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Systemic Therapies

An Overview of Systemic Therapies - Pierluigi Porcu, MD

An Overview of Systemic Therapies

The term systemic therapy defines any drug treatment that, after absorption, reaches the bloodstream and is therefore distributed across the body “system.”

Targeted/ Biological / Immuno-therapies: Oral Corticosteroids

Corticosteroids are medications used to treat a variety of skin diseases that may be both acute and chronic in nature.

Targeted/ Biological / Immuno-therapies: Bexarotene (Targretin®) Capsules

Bexarotene is a retinoid, a type of synthetic vitamin A, that has been shown to be effective in CTCL when administered systemically in capsule form. Oral bexarotene has been approved for the treatment of all stages of CTCL. It may be given as a single agent or may be combined with other forms of therapy. Most patients require several months of this oral therapy before improvement is observed. Blood tests to monitor side effects are required while taking oral bexarotene.

Targeted/ Biological / Immuno-therapies: Extracorporeal Photopheresis (ECP)

ECP is an immunotherapy approved for CTCL and recommended for patients with blood involvement. That is, CTCL cells detected in the blood (Sezary Syndrome and Stage III Erythrodermic MF).

Targeted/ Biological / Immuno-therapies: Interferons (IFN)

Interferons (IFN) are naturally occurring compounds that are produced by human cells in response to many triggers of the immune system.

Targeted/ Biological / Immuno-therapies: Denileukin Diftitox (Ontak®)

Denileukin Diftitox (DD) is a targeted therapy. That is, it was designed to attack CTCL cells through a selected surface receptor and deliver a toxin (poison) that kills CTCL cells. DD is fusion toxin - a synthetic molecule that combines a portion of the interleukin-2 protein with the diphtheria toxin protein.

Targeted/ Biological / Immuno-therapies: Alemtuzumab (Campath-1H)

Alemtuzumab is a monoclonal antibody directed against CD52, an antigen (or surface marker) found on both B and T lymphocytes. The drug is used most often to treat chronic lymphocytic leukemia and has been used in the treatment of advanced CTCL. Alemtuzumab is given intravenously (in the vein) in a personalized treatment schedule for each patient.

A Primer on Extra Corporeal Photopheresis

Extracorporeal photopheresis (ECP) was the first therapy approved by the Food and Drug Administration (FDA) specifically for the treatment of cutaneous T-cell lymphoma (CTCL).

HDAC Inhibitors: Vorinostat

HDAC inhibitors target DNA associated proteins (histones) and allow the cell’s genes to be transcribed or expressed in a way that may help cancer cells die. Vorinostat is a histone deacetylase inhibitor (HDAC inhibitor) that is being used for the treatment of CTCL.

HDAC inhibitors: Romidepsin

HDAC inhibitors target DNA associated proteins (histones) and allow the cell’s genes to be transcribed or expressed in a way that may help cancer cells die.

Chemotherapies (Single & Multi-Agent): Methotrexate

Methotrexate is an anti-metabolite agent that is used in a variety of immune mediated diseases such as rheumatoid arthritis and psoriasis.

Chemotherapies (Single & Multi-Agent): Pentostatin

A kind of antibiotic, pentostatin is only used for chemotherapy, and helps to slow or stop the growth of cancer cells. Pentostatin has been used as a single agent for the treatment of advanced stages (Stage III and IV) of CTCL.

Chemotherapies (Single & Multi-Agent): Liposomal Doxorubicin (Doxil®)

Doxil‚ is a special formulation of doxorubicin, a drug that prevents cancer cells from growing by interfering with their DNA, the genetic material of the cell. A liposome is a microscopic sphere with layers of fat surrounding it. Giving doxorubicin in this form minimizes some of the side effects, and allows the drug to be more active. The drug has been used in many forms of cancer including Kaposi’s sarcoma, ovarian cancer, and advanced CTCL. Doxil is administered in the outpatient oncology setting by intravenous infusion every 2-4 weeks.

Chemotherapies (Single & Multi-Agent): Gemcitabine

Gemcitabine is a chemotherapy drug that interferes with cancer cell’s production of DNA, the genetic material of the cell. The drug has been used by itself and with other drugs in the treatment of many kinds of cancer including lung cancer, pancreatic cancer, and advanced CTCL.

Chemotherapies (Single & Multi-Agent): Cyclophosphamide-Oral

Cyclophosphamide is a chemotherapy in the "alkylator" group of chemotherapy drugs that may stop cancer cells from growing by interfering with the cells’ DNA (the genetic material in cells). It is used in the treatment of many cancers including breast cancer and lymphomas, as well as in the treatment of autoimmune diseases like systemic lupus erythematosis.

Combination chemotherapy (CHOP regimen)

Combination chemotherapy with more than one chemotherapy agent is usually reserved for advanced stages (Stage III and IV) of CTCL or that are resistant to other forms of therapy or in the treatment of large cell transformation of CTCL.

Bone marrow/Stem cell Transplantation

Bone marrow/stem cell transplantation (BMT) and peripheral blood stem cell transplantation (PBSCT) are considered in individual cases for CTCL.