benign tumours are the most common; capillary haemangiomas and dermoid cysts in children, and cavernous haemangiomas in adults.
The most common malignant tumours in children include rhabdomyosarcoma, and in adults lymphoma cancers of the lacrimal gland and metastases.
Imaging studies (CT and nuclear magnetic resonance scans) allow precise location of the tumour, its size to be measured and certain biological characteristics to be known. This information, together with the patient's age and the speed of the tumour's growth, enables an initial assessment of whether or not it is malignant.
A definitive diagnosis is made after a biopsy of part or all of the tumour.
In most cases, the main treatment is surgery to remove the tumour and therefore avoid the damage it may cause if left to grow within the eye socket by compressing or displacing the eyeball and other structures.
Modern-day orbital surgery techniques allow extraction of the tumour by making small incisions in areas that are hidden or not very visible. This enables faster postoperative recovery.
In the case of malignant tumours, different combinations of surgery, radiotherapy and chemotherapy are used. It should be noted that regular check ups are needed after treatment.
Where there are no symptoms, observation and monitoring of the speed of growth is usually sufficient.
There are currently no preventative guidelines to reduce the risk of orbital tumours.
These syndromes are a group of diseases characterised by insufficient blood cell production (anaemia, neutropenia and thrombocytopenia), constitutional malformations and the risk of cancer.
They are usually diagnosed in childhood but there some cases diagnosed in adults. Specifically, these syndromes are: Fanconi anaemia, dyskeratosis congenita or selectively severe congenital neutropenia, Diamond-Blackfan anaemia, Diamond-Shwachman syndrome, and amegakaryocytic thrombocytopenia.
Malformations caused by inherited bone marrow failure syndromes affect the skin, bones, heart and digestive system as well as the urinary system, the central nervous system, and others. They may also affect the area around the bone marrow.
Besides this, they also increase the predisposition to cancers such as acute myeloid leukaemia, myelodysplastic syndrome and squamous carcinoma of the head, neck and reproductive organs.
These syndromes have important biological pathways in common related to cell growth and division such as the activation of the p53 gene that is responsible for halting the cell cycle, cell ageing and cell death. In addition, mutations have been identified in more than 80 genes.
Although this is treated in childhood, once the patient reaches adulthood monitoring is very important. Early diagnosis of these syndromes is essential to ensure the patient receives appropriate treatment. The aim is to minimise toxicity at the same time as allowing genetic counselling, and implementing strategies for cancer prevention and monitoring. This treatment should focus on treating the bone marrow failure, but also any constitutional malformations and extra-haematological manifestations, in addition to cancer treatment.
A multidisciplinary team of specialists with extensive experience of these diseases is necessary to ensure optimum patient care.
The first symptoms are related to a lack of blood cell production:
There are also signs related to malformations such as:
Estimated incidence rates of the different syndromes are:
The usual tests to detect inherited bone marrow failure are:
The following methods are used to treat these pathologies:
To prevent these diseases it is important to avoid smoking, alcohol, sun exposure, and to have a balanced diet. In addition, a cancer prevention programme should be followed with regular visits to the Cancer Prevention Unit and the Ear, Nose and Throat, Maxillofacial and Gynaecology departments.
Hematopoietic Stem Cell Transplant (HPSCT) is the definitive treatment for many primary immunodeficiency disorders (PID). It is a total replacement of the blood cells in our body. It is also called a bone marrow transplant (BMT).
The aim of this treatment is to regenerate a haematopoiesis (the process by which the different types of blood cells form, mature, and circulate from stem cells), which has been eliminated by administering drugs or ionizing radiation, followed by the implantation of the donor's immune system, which is able to recognise and attack the malignant cells in the patient.
In this way, the bone marrow stem cells (factory of the defences) are changed for those of a healthy person (the donor). To undergo this process the patient is admitted to hospital for between one and three months.
1st step:
2nd step:
3rd step:
Chemotherapy consists of combining different types of medications that destroy cancer cells in different ways. It acts on these cells that are rapidly reproducing, cancer cells and healthy cells, too.
Chemotherapy is administered in different ways and for various reasons:
The human body is made up of different cells that each have a certain function. Cancer begins when a group of cells reproduces very quickly and uncontrollably. This affects the cells’ function and, therefore, stops the body functioning normally.
Chemotherapy acts on these cells, which may or may not be cancerous, that are rapidly reproducing. This causes side effects, which will depend on the medication, dose, duration and each individual person.
Chemotherapy can be intravenous or oral, meaning it can be administered by the vein or by the mouth. The first option is the most common.
To administer this treatment, sometimes a catheter is left in place that is then connected to a disc below the skin. The medication is administered through this device. This catheter is called a port-a-cath, although there are more types of catheters. The way the vein is accessed depends on the characteristics of the person and the duration of the treatment.
Chemotherapy is applied at intervals and the duration depends on the type of programme, control and treatment.
Chemotherapy can produce the following side effects:
The Medical Oncology Department was created in 1995 to meet the new demands of oncology care and to provide a coordination centre for different departments to create fully interrelated multidisciplinary healthcare teams.
Since its creation, it has experienced significant quantitative and qualitative growth in the areas of patient care, teaching and research perspective, to become a reference service in Europe thanks to a translational model in which care and research work in coordination to transfer laboratory discoveries to patients.
The complexity of the diagnoses and treatments performed by the Medical Oncology Department requires working in multidisciplinary teams of specialists, with the aim of providing an integrated approach from diagnosis to the end of treatment. For this same reason, we work in expert cancer-specific committees and assess each case as a team to determine the appropriate treatment for each person. If you train with us, you will be working with highly qualified professionals and the latest generation technology. Training is backed up by our own training programmes and through collaboration with centres of recognised quality and prestige.
Medical Oncology training itinerary
The core of this teaching unit is provided by the Medical Oncology Department, with participation from Haematology, Internal Medicine, Radiation Oncology, Radiology, Pathological Anatomy, Infectious Diseases and Palliative Care, the Intensive Care Unit and the A&E Department. The Unit can accommodate three residents per year. Residents’ training in Medical Oncology takes five years in total. The two first years are spent on core training, with the following three years dedicated specifically to specialisation.
Residents in medical oncology are expected to have in-depth knowledge of preventative, diagnostic and therapeutic choices for cancer. For this reason it is important that they continuously update their knowledge of cancer biology. To this end, they must take part in research projects that promote excellence in research. They will also have the opportunity to become familiar with the main lines of research in the Department and to take part in some of them. Over the course of training, residents learn to have a critical and open approach to the high volume of clinical studies and advances in the specialisation, whilst always keeping ethical considerations at the forefront of their work.
Why do your residency at Vall d’Hebron?
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