adult T-cell lymphoma/leukemia (ATL/L)
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Etiology
Epidemiology
- *average age of onset: 35-55 years
- males > females
- occurs most commonly in Japanese & blacks
- geographic distribution
- southwestern Japan (Kyushu)
- Africa
- Caribbean islands
- southeastern United States
- transmission:
- sexual intercourse
- perinatal transmission
- exposure to blood products
- *leukemia develops 20-40 years after exposure
Pathology
- dermatopathology
- perivascular &/or diffuse infiltrates
- large, abnormal lymphocytes in the upper to middle dermis
- epidermis is often spared
- intradermal infiltration & microabscesses containing many large, abnormal lymphocytes +/- giant cells
- hypercalcemia due to osteoclastic bone resorption
- opportunistic infection & disseminated intravascular coagulation (DIC) are major causes of death
Genetics
- overexpression of PMAIP1
- PRDM16 isoform 4 is specifically expressed in adult T-cell leukemia
Clinical manifestations
- skin lesion (50% of patients)
- multiple papules/nodules (firm)
- +/- purpura
- large plaques
- +/- ulceration
- generalized erythroderma
- poikiloderma
- papulosquamous lesions
- diffuse alopecia
- distribution: trunk > face > extremities
- hepatomegaly (50%)
- splenomegaly (25%)
- lymphadenopathy (75%)
- spares mediastinal nodes
Laboratory
- complete blood count (CBC)
- WBC ranges from normal to 500,000/uL
- peripheral smear: examine for Sezary cells
- serum chemistries
- hypercalcemia 25% at time of diagnosis, 50% during course of disease
- serology:
- seropositive ELISA & western blot for HTLV-1
- IV drug abusers may be positive for both HIV & HTLV-1
Differential diagnosis
Management
- prognosis
- course may be smoldering or chronic
- mean survival in patients with acute crisis:
- hypercalcemia: 12 weeks
- normocalcemia: 4 years
- chemotherapy
- 30% complete response
- relapses common
- obtain HTLV-1 serology of family members & sexual partners
More general terms
Additional terms
- granuloma faciale; eosinophilic granuloma of skin
- leukemia cutis
- mycosis fungoides/Sezary syndrome
- sarcoidosis
- Sezary cell
References
- ↑ Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 540
- ↑ Lorenzo ME, Carter JB, Barnes JA, Nazarian RM. CASE RECORDS of the MASSACHUSETTS GENERAL HOSPITAL. Case 19-2016. A 65-Year-Old Man with End-Stage Renal Disease and a Pruritic Rash. N Engl J Med. 2016 Jun 23;374(25):2478-88. <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/27332907 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMcpc1512457