Case Reports in Hematology / 2017 / Article / Tab 2

Case Report

Unusual Presentation of a Small-Cell Variant of Anaplastic Large-Cell Lymphoma Case: When a Septic Picture Is Not Sepsis

Table 2

Comparison of classic and small-cell variant ALCL.

Classic ALCL variantSmall-cell ALCL variant

Frequency70% of ALCL5%–10% of ALCL

ALK rearrangementALK-positive and ALK-negativeALK-positive

MorphologyCharacterized with numerous large round or pleomorphic cells with horseshoe-shaped nuclei with multiple (or single) prominent nucleoli; these cells are also called “hallmark” cellsThe majorities of cells are small to medium in size and have clear cytoplasm and irregular nuclei; a minor population of “hallmark” cells can present singly or in cluster [5]

Peripheral leukemia involvementExtremely rareVery common [5, 6]

Bone marrow involvementRelatively infrequent (10%–30% of cases)Common; usually subtle bone marrow involvement with a small cluster of small lymphocytes and only rare scattered large tumor cells [5, 6]

Systemic involvementRelatively less infrequentFrequently associated with widespread disseminated disease [6]

Prognosis2-year survival of 73% 2-year survival of 50% and 3-year disease-free survival of 25% [3, 5]