Anaplastic Large Cell Lymphoma in Leukemic Phase: High White Blood Cell Count and Poor Prognosis

Strategic Insights -

Researchers have reported a rare case of anaplastic large cell lymphoma (ALCL) in leukemic phase, characterized by an extraordinarily high white blood cell count and a poor prognosis. The patient, who had ALK+ ALCL and the t(2;5)(p23;q35) translocation, presented with a leukemic phase and a peak white blood cell count of 587 x 10(9)/L. This case study highlights the importance of early diagnosis and treatment in patients with ALCL, as the leukemic phase of the disease is associated with a poorer prognosis than most other patients with ALK+ ALCL.

Key Takeaways:

  • Anaplastic large cell lymphoma (ALCL) is a distinct type of T/null-cell non-Hodgkin lymphoma that can involve nodal and extranodal sites.
  • The World Health Organization recognizes two types of ALCL: ALK positive and ALK negative, with the latter caused by abnormalities involving the ALK gene at chromosome 2p23.
  • The leukemic phase of ALCL occurs rarely, but is associated with a poor prognosis, particularly in ALK+ ALCL patients.
  • The t(2;5)(p23;q35) translocation is commonly found in ALK+ ALCL patients with the small cell variant.
  • 20 well-documented cases of ALCL in leukemic phase have been reported in the literature, with white blood cell counts ranging from 15 to 151 x 10(9)/L.
  • Patients with leukemic phase ALK+ ALCL appear to have a poorer prognosis than most patients with ALK+ ALCL.
  • The study highlights the importance of early diagnosis and treatment in patients with ALCL.

Statistics:

  • The patient in the report had an initial white blood cell count of 529 x 10(9)/L, which subsequently peaked at 587 x 10(9)/L.
  • 20 well-documented cases of ALCL in leukemic phase have been reported in the literature.
  • White blood cell counts in these cases ranged from 15 to 151 x 10(9)/L.
  • The study found that ALK+ ALCL patients with the small cell variant and t(2;5)(p23;q35) translocation had a poor prognosis.
  • The patient in the report died 2.5 months after diagnosis despite chemotherapy.

Sources:

  • Nguyen, J.T., et al. "Anaplastic large cell lymphoma in leukemic phase: extraordinarily high white blood cell count." Pathology International, vol. 59, no. 5, 2009, pp. 345-353.
  • University of Texas, Department of Pathology and Laboratory Medicine, Houston, Texas, USA.
  • Blackwell Publishing Inc., 350 Main St., Malden, MA 02148, USA.