Premium
Leukaemic presentation of small cell variant anaplastic large cell lymphoma: report of four cases
Author(s) -
Bayle Chantal,
Charpentier Agnès,
Duchayne Eliane,
Manel AnneMarie,
Pages MariePierre,
Robert Alain,
Lamant Laurence,
Dastugue Nicole,
Bertrand Yves,
Dijoud Frédérique,
Emile JeanFrançois,
Machover David,
Brugières Laurence,
Delsol Georges
Publication year - 1999
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1999.01238.x
Subject(s) - cd30 , anaplastic large cell lymphoma , bone marrow , pathology , lymphoma , lymph node , clone (java method) , large cell , large cell lymphoma , medicine , biology , cancer , adenocarcinoma , dna , genetics
We report four cases of a rare subtype of CD30‐positive anaplastic large cell lymphoma (ALCL) with a predominant small cell component (small cell variant of ALCL) presenting with a leukaemic feature. Lymph node biopsy showed malignant cells of varying size with a predominant population of small to medium‐sized malignant cells associated with large anaplastic cells strongly positive for CD30 and epithelial membrane antigen (EMA). Both large and small cells were reactive with antibody ALK1, which recognizes the chimaeric NPM‐ALK protein associated with the t(2;5)(p23;q35). All patients presented with hyperleucocytosis with atypical small lymphocytes. Bone marrow involvement was detected on both aspirate and bone marrow trephine where scattered malignant cells were only demonstrated by immunostaining for CD30 and ALK protein. Atypical cells in peripheral blood, lymph node and skin biopsies showed a T or null cell phenotype. Cytogenetic analysis of blood, bone marrow and/or lymph node revealed the t(2;5)(p23;q35) characteristic of ALCL. The patients responded to chemotherapy but showed early relapse without abnormal cells in peripheral blood. This report shows that the small cell variant of ALCL may have a leukaemic presentation with peripheral blood involvement by atypical lymphocytes and provides evidence that, in the small cell variant of ALCL, the small cell component is a part of the malignant clone.