FACT SHEET
Plasma cell leukaemia (PCL)
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Last updated: May 2015
Plasma cell leukaemia (PCL) is a type of cancer
that affects the plasma cells and belongs to a
family of disorders called the plasma cell
dyscrasias, the most common of which is
multiple myeloma.
Plasma cells are a type of white blood cell involved in defending the body
against disease and infection. Plasma cells originate from lymphocytes and
are involved in the production of antibodies. PCL is generally more
aggressive than myeloma and usually requires more intensive monitoring
and treatment.
What is PCL?
The simple definition of PCL is an unusually high level of abnormal plasma
cells in the bloodstream. In PCL, abnormal plasma cells make up more than
20% of the total number of white blood cells present in the peripheral
(circulating) blood. This is compared to myeloma where the abnormal
plasma cells remain in the bone marrow. About 60% of people present with
the features of PCL at their first visit. This is called primary PCL where there
is no history of myeloma. About 1 in 100 patients with myeloma will
eventually develop a leukaemic transformation, called secondary plasma cell
leukaemia, which is thought to be related to the person with myeloma
having a greater number of genetic abnormalities. Unfortunately, when this
happens, the outlook may be poor. At this stage there is no way of
predicting whether someone with myeloma will transform into PCL.
What causes it?
Who develops PCL?
PCL is rare. The average age of diagnosis is 67 years old and almost twothirds of people with PCL are older than 60 years when diagnosed 1. PCL is
very rare under the age of 30. People with primary PCL tend to be at the
younger end of the spectrum.
What causes PCL?
The exact causes of PCL and myeloma are not fully understood. It is thought
to be started by a series of genetic changes during the development of a
plasma cell that leads to the plasma cell’s uncontrolled growth. However,
what triggers these changes is not known. Risk factors that may play an
important part include exposure to radiation, industrial and environmental
factors.
What are the symptoms and complications of PCL?
PCL patients normally present with similar symptoms and complications to
myeloma, however, these tend to be more severe. Signs and symptoms can
include:
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Bone pain
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Fatigue
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FACT SHEET
Plasma cell leukaemia (PCL)
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Low red blood cells (anaemia)
High white cell count (leucocytosis)
Recurrent infections
Bleeding (thrombocytopaenia)
High blood calcium levels (hypercalcaemia)
Kidney damage
Weakened bones with lytic bone lesions and or fractures.
People diagnosed with PCL also may have an enlarged liver or spleen, caused by a large number
of abnormal plasma cells accumulating and being deposited in these organs. This is more common
in primary than in secondary PCL.
How is PCL diagnosed?
PCL is diagnosed by the number of abnormal plasma cells in the blood. A diagnosis is made when
there are more than 2 million abnormal plasma cells per millilitre of blood, or when abnormal
plasma cells make up more than 20% of the total number of white blood cells present in the
blood. The diagnosis must be confirmed by bone marrow examination. The other two features,
usually seen in the blood in PCL, are the presence of abnormal plasma cells and reduced numbers
of other blood cells due to the replacement of normal bone marrow tissue with plasma cells.
How is PCL treated and managed?
Current treatments for primary PCL may be similar to those used in myeloma and generally
includes an intensive bortezomib (Velcade®) based combination therapy with chemotherapy and
steroids at time of diagnosis followed by autologous stem cell transplantation. An allogeneic stem
cell transplant may be considered in younger, fitter patients as part of front-line treatment.
Most secondary PCL patients already will have had several anti-myeloma treatments and some
people may have become refractory (resistant) to these. For these patients, more intensive
treatments using combinations of chemotherapy drugs, including cyclophosphamide, doxorubicin,
vincristine, etoposide and cisplatin, together with the steroids, dexamethasone or prednisolone,
may be considered.
Supportive treatment is important to help prevent or reduce the symptoms and complications of
PCL. These may include painkillers, blood transfusions and antibiotics.
In the future, with more clinical research into the plasma cell cancers, the development of more
effective therapies may lead to better responses and improved duration of responses for people
with PCL. There also may be a better understanding of the causes and the risk factors involved,
which ultimately will help optimise the selection of treatment strategies to help with disease
control.
Reference:
1
Ramsinggh G, Maehan P, Luo J, Vij R, Morgensztern D. Primary plasma cell leukaemia: a
surveillance, epidemiology, and end results database analysis between 1973 and 2004. Cancer
115 (24), 5734-5739. Analysis of the Surveillance, Epidemiology, and End Results (SEER)
database evaluating characteristics and survival of 291 patients with plasma cell leukaemia.
For more information, freecall 1800 620 420
email [email protected] or visit www.leukaemia.org.au
Leukaemia · Lymphoma · Myeloma · Related Blood Disorders | www.leukaemia.org.au