How I treat prolymphocytic leukemia

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Presentation transcript:

How I treat prolymphocytic leukemia by Claire Dearden Blood Volume 120(3):538-551 July 19, 2012 ©2012 by American Society of Hematology

Clinical features of T-PLL. Clinical features of T-PLL. (A) Nodular skin infiltration. (B) Periorbital and conjunctival edema. Claire Dearden Blood 2012;120:538-551 ©2012 by American Society of Hematology

PB morphology from a typical case of T-PLL showing medium-sized lymphoid cells with a regular nuclear outline, single nucleolus, and intense basophilic cytoplasm. PB morphology from a typical case of T-PLL showing medium-sized lymphoid cells with a regular nuclear outline, single nucleolus, and intense basophilic cytoplasm. An occasional cell shows a cytoplasmic protrusion. Claire Dearden Blood 2012;120:538-551 ©2012 by American Society of Hematology

Flow cytometry in T-PLL showing negative membrane expression of CD3, strong expression of CD7 and CD5, and coexpression of CD4 and CD8. Flow cytometry in T-PLL showing negative membrane expression of CD3, strong expression of CD7 and CD5, and coexpression of CD4 and CD8. Courtesy of Ricardo Morilla (Royal Marsden Hospital). Claire Dearden Blood 2012;120:538-551 ©2012 by American Society of Hematology

Genetics in T-PLL. Genetics in T-PLL. (A) Complex karyotype from a case of T-PLL showing the characteristic abnormalities of inversion 14 and trisomy 8 [gain of whole chromosome rather than i(8q)]. The karyotype is 47,XX, +8, del12(p13.1p13.3), inv(14) (q11q32). (B) The deregulation of the oncogenes TCL-1 (chromosome 14) and MTCP-1(X chromosome) through translocations involving the TCR alpha locus on chromosome 14. Courtesy of John Swansbury (Royal Marsden Hospital). Claire Dearden Blood 2012;120:538-551 ©2012 by American Society of Hematology

Treatment algorithm for T-PLL. Claire Dearden Blood 2012;120:538-551 ©2012 by American Society of Hematology

Survival curve for 86 T-PLL patients treated with alemtuzumab. Survival curve for 86 T-PLL patients treated with alemtuzumab. Survivors beyond 72 months are those who received consolidation with a HSCT. Claire Dearden Blood 2012;120:538-551 ©2012 by American Society of Hematology

PB morphology of 4 different B-cell leukemias. PB morphology of 4 different B-cell leukemias. (A) B-PLL, showing monomorphic prolymphocytes (PL) with condensed chromatin, prominent nucleolus, and scanty basophilic cytoplasm. (B) CLL/PL showing a single prolymphocyte (PL), and several typical CLL cells, which are half the size of the PL, have less cytoplasm and no nucleolus. (C) HCL-V showing cells with condensed chromatin and a conspicuous single nucleolus, but with more abundant pale cytoplasm with cytoplasmic projections. (D) SMZL showing lymphocytes with short polar villi and basophilic cytoplasm. Claire Dearden Blood 2012;120:538-551 ©2012 by American Society of Hematology

Histology of the spleen in B-PLL. Histology of the spleen in B-PLL. (A) Low power view (original magnification ×20) showing replacement of the white pulp and infiltration of the red pulp. (B) High power view (original magnification ×100) of the white pulp showing the typical prolymphocyte morphology with abundant cytoplasm, round nuclei, and a central eosinophilic nucleolus. Courtesy of Andrew Wotherspoon (Royal Marsden Hospital)‏ Claire Dearden Blood 2012;120:538-551 ©2012 by American Society of Hematology

Treatment algorithm for B-PLL. Claire Dearden Blood 2012;120:538-551 ©2012 by American Society of Hematology