What are the NCCN guidelines for hairy cell leukemia (HCL) treatment?

Updated: Sep 16, 2018
  • Author: Emmanuel C Besa, MD; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Answer

NCCN guidelines recommend observation in the absence of the following clinical signs [15] :

  • Systematic symptoms
  • Splenic discomfort
  • Recurrent infection
  • Hgb < 12g/dL
  • Platelets < 100,000/μL
  • Absolute neutrophil count (ANC) < 1000/μL

If clinical indications are present, intial first-line treatments are the purine analogues cladribine or pentostatin. [15]

ESMO concurs with the NCCN recommendations, but includes an additional recommendation of interferon alfa for pregnant patients or those with very severe neutropenia. [16]

NCCN defines CR to treatment as the following [15] :

  • Near normalization of peripheral blood counts
  • Absence of HCL cells on bone marrow biopsy or peripheral blood samples
  • Regression of splenomegaly by physical exam
  • Hgb >11g/dL (without transfusion)
  • Platelets >100,000/μL
  • ANC >1500/μL

For patients who do not acheive CR or who relapse less than 24 months after achieving CR, NCCN recommends the following treatment options [15] :

  • Enrollment in appropriate clinical trial
  • Alternate purine analogue with or without rituximab
  • Rituximab monotherapy, if the patient is unable to receive purine analogues
  • Interferon alfa

For patients who relapse more than 24 months after achieving CR, NCCN recommends the following treatment options [15] :

  • Re-treat with the initial purine analogue with or without rituximab
  • Alternate purine analogue with or without rituximab
  • Rituximab monotherapy, if the patient is unable to receive purine analogues

For progressing disease thta is not responsive to purine analogue therapy, NCCN recommends enrollment in ca linical trial or vemurafenib with or without rituximab. [15]


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