Blastic NK-cell lymphoma

BLASTIC NK-CELL LYMPHOMA

Epidemiology : occurs in older patients (median 52 yrs)
Aetiology : HHV-4 / EBV is negative
Symptoms & signs : multiple pigmented or erythematous plaques (60-70%)^{ref1,
ref2,
ref3}. Marrow involvement is frequent (50-75%)
Laboratory examinations :

cytomorphology : tumor cells have a blastic appearance and involve the superficial and deep dermis. Subcutaneous infiltration is also present but has a diffuse pattern rather than the lobular pattern seen in subcutaneous panniculitis-like T-cell lymphoma. There is no evidence of angioinvasion/angiodestruction and necrosis. Cytoplasmic granules are absent or inconspicuous in 75%
immunophenotype : CD2^+/-, sCD3^-, CD4⁺, CD5^-/+, CD7^-/+, CD33^{-/+ (weak)}, CD56⁺, CD57, TdT^+/-, TIA-1^-/+, CD34^-/+

Prognosis : the clinical course is aggressive; some progress to overt leukemia
Therapy : autologous peripheral blood stem-cell transplantation (APBSCT)

^ref

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