Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1999-6-18
pubmed:abstractText
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.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Mar
pubmed:issn
0007-1048
pubmed:author
pubmed:issnType
Print
pubmed:volume
104
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
680-8
pubmed:dateRevised
2009-11-19
pubmed:meshHeading
pubmed-meshheading:10192426-Adolescent, pubmed-meshheading:10192426-Cell Size, pubmed-meshheading:10192426-Child, pubmed-meshheading:10192426-Chromosomes, Human, Pair 2, pubmed-meshheading:10192426-Chromosomes, Human, Pair 5, pubmed-meshheading:10192426-Fatal Outcome, pubmed-meshheading:10192426-Female, pubmed-meshheading:10192426-Flow Cytometry, pubmed-meshheading:10192426-Humans, pubmed-meshheading:10192426-Immunohistochemistry, pubmed-meshheading:10192426-Infant, pubmed-meshheading:10192426-Leukocytosis, pubmed-meshheading:10192426-Lymphocytes, pubmed-meshheading:10192426-Lymphoma, Large-Cell, Anaplastic, pubmed-meshheading:10192426-Male, pubmed-meshheading:10192426-Protein-Tyrosine Kinases, pubmed-meshheading:10192426-RNA, Messenger, pubmed-meshheading:10192426-Reverse Transcriptase Polymerase Chain Reaction, pubmed-meshheading:10192426-Translocation, Genetic
pubmed:year
1999
pubmed:articleTitle
Leukaemic presentation of small cell variant anaplastic large cell lymphoma: report of four cases.
pubmed:affiliation
Service d'Hématologie Biologique, Institut Gustave Roussy, Villejuif, France.
pubmed:publicationType
Journal Article, Case Reports, Research Support, Non-U.S. Gov't