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Types of Lymphoma > Hairy Cell Leukemia

Last update: 09/26/2012

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Background

Hairy cell leukemia (HCL) is a cancer of lymphocytes (B cells) that leads to low blood counts. 2 HCL affects both the blood and the bone marrow. 

"This rare type of leukemia gets worse slowly or not at all. The disease is called hairy cell leukemia because the leukemia cells look "hairy" when viewed under a microscope." 3

"Early in the course of the disease, no treatment may be necessary. Some patients may need an occasional blood transfusion. ... Newer treatments using chemotherapy have greatly improved the survival of patients with hairy cell leukemia.2

See WHO Classification

Incidence

HCL is an uncommon cancer of the blood.


Diagnosis   

To make an accurate diagnosis of lymphoma, a biopsy must be performed by the surgical removal (resection) of a lymph node.  A fine needle aspiration may be performed if a lymph node is not accessible, but this is not considered a definitive way to determine the diagnosis.

A series of tests will then be performed to determine the characteristics of the cells.  If a malignancy is determine, these characteristics will allow your doctors to determine the appropriate treatments to use when needed. 

"The best approach to establishing the diagnosis of hairy cell leukemia is to carefully examine blood and bone marrow biopsy specimens to identify cells with the morphologic features of hairy cells and to demonstrate that the neoplastic cells have an antigenic profile that is characteristic for hairy cell leukemia.  ...

Monoclonal antibody and gene rearrangement studies have indicated that the hairy cell is a postgerminal B cell. ...  relatively mature B cell, perhaps at a preplasma cell stage of development." 1  

Specific markers:  "hairy cells express the pan-B-cell antigens CD19, CD20, CD22, and CD79a, although they usually lack CD21"  1

Common signs and symptoms

bullet Easy bruising and bleed (low platelets)
bullet Fatigue
bullet Enlarged lymph nodes - painless swelling in the neck, armpit or groin - often in more than one group
bullet Recurrent infection and fever

Other symptoms may include night sweats, unexplained high temperatures and weight loss. These are known as B symptoms.

Tests

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CBC to test White Blood Cell (WBC) count, low red cell count, and low platelets 

bullet

CT scan shows an enlarged spleen and liver 

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Bone marrow biopsy 

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Blood tests may show HCL cells

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Palpation (physical examination) to check for enlarged spleen or liver 

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Tartrate-resistant acid phosphatase can confirm the diagnosis 

References

  1. About  ncbi.nlm.nih.gov/books
  2. About  MedlinePlus
  3. Patient PDQ  www.cancer.gov 
  4. Professional PDQ  www.cancer.gov 
 

Treatment

TOPIC SEARCH PubMed 

"Hairy cell leukemia is a chronic lymphoproliferative disorder that is easily controlled. 

The decision to treat is based on symptomatic cytopenias (low blood counts), massive splenomegaly (enlarged spleen), or the presence of other complications. 

About 10% of all patients will never require therapy."  www.cancer.gov

"If treatment, a variety of chemotherapy drugs (cladribine, pentostatin) can be used. Interferon is also used. In the majority of cases these drugs can produce a remission (complete relief from the disease) that lasts for many years. 

It is unclear if chemotherapy will cure the disease, however, because most patients will relapse over time. The removal of the spleen may improve blood counts, but is unlikely to cure the disease. 

Symptomatic treatment of infections with antibiotics or low blood counts with growth factors and transfusions may be required." MedlinePlus 

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Treatment statement for health professionals meb.uni-bonn.de 

"Untreated hairy cell leukemia is characterized by splenomegaly, varying degrees of leukopenia (occasionally leukocytosis) and/or pancytopenia, and bone marrow infiltration by an atypical cell with prominent cytoplasmic projections (i.e., hairy cells). The bone marrow is usually fibrotic and is not easily aspirated. Bone marrow biopsies are, therefore, required for diagnosis and evaluation of the degree of hairy cell infiltration.

Progressive hairy cell leukemia

Progressive hairy cell leukemia, postsplenectomy (or following any systemic therapy) is characterized by progressive bone marrow replacement by hairy cells with pancytopenia refractory to treatment. For patients with advanced hairy cell leukemia treated with cladribine (2-chlorodeoxyadenosine, 2-CdA), pentostatin, or interferon alfa, the survival rate appears to be >85% at 5 years after the initiation of any one of these therapies. [1] [2

Rituximab (Rituxan) can induce durable complete remissions with minimal toxic effects in the majority of patients with relapsing or refractory disease after purine analog therapy. [5] [6] [7]

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References and related articles

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Efficacy of anti-CD20 monoclonal antibodies (Mabthera) in patients with progressed hairy cell leukemia.
Haematologica. 2001 Oct;86(10):1046-50. PMID: 11602410
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Hairy cell leukemia: survival and relapse. Long-term follow-up of purine analog-based therapy and approach for relapsed disease.
Transfus Apher Sci. 2005 Feb;32(1):99-103. Review. PMID: 15737878
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Role of interferon-alpha administration after 2-deoxycoformycin in the treatment of hairy cell leukemia patients.
Eur J Haematol. 2006 Aug;77(2):109-13. PMID: 16856905
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Results of treatment in patients with hairy cell leukemia with splenectomy, alpha-interferon and deoxycoformycin Srp Arh Celok Lek. 2000 Jul-Aug;128(7-8):262-70. Serbian. PMID: 11089434 
 
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