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Hairy cell leukemia

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.Classification and risk stratification

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)
Recognize that complete response is associated with a significantly longer disease-free survival than partial response.
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2.Diagnostic investigations

Peripheral blood smear: as per ESMO 2015 guidelines, obtain peripheral blood smear and peripheral blood flow cytometry in patients with suspected classical HCL.

3.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.
Obtain BRAF mutation analysis of exon 15 in difficult cases.

4.Medical management

Indications for treatment, asymptomatic patients, ESMO: do not initiate treatment in asymptomatic patients.
follow-up patients every 3-12 months with complete history, physical examination, CBC, and routine chemistry.

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  • Indications for treatment (symptomatic patients)

  • Purine analogs

  • Interferon-alpha

  • Growth factors

5.Therapeutic procedures

Blood product transfusion: 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.

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  • Stem cell transplantation

6.Surgical interventions

Indications for splenectomy: as per ESMO 2015 guidelines, consider performing splenectomy in patients with resistant massive symptomatic splenomegaly (> 10 cm below the costal margin) with accompanied low-level bone marrow infiltration.
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7.Specific circumstances

Pregnant patients: initiate treatment in pregnant patients with HCL only when truly warranted.
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  • Patients with HCL variant

8.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.

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  • Antiviral prophylaxis

9.Follow-up and surveillance

Assessment of treatment response: as per ESMO 2015 guidelines, perform bone marrow examination for the assessment of response to treatment.

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  • Management of relapsed/refractory disease