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Aggressive natural killer cell leukaemia/lymphoma in two patients with lethal midline granuloma
Author(s) -
Soler Jesús,
Bordes Ramon,
Ortouno Francisco,
Montagud Mario,
Martorell Jaume,
Pons Cristina,
Nomdedeu Josep,
LopezLopez Juan Jose,
Prat Jaime,
Rutllant Miquel
Publication year - 1994
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 - 10.1111/j.1365-2141.1994.tb04804.x
Subject(s) - immunophenotyping , lymphoma , cd20 , cd8 , natural killer cell , immunology , cd38 , antibody , cd43 , cd3 , cytotoxic t cell , medicine , cd19 , monoclonal antibody , pathology , biology , antigen , stem cell , cd34 , in vitro , biochemistry , genetics
Summary. We report two patients with leukaemic proliferations of large granular lymphocytes. The immunophenotype study showed that the leukaemic cells were positive for CD2, CD38, CD56 and anti‐HLA‐DR monoclonal antibodies and negative for other T‐cell (CD3, CD4, CD8) and B‐cell markers (CD19, CD20 and surface immunoglobulins). The clinical course was acute and a diagnosis of aggressive natural killer cell leukaemia/lymphoma was made. No clonal rearrangements of either Cβ T‐cell receptor or J H immunoglobulin genes were found. Functional studies done in one patient demonstrated non‐restricted cytotoxic activity after activation with IL‐2. Lethal midline granuloma had been previously diagnosed in both patients. A possible relationship between this entity and the natural killer cell leukaemia is discussed.