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adult T-cell lymphoma/leukemia (ATL/L)
Etiology:
- HTLV-1
Epidemiology:
1) *average age of onset: 35-55 years
2) males > females
3) occurs most commonly in Japanese & blacks
4) geographic distribution
a) southwestern Japan (Kyushu)
b) Africa
c) Caribbean islands
d) southeastern United States
5) transmission:
a) sexual intercourse
b) perinatal transmission
c) exposure to blood products
6) *leukemia develops 20-40 years after exposure
Pathology:
1) dermatopathology
a) perivascular &/or diffuse infiltrates
b) large, abnormal lymphocytes in the upper to middle dermis
c) epidermis is often spared
d) intradermal infiltration & microabscesses containing many large, abnormal lymphocytes +/- giant cells
2) hypercalcemia due to osteoclastic bone resorption
3) 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:
1) skin lesion (50% of patients)
a) multiple papules/nodules (firm)
b) +/- purpura
c) large plaques
d) +/- ulceration
e) generalized erythroderma
f) poikiloderma
g) papulosquamous lesions
h) diffuse alopecia
i) distribution: trunk > face > extremities
2) hepatomegaly (50%)
3) splenomegaly (25%)
4) lymphadenopathy (75%)
- spares mediastinal nodes
Laboratory:
1) complete blood count (CBC)
a) WBC ranges from normal to 500,000/uL
b) peripheral smear: examine for Sezary cells
2) serum chemistries
- hypercalcemia 25% at time of diagnosis, 50% during course of disease
3) serology:
a) seropositive ELISA & western blot for HTLV-1
b) IV drug abusers may be positive for both HIV & HTLV-1
Differential diagnosis:
- cutaneous T-cell lymphoma (mycosis fungoides)
Management:
1) prognosis
a) course may be smoldering or chronic
b) mean survival in patients with acute crisis:
1] hypercalcemia: 12 weeks
2] normocalcemia: 4 years
2) chemotherapy
a) 30% complete response
b) relapses common
3) obtain HTLV-1 serology of family members & sexual partners
Interactions
disease interactions
Related
granuloma faciale; eosinophilic granuloma of skin
leukemia cutis
mycosis fungoides/Sezary syndrome
sarcoidosis
Sezary cell
General
lymphoid leukemia
peripheral T-cell lymphoid neoplasm
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.
PMID: 27332907
http://www.nejm.org/doi/full/10.1056/NEJMcpc1512457