9
Learning The Basics
is established. Both the clinical findings (based on both history and
examination) and the skin biopsy findings are essential for diagnosis.
Physicians will examine lymph nodes and often order blood tests including
a test for Sézary cells in the blood. Other screening tests such as computed
tomography (CT) or positron emission tomography (PET) scans may be
indicated, depending on the patient’s history.
Sézary Syndrome (SS)
Sézary syndrome is a less common but more aggressive type of CTCL that
is related to MF but presents with very severe itching, total body redness
(erythroderma), intense scaling of the skin and frequent hair loss. Lymph
nodes are usually enlarged, and the malignant T-cells found in the skin are
also seen circulating in the bloodstream.
SS is the only type of CTCL that always affects the skin and the blood. The
skin may be red from head to toe. Tumor cells are found in the blood, and
lymph nodes are larger than usual. The skin may be hot, sore, extremely
itchy, occasionally flaking and burning. Oozing of clear fluid from the skin
is common. Because much heat is lost through the skin, people often feel
cold. Symptoms may be accompanied by changes in nails, hair or eyelids.
Approximately 15% of patients with CTCL have SS. This disease usually
occurs in adults older than 50 and is found more in men than women.
Both MF and SS are rare cancers requiring a definitive diagnosis in order
to proceed with treatments. Like all cancer diagnoses, both are classified
according to stage, which defines how advanced the disease is at the time of
diagnosis.
Other Types of CTCL
Lymphomatoid Papulosis (LyP) is a disease of the immune system that
manifests with self-healing small bumps and spots on the skin that come
and go. It can be persistent with frequent, recurring eruptions or it can
disappear for an extended period of time before showing up again. Stress is
often reported to trigger the breakouts.
LyP belongs to a family of conditions called primary cutaneous CD30-
positive lymphoproliferative disorders (pcCD30+LPD). The name
lymphoproliferative disorder is used to define a broad range of diseases of
the immune system that share a common biology (in this case the presence
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