Mycosis fungoides- familial

Synonyms

2

Overview

Mycosis fungoides is a disease in which T-cell lymphocytes (a type of white blood cell) become malignant (cancerous) and affect the skin. This condition is one of the most common types of T-cell lymphoma. Mycosis fungoides is characterized by a scaly, red rash that develops on the skin, particularly on areas that are not usually exposed to the sun. The rash may last for months or years without causing any symptoms. Over time, a thin, reddened, eczema-like rash may develop, followed by thickened, red patches of skin. Finally, tumors form which may develop into ulcers and become infected. Mycosis fungoides is difficult to cure. Treatment is usually palliative, with the intention of relieving symptoms and improving the quality of  life.

Symptoms

  • Abnormality of lymphocytes
  • Dry skin
  • Eczema 
  • Lymphoma 
  • Neoplasm of the skin
  • Pruritus
  • Skin rash 
  • Alopecia 
  • Hypopigmented skin patches
  • Irregular hyperpigmentation 
  • Lymphadenopathy
  • Poikiloderma 
  • Abnormality of bone marrow cell morphology 
  • Abnormality of the eyelid 
  • Abnormality of the nail 
  • Edema 
  • Hepatomegaly 
  • Hyperkeratosis
  • Skin ulcer 
  • Splenomegaly
  • Erythema 
  • Skin plaque

Causes

The cause of MF is unknown. Most persons with MF have it for years and it can lead to death, but this is unusual. In the United States, MF strikes more than 1,000 people of all races annually; most are between ages 40 and 60.

Prevention

Information not available.

Diagnosis

Dermatologists work with hematologists, pathologists, radiologists and other experts to diagnose cutaneous T-cell lymphoma. Tests and procedures used to confirm a diagnosis include:

  • Physical exam. Your doctor will examine your skin for patchy, scaly regions or solid, raised growths. You will also be examined for signs that your lymph nodes or other organs might be affected.
  • Blood tests. Your blood may be tested to determine if it contains skin lymphoma cells. Doctors may use advanced laboratory tests to analyze your blood in order to better understand your condition, including flow cytometry and polymerase chain reaction testing.
  • Skin biopsies. A pathologist examines a small sample of skin removed during a biopsy to determine whether it contains cancer cells. Sometimes multiple skin biopsies are necessary in order to confirm your diagnosis. Advanced laboratory tests, such as immunohistochemistry and polymerase chain reaction, may be used to help your doctor understand your prognosis and select the most effective treatment.
  • Imaging tests. Imaging tests, such as computerized tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET), can help determine if cancer cells have spread to other areas of the body.

Prognosis

Mycosis fungoides (MF) has an indolent (low-grade) clinical course, which means that it may persist in one stage, or may slowly progress to another stage (from patches to thicker plaques and eventually to tumors) over years or sometimes decades. The condition is incurable in most affected individuals, with the exception of those with stage IA disease. The prognosis for each individual varies and is related to the disease stage at the time of diagnosis, the type and extent of skin lesions, and whether the disease is present in other areas of the body (extracutaneous).
Studies regarding the survival outcomes and prognosis have generally found that:

  • Stage IA disease at the time of diagnosis who undergo treatment have a normal life expectancy (e.g. that expected based on age, sex and race) -- a 10-year survival rate of 97-98%.
  • Stage IIB with cutaneous tumors have a median survival rate of 3.2 years (a 10-year survival rate of 42%).
  • Stage III (generalized erythroderma) have a median survival rate of 4-6 years (a 10-year survival rate of 83%).
  • Stage IVA (lymph nodes) or stage IVB (viscera) disease have a survival rate of less than 1.5 years (a 10-year survival rate of 20% for those with histologically documented lymph node involvement).
  • individuals with effaced lymph nodes, visceral involvement, and transformation to large T-cell lymphoma have an aggressive clinical course and usually die of systemic involvement or infections.

Other factors that may be associated with reduced survival and increased risk of disease progression include increased age, male sex, and increased lactate dehydrogenase (LDH).

Treatment

Treatment options may include:

  • Skin creams and ointments. Medicines can be applied to your skin in the form of creams, foams, gels and ointments. Corticosteroids can help control skin redness and itchiness. Chemotherapy and retinoids can be applied to the skin to attack cancer cells.
  • Light therapy. Light therapy involves exposing your skin to ultraviolet light. Sometimes a medicine is administered to make the cancer cells more sensitive to light. Healthy cells regenerate quickly, but cancer cells do not. Light therapy can use ultraviolet A light or ultraviolet B light.
  • Radiation therapy. Radiation therapy uses beams of radioactive particles to kill cancer cells. Radiation therapy for cutaneous T-cell lymphoma uses electron beams, which target the skin and don't affect internal organs. Electron beam radiation may be directed to a specific area or applied to all of the skin.
  • Targeted therapy. Mechlorethamine (Valchlor) - FDA-approved indication: Topical treatment of Stage 1A and 1B mycosis fungoides-type cutaneous T-cell lymphoma in patients who have received prior skin-directed therapy. 
  • Exposing blood cells to light. A procedure called extracorporeal photopheresis involves drawing blood from your body and treating it with a photosensitizing medicine. The blood is then exposed to ultraviolet light, which damages the cancer cells. Following this procedure, the blood is returned to your body.
  • Stem cell transplant. A stem cell transplant is a procedure to replace your diseased bone marrow with healthy bone marrow from a matched donor (allogenic stem cell transplant). During a transplant you'll receive chemotherapy drugs to suppress your diseased bone marrow. Then healthy stem cells are infused into your body where they travel to your bones and begin rebuilding your bone marrow.
  • Clinical trials. Clinical trials are research studies that give you access to the latest treatments for cutaneous T-cell lymphoma.