Table of contents
Expand All Topics
Hairy cell leukemia
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of hairy cell leukemia are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2015) and the British Committee for Standards In Haematology (BCSH 2012).
1
2
Classification and risk stratification
Prognosis
As per ESMO 2015 guidelines:
Recognize that the following biological factors are associated with poor outcomes:
presence of TP53 mutations
lack of somatic mutations in the IGVH genes (occur in a minor proportion of patients)
VH4-34 family usage (more frequently seen in HCL variant)
B
Recognize that complete response is associated with a significantly longer disease-free survival than partial response.
B
Diagnostic investigations
Diagnostic procedures
Bone marrow biopsy
As per ESMO 2015 guidelines:
Obtain flow cytometry in bone marrow aspirate and perform bone marrow trephine biopsy with immunohistochemistry in patients with suspected classical HCL. Perform bone marrow examination for the diagnosis, particularly after treatment, to assess response.
B
Obtain BRAF mutation analysis of exon 15 in difficult cases.
B
Medical management
Indications for treatment, asymptomatic patients: as per ESMO 2015 guidelines, do not initiate treatment in asymptomatic patients.
D
follow-up patients every 3-12 months with complete history, physical examination, CBC, and routine chemistry. D
More topics in this section
Indications for treatment (symptomatic patients)
Purine analogs
Interferon-alpha
Growth factors
Therapeutic procedures
Blood product transfusion: as per BCSH 2012 guidelines, administer only irradiated blood products in patients treated with cladribine or pentostatin and requiring transfusion, in order to minimize the risk of transfusion-associated GvHD.
B
More topics in this section
Stem cell transplantation
Surgical interventions
Specific circumstances
Pregnant patients: as per ESMO 2015 guidelines, initiate treatment in pregnant patients with HCL only when truly warranted.
B
Show 3 more
More topics in this section
Patients with HCL variant
Preventative measures
Antibiotic prophylaxis: as per ESMO 2015 guidelines, administer prophylactic co-trimoxazole (960 mg 3 times weekly) until the lymphocyte count increases to > 1×10⁹/L for the prevention of Pneumocystis infections in patients with lymphopenia treated with nucleoside analogs.
B
More topics in this section
Antiviral prophylaxis