Angiocentric Cutaneous T-Cell Lymphoma of Childhood

Angiocentric Cutaneous T-Cell Lymphoma of Childhood

Article
Skin Care
Diseases & Conditions
+2
Contributed byLester Fahrner, MD+1 moreFeb 04, 2022

The topic Angiocentric Cutaneous T-Cell Lymphoma of Childhood you are seeking is a synonym, or alternative name, or is closely related to the medical condition Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma.

Quick Summary:

  • Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma is a rare T-cell non-Hodgkin’s lymphoma that is observed in children. The skin lesions (papules and vesicles) are observed on the sun-exposed areas
  • This is a type of primary cutaneous non-Hodgkin lymphoma, which means that it is a lymphoma that originates first in the skin. It can later involve other parts of the body including the lymph nodes and bone marrow; in which case it is called systemic Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma
  • The cause of the condition is not well-established, though certain genetic mutations are suspected. However, Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma is known to be associated with chronic exposure to sun and Epstein-Barr viral infections
  • A treatment may be undertaken depending upon the signs and symptoms observed and this could be skin-directed treatment and/or systemic treatment, when the whole body is affected
  • In some children, the condition progresses rapidly, while in others the progress is slower. Nevertheless, in general, the prognosis of Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma is poor even with treatment

General information on lymphoma and lymphocytes:

  • Lymphoma is a type of cancer stemming from uncontrollably dividing lymphocytes (type of white blood cells). There are two types of lymphomas:
    • Hodgkin lymphoma
    • Non-Hodgkin lymphoma
  • Lymphocytes are the main white blood cells found in the lymph, which is the fluid of the lymphatic system; just as blood is the fluid of the circulatory system
  • Lymphocytes are made in bone marrow, and can develop into either B-cells or T-cells. T-cells mature in the thymus; Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma arises from cancerous T-cells
  • Lymph results from filtration of blood as it travels to and from tissues. Lymph is colorless because it lacks red blood cells; instead, it contains lymphocytes. It is central to the immune system
  • There are 3 different kinds of lymphocytes:
    • T-lymphocytes or T cells: They help combat infections and abnormalities within the cells (cell-mediated immunity). They fight viruses and cancerous cells
    • B-lymphocytes or B cells: They produce antibodies that are bodily defense proteins, which target foreign invaders outside the cells (humoral immunity). They fight bacterial cells, cell fragments, and other immunogenic elements
    • Natural killer cells or NK cells: They perform diverse functions related to both cell-mediated and humoral immunity. They also scout for cancer cells, a process called immune surveillance

Please find comprehensive information on Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Lester Fahrner, MD picture
Reviewed by

Lester Fahrner, MD

Chief Medical Officer, DoveMed Team

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