T-PLL relapse

Author:  Girish Venkataraman, MD, MBBS; Elizabeth Hyjek, MD; Andrew S Artz, MD, 07/14/2018
Category: Lymphoma: Mature T and NK cell lymphoproliferations > Mature T-cell Leukemias > T cell Prolymphocytic Leukemia
Published Date: 07/14/2018

This is a fifty-six year old male patient with relapsed T-cell prolymphocytic leukemia 2.5 years after matched unrelated donor stem cell transplantation.

He was initially diagnosed in 2015 with white cell count of 54,400 per microliter at the time with extensive bone marrow infiltration and diffuse lymphadenopathy as well as splenomegaly. Flow cytometry at that time demonstrated expression of CD45, CD3 and CD4 without CD8.  However when he relapsed with disease after transplant in the skin and bone marrow, there was a change in phenotype with notable double expression of CD4 and CD8 with loss of CD45.

He was on anti-CD52 therapy since the most recent relapse with addition of pentostatin and bendamustine subsequently but with progressively rising blood counts and lymphocytosis. The slides and flow plots since this therapy after relapse are depicted below.

Learning point from case below:
1. T-PLL often shows blebs as noted in the smears below and presents with high counts in the blood.
2. Change in phenotype may occur in T-cell processes, so careful attention must be paid to such changes at relapse.

Blood in T-PLL

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TProlymphocytic-leukemia
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TProlymphocytic-leukemia
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TProlymphocytic-leukemia
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Gating plot in T-PLL

Gating CD45 versus side scatter plot shows a large population of cells (colored aqua) negative for CD45 with low side scatter in comparison with the lymphocytes seen to the far right (colored red and green) expressing moderate CD45.

TProlymphocytic-leukemia
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Pan T-cell antigens in T-PLL

The abnormal T-cell cluster is positive for CD7, CD5 and CD3, all of which are pan T-cell antigens. CD7 additionally stains normal NK-cells which are negative for CD3 and CD5. Note in the second CD3 versus CD7 plot, the abnormal population of T-PLL is positive for CD3 while the background NK cells are CD3 negative the right CD7 expression. The other green cluster in that plot negative for both CD3 and CD7 are B-cells. Both CD5 and CD7 are abnormally bright relative to the other T-cells in pink color.

TProlymphocytic-leukemia
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TProlymphocytic-leukemia
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TProlymphocytic-leukemia
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CD4 and CD8

The T-PLL cells (aqua) are positive for both CD4 and CD8 while the normal CD4 (red) and normal CD8 (pink) are visible as separate clusters. The abnormal cells additionally express T-cell receptor-alpha and dim CD52. CD52 expression assessment is important in B- and T-PLL for anti-CD52 (CAMPATH) therapy.

TProlymphocytic-leukemia
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TProlymphocytic-leukemia
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TProlymphocytic-leukemia
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