Adult T-cell leukemia/lymphoma

Alternate names

ATLL; Adult T-cell leukaemia; Adult T-cell leukaemia/lymphoma (HTLV-1 positive); Adult T-cell lymphoma

Definition

(uh-DULT T-sel loo-KEE-mee-uh/lim-FOH-muh)An aggressive (fast-growing) type of T-cell non-Hodgkin lymphoma caused by the human T-cell leukemia virus type 1 (HTLV-1). It is marked by bone and skin lesions, high calcium levels, and enlarged lymph nodes, spleen, and liver. Also called ATLL.

Epidemiology

• ATLL and HTLV‐I are prevalent in Japan, the Caribbean basin, and certain regions of South America and Africa, and in immigrants from these countries to other regions. .
• It has also been recognised in patients originating from Iran and Central Europe.
• In the UK, ATLL is mainly, but not exclusively, seen in first or second generation immigrants of Afro‐Caribbean descent.

Cause

• It is aetiologically linked to the human T‐cell lymphotropic virus, HTLV‐I.
• HTLV‐I is detected in cells from virtually all cases of ATLL, with exceedingly rare exceptions.

Types

There are four basic clinical variants of ATL: acute (60% of cases), lymphomatous (20 % of cases), chronic (10% of cases) and smoldering (10% of cases).

Signs and symptoms

• The clinical features of ATL include generalized swelling of the lymph nodes (lymphadenopathy), increased liver and spleen size (hepatosplenomegaly), immunosuppression, high levels of calcium in the blood, lytic bone lesions (spots that appear as “holes” on a standard bone x-ray), and skin lesions.

Diagnosis

• The diagnosis should be based on a constellation of clinical features and laboratory investigations. .
• The latter comprise: lymphocyte morphology, immunophenotype, histology of the tissues affected in the pure lymphoma forms and serology or DNA analysis for HTLV‐I.

Treatment

• The best treatment for these patients is unclear and patients should be enrolled in clinical trials whenever possible.
• Medication may include CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) or EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide and prednisone).
• In some patients, a bone marrow transplant may be recommended..

NIH genetic and rare disease info

• NIH info on Adult T-cell leukemia/lymphoma

Adult T-cell leukemia/lymphoma is a rare disease.

Adult T-cell leukemia/lymphoma Resources
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Adult T-cell leukemia/lymphoma Resources
Need Help Finding a Doctor? Need help finding a doctor or specialist anywhere in the world? Explore our world directory. WikiMD's DocFinder can assist you in locating millions of physicians.	Medical Knowledge Access the latest research - Most recent articles Ongoing trials.

Translate to: East Asian 中文, 日本, 한국어, South Asian हिन्दी, Urdu, বাংলা, తెలుగు, தமிழ், ಕನ್ನಡ,
Southeast Asian Indonesian, Vietnamese, Thai, မြန်မာဘာသာ, European español, Deutsch, français, русский, português do Brasil, Italian, polski