CD30 and the NPM‐ALK fusion protein (p80) are differentially expressed between peripheral blood and bone marrow in primary small cell variant of anaplastic large cell lymphoma

Abstract

The t(2;5)(p23;q35) translocation results in the formation of a unique chimeric NPM-ALK protein (p80). Expression of this protein is considered to be one of the clinical features of anaplastic large cell lymphoma (ALCL). Recently recognized as one clinical subtype of ALCL, the small cell variant is prone to have a leukemic presentation. Although the small cell variant has been recognized as a subtype of ALCL, the clinical properties of this subtype, especially the immunophenotype of lymphoma cells in peripheral blood, have not yet been fully described. This report shows that neither CD30 nor p80 is detected by immunostaining in the predominant small cell malignant clone and also in large lymphoma cells in peripheral blood, while large cells and occasionally observed small cells in bone marrow were found to be positive for CD30 and p80. Our ®ndings suggest that differential expression of CD30 and p80 between peripheral blood and bone marrow lymphoma cells is a property of the small cell variant of ALCL. Am.

Small cell variant of anaplastic large cell lymphoma: a 10‐year review of the All Wales Lymphoma Panel database

“…Interestingly, two cases showed variable expression of CD30. This feature has been described previously, 4 and in cases with leukaemic presentation the CD30– cells may specifically represent the leukaemic component. In all cases ALK was uniformly positive in most tumour cells, both in the small and large tumour cells with a variable pattern of granular and diffuse cytoplasmic immunopositivity, with or without nuclear expression.…”

Small cell variant of anaplastic large cell lymphoma with leukemic presentation: a diagnostic challenge

“… 9 , 18 So far a total of 26 cases of leukemic ALCL have been identified, mostly presenting with an elevated white blood cell count (average <100 × 10 9 /L). 6 , 8 , 9 , 10 , 12 , 13 , 14 , 17 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 Clinically, most patients presented with B-type symptoms, with fever being reported most (85.18%). The age of affected patients ranged from seven months to 63 years, but with 70% being younger than 30 years of age.…”

Anaplastic large cell lymphoma in leukemic phase: Extraordinarily high white blood cell count

“…In our review of the literature we have identified only 20 cases of ALCL with a leukemic phase (Table 1). [5][6][7][8][9][10][11][12][13][14][15][16][17] Typically, patients present with an elevated WBC count <100 ¥ 10 9 /L.…”