How I treat LGL leukemia

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Presentation transcript:

How I treat LGL leukemia by Thierry Lamy, and Thomas P. Loughran Blood Volume 117(10):2764-2774 March 10, 2011 ©2011 by American Society of Hematology

How to establish the diagnosis of LGL leukemia. How to establish the diagnosis of LGL leukemia. The diagnosis of LGL leukemia is made on the following criteria: common criteria of LGL leukemia. The diagnosis is based on a LGL peripheral expansion (> 0.5 × 109/L). Specific criteria for T-LGL leukemia include: expression of LGL surface markers compatible with an activated T-cell (commonly CD3+/CD8+/CD57+ and/or CD16+) phenotype; and clonal rearrangement of TCR-γ gene using PCR or specific and clonal Vβ expression using FCM. Specific criteria for NK-LGL leukemia and NK-LGL lymphocytosis include: expression of LGL surface markers compatible with an NK cell (commonly CD3−/CD8+/CD16+ and/or CD16+/CD56+) phenotype. The term chronic NK-LGL lymphocytosis is used for patients with relatively few symptoms and chronic illness, whereas patients with massive tissue LGL infiltration of the spleen, liver, and bone marrow and presenting aggressive clinical behavior are considered as having NK-LGL leukemia. Thierry Lamy, and Thomas P. Loughran Jr Blood 2011;117:2764-2774 ©2011 by American Society of Hematology

Small and large granular lymphocytes. Small and large granular lymphocytes. Distinction between small lymphocytes (A) and large granular lymphocytes (B) on blood smears. Micrographs were viewed with a Leica Leitz DMRB microscope using a 100×/1.30 oil immersion objective. Images were captured with a Sony Exwave HAD camera and manipulated using Tribyn Version 1.3 software. Thierry Lamy, and Thomas P. Loughran Jr Blood 2011;117:2764-2774 ©2011 by American Society of Hematology

Bone marrow features of T-LGL leukemia. Bone marrow features of T-LGL leukemia. Hematoxylin and eosin staining of marrow biopsy reveals a slightly hypercellular marrow with subtle increase in interstitial lymphocytes, as shown by arrow (left). Immunohistochemistry analyses (right) highlight the presence of intrasinusoidal cytotoxic T-cell infiltrates, staining positive for CD8 (arrow) and granzyme B (inset). Micrographs were viewed with a Leica Leitz DMRB microscope using a 100×/1.30 oil immersion objective. Images were captured with a Sony Exwave HAD camera and manipulated using Tribyn Version 1.3 software. Thierry Lamy, and Thomas P. Loughran Jr Blood 2011;117:2764-2774 ©2011 by American Society of Hematology

Flow cytometry analysis in LGL leukemia. Flow cytometry analysis in LGL leukemia. (A) Blue: Normal nonclonal CD3+ T cells showing a polyclonal Vβ repertoire pattern. Red: LGL population showing a CD3+/CD8+/CD5dim phenotype with a unique clonal Vβ7.1 expression (> 92%). (B) The NK CD3− LGL population strongly expresses the CD94/KIR ag. Thierry Lamy, and Thomas P. Loughran Jr Blood 2011;117:2764-2774 ©2011 by American Society of Hematology

Treatment algorithm. Treatment algorithm Thierry Lamy, and Thomas P. Loughran Jr Blood 2011;117:2764-2774 ©2011 by American Society of Hematology