Blastic plasmacytoid dendritic cell neoplasms (BPDCN) are a very rare type of cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs that often shows features of both a lymphomacancers of the lymphatic system and a leukaemiacancer of blood and/or blood forming tissues. It was previously referred to as natural killer (NK) cell leukaemia/lymphoma.
BPDCN develops from precursor (or blastic) plasmacytoid dendritic cellsthe basic structural and functional unit of all living things (pDCs), which are responsible for producing type I interferons. Interferons are a group of proteins that alert the immune system to viruses in the body. In response to interferon production, the immune system produces killer immune cells to fight these viruses.
BPDCN is slightly more common in males and tends to be diagnosed in people over 60. However, anyone can develop this disease.
Treatment
When cancers are detected, they are staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you. However, because of how rare BPDCNs are, there is currently no standard staging and grading system for this disease. Instead of staging and grading, your doctor will recommend a treatment plan based on the following factors:
- Cancer location.
- Whether or not the cancer has metastasised.
- Your age.
- General health.
- Your treatment preferences.
Your doctor may also recommend genetic testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, which analyses your tumoura tissue mass that forms from groups of unhealthy cells DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment options for BPDCN may include:
- Cytotoxic medicationmedications that are toxic to living cells used to inhibit cancer growth.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Stem cell transplanta procedure that involves replacing unhealthy blood-forming cells (stem cells) with healthy stem cells.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
Because of how rare BPDCN is, there has been limited research done into the riskthe possibility that something bad will happen factors of this disease.
Symptoms
The symptoms of BPDCN often vary depending on location.
In most cases, patients with BPDCN will affect the skin. Some of the symptoms experienced may potentially include:
- Single or multiple lesions, most often on the arms, legs, face, or neck.
- Bruise-like lesions.
- Deep purple lesions.
- Fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
- Fever.
- Night sweats.
In rare cases, patients may also experience BPDCN in the bone marrowsoft, spongy tissue found in bones that makes blood cells or bloodthe red bodily fluid that transports oxygen and other nutrients around the body. Some of the symptoms experienced may potentially include:
- Lymphadenopathyswollen lymph nodes/glands, also known as adenopathy.
- Splenomegalyenlargement of the spleen.
- Hepatomegalyenlargement of the liver.
- Leptomeningeal disease (cancer in the cerebrospinal fluid).
- Cytopenialow levels of red blood cells, white blood cells or platelets in the blood; one or more blood cell type can be affected.
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Diagnosis
A diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results of BPDCN is very difficult, and the condition is often misdiagnosed.
If your doctor suspects you have BDPCN, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) Centre
The BPDCN centre at the Dana-Farber Brigham cancer centre is one of the only facilities to focus solely on the care and research of this disease. The centre is located in Boston, Massachusetts, and is staffed by a team of medical oncologists, pathologists, dermatologists, and other specialists who are dedicated to providing excellent care to patients with BPDCN. The team at the BPDCN centre conducts innovative and cutting edge research in order to provide effective and personalised treatment options for patients with this disease.
For more information, please refer to the Dana-Farber Cancer Institute link in the references below.