Table of Contents



1.1 verplicht aanvullend onderzoek

  • CT
  • IFT perifeer bloed of beenmerg:high levels of CD52 and also express pan-T cell markers (CD2, CD3, and CD7) and TCL1
  • HTLV negatief

1.2 beleid

  • asymptomatic: wait-and-see
  • CD52-positief: alemtuzumab-based chemotherapy rather than with other chemotherapy combinations
  • complete remission:
    • SCT
      • allogeneic HCT for younger adults with a good performance status
      • autologous HCT if not candidate for allo
    • single agent alemtuzumab: addition of pentostatin

1.3 prognose

  • usually less than two years despite therapy
  • a more indolent variant may exist in a small subset of patients
  • prognostic markers have been difficult to determine


2.1 verplicht onderzoek

2.2 behandeling

  • rare asymptomatic patient without complications: wait-and-see
  • not cured with conventional treatment
  • younger patients may be candidates for allo
  • most commonly treated with regimens used for CLL, not directly compared
  • most frequently partial responses, and rarely durable
  • without deletion 17p or TP53 mutations:
    • FCR or BR
    • >70 years or comorbidities ibrutinib
    • evt. single agent rituximab
  • 17p or TP53 mutations: ibrutinib, alemtuzumab compassionate use
  • R/R: ibrutinib, idelalisib, venetoclax, rituximab, obinutuzumab, splenectomy, splenic irradiation
  • chlorambucil alone is not very effective in B-PLL
  • CHOP partial responses in up to one-third of cases
  • incorporation of rituximab appears to increase response rates and deepen the level of response

3 prognose

  • heterogeneous
  • prognostic markers difficult: anemia, thrombocytopenia, advanced age, MYC abnormalities, TP53 mutations

4 criteria

  • >55% prolymfocyten in bloed/beenmerg en MCL uitgesloten (cycline D1, of SOX11)
  • B-PLL de novo
    • bij vnl. oudere Kaukasiers
    • vaak splenomegalie en geen of beperkte lymfadenopathie, vaak B-symptomen
    • vaak anamie, trombopenie, hoog leukocytengetal
    • denk aan SMZL: soms lastig onderscheid, B-PLL aggressiever, villi
  • prolymfocytaire transformatie uit CLL
    • zeldzaam
    • PA/IFT zoals bij CLL, soms sterke SmIg
    • geen monomorf beeld maar mengsel CLL/PLL-cellen
    • histologie beenmerg als bij CLL

5 immunofenotypering

  • bright surface IgM +/- IgD, en kappa or lambda light chain, en niet DIM
  • bright CD20 en niet dim
  • CD19, CD22, CD79a, and FMC7
  • CD5 and CD23 bij een derde
  • absence of expression of CD11c, CD103, CD10, CD25, and cyclin D1 1.

6 genetica

  • genetic changes common but non-specific
  • most cases complex karyotypes (≥3 abnormalities)
  • abnormalities of MYC (rearrangements and increased copy number) are seen in approximately 60 percent of cases and are associated with a more aggressive clinical behavior
  • deletions of 17p (the chromosomal arm that carries the TP53 gene) and TP53 mutations are found in approximately 40 percent
  • trisomy 18 and del13q (approximately 30 percent each), and trisomy 3 and deletion 8p (approximately 25 percent each)
  • mutations have also been found in TP53, MYD88, BCOR, MYC, SF3B1, SETD2, CHD2, CXCR4, and BCLAF1
  • voorheen frequent t(11;14)(q13;q32), betreft echter MCL




Author: "Koen de Heer"

Created: 2020-02-24 ma 11:25

Emacs 25.2.2 (Org mode 8.2.10)