B-cell prolymphocytic leukemia
ICD-10 C91.3 · ICD-11 2A82.1

Treatment of B-cell Prolymphocytic Leukemia with TP53 Deletion or Mutation

Clinical Scenario

This protocol addresses patients with B-cell prolymphocytic leukemia (B-PLL) who carry a TP53 deletion and/or mutation. The presence of TP53 abnormality defines a distinct high-risk subgroup that requires a specific first-line management strategy.

Key Finding
TP53 deletion / mutation

In B-PLL, patients with deletions or mutations of TP53 have nonfunctional TP53, which has historically driven a distinct treatment pathway compared with patients who have normal TP53. Identifying this molecular finding is central to selecting the appropriate treatment approach.

Treatment Approach (overview)

For eligible patients with B-PLL and TP53 abnormality, first-line management may involve a potentially curative transplant-based approach — with the aim of achieving and consolidating remission early in the disease course.

The full structured regimen, eligibility criteria, and sequencing details are available in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

  1. For T-PLL, first-line therapy is with intravenous alemtuzumab and for B-PLL is with combination chemo-immunotherapy for patients with normal TP53 and with alemtuzumab or BCR inhibitors for those with deletions or mutations of TP53.
  2. For those patients with nonfunctional TP53, alemtuzumab has historically been the mainstay of treatment and, although no longer licensed in CLL, is available via a patient access scheme.
  3. For this reason, suitable patients may be considered for early allogenic SCT, as in T-PLL.
  4. For these reasons, it is appropriate to consider potentially curative treatment with allogenic SCT in first remission for eligible patients.
DOI: 10.1182/asheducation-2015.1.361
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