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Cutaneous T-Cell Lymphomas

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

NORD is very grateful to Javier Munoz, MD, FACP, Hematology and Oncology, Banner MD Anderson Cancer Center, Gilbert, Arizona, for assistance in the preparation of this report.

Synonyms of Cutaneous T-Cell Lymphomas

  • CTCL

Disorder Subdivisions

  • Granulomatous Slack Skin
  • Lymphomatoid Papulosis
  • Mycosis Fungoides
  • Pagetoid Reticulosis (Woringer-Kolopp Disease)
  • Primary Cutaneous Anaplastic Large Cell Lymphomas
  • Sezary Syndrome
  • Subcutaneous Panniculitic T-Cell Lymphoma

General Discussion

General Discussion
Summary
Cutaneous T-cell lymphomas (CTCLs) are a group of disorders characterized by abnormal accumulation of malignant T-cells in the skin potentially resulting in the development of rashes, plaques and tumors. CTCLs belong to a larger group of disorders known as non-Hodgkin's lymphomas (NHLs), which are related malignancies (cancers) that affect the lymphatic system (lymphomas).

Introduction
Functioning as part of the immune system, the lymphatic system helps to protect the body against infection and disease. It consists of a network of tubular channels (lymph vessels) that drain a thin watery fluid known as lymph from different areas of the body into the bloodstream. Lymph accumulates in the tiny spaces between tissue cells and contains proteins, fats, and certain white blood cells known as lymphocytes.

There are two main types of lymphocytes: B-lymphocytes, which may produce specific antibodies to "neutralize" certain invading microorganisms, and T-lymphocytes, which may directly destroy microorganisms or assist in the activities of other lymphocytes. CTCLs result from errors in the production of T-lymphocytes or transformation of T-lymphocytes into malignant cells. In CTCLs, abnormal, uncontrolled growth and multiplication (proliferation) of malignant T-lymphocytes result in accumulation of these lymphocytes in the skin. In some cases, malignant lymphocytes may spread to affect the lymph nodes and eventually to other bodily tissues and organs, potentially resulting in life-threatening complications. The specific symptoms and physical findings may vary from case to case, depending upon the extent and region(s) of involvement, the specific type of CTCL present, and various additional factors.

Non-Hodgkin's lymphomas, such as CTCLs, may also be categorized based upon certain characteristics of the cancer cells as seen under a microscope and how quickly they may tend to grow and spread. For example, CTCLs may be characterized as "low-grade" (or indolent) lymphomas, which tend to grow slowly and result in few associated symptoms, or "intermediate-grade" or "high-grade" (aggressive) lymphomas, which typically grow rapidly, requiring prompt treatment. Most cases of CTCL, especially the classic form (mycosis fungoides), are slow-growing (indolent) lymphomas.

Cutaneous T-Cell Lymphomas Resources

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