Lymphomas Overview
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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
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Incidence
HCL is an uncommon cancer of the blood.
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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
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Common signs
and symptoms
- Easy bruising and bleed (low platelets)
- Fatigue
- Enlarged lymph nodes - painless swelling in the
neck, armpit or groin - often in more than one group
- Recurrent infection and fever
Other symptoms may include night sweats, unexplained
high temperatures and weight loss. These are known as B
symptoms.
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Tests
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CBC to test White
Blood Cell (WBC) count, low red cell count, and low platelets
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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
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References
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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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Clinical Trials
Lymphoma-specific clinical trials by:
Other
criteria ... includes filters for age, frontline,
grade, phase, stage, and refractory disease
By
State & Country
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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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