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Hairy Cell Leukemia: Causes, Symptoms & Treatment

Hairy cell leukemia is a rare, slow-growing blood cancer in which abnormal B-lymphocytes with hair-like projections accumulate in the bone marrow and spleen.

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Things worth knowing about "Hairy Cell Leukemia"

Hairy cell leukemia is a rare, slow-growing blood cancer in which abnormal B-lymphocytes with hair-like projections accumulate in the bone marrow and spleen.

What is Hairy Cell Leukemia?

Hairy cell leukemia (HCL) is a rare, chronic form of B-cell leukemia, a cancer of the blood-forming system. It accounts for approximately 2% of all leukemias and primarily affects middle-aged to older men. The name refers to the fine, hair-like cytoplasmic projections visible on the surface of the malignant cells under a microscope.

The disease typically progresses slowly and, thanks to modern therapies, is manageable for the vast majority of patients.

Causes

The exact cause of hairy cell leukemia is not yet fully understood. It arises from an acquired, non-hereditary genetic mutation in a single B-lymphocyte. The central molecular driver is the BRAF V600E mutation, a point mutation in the BRAF gene found in nearly all classic HCL cases. This mutation leads to uncontrolled cell proliferation.

No established environmental risk factors
No direct familial inheritance pattern
A possible association with certain chemical exposures or radiation is being discussed

Symptoms

Many patients have few or no symptoms at diagnosis. Common symptoms arise from the displacement of normal blood cell production in the bone marrow and enlargement of the spleen (splenomegaly):

Persistent fatigue and exhaustion
Frequent infections due to low levels of healthy white blood cells (granulocytopenia)
Increased bleeding tendency due to low platelet counts (thrombocytopenia)
Pallor and shortness of breath due to anemia
Pressure or pain in the upper left abdomen from an enlarged spleen
Weight loss and night sweats (less common)

Diagnosis

Diagnosis is established through a combination of blood tests, bone marrow biopsy, and imaging:

Blood count: Detection of pancytopenia (reduction in all blood cell lines) and characteristic hairy cells on a blood smear
Flow cytometry: Immunophenotyping to identify typical surface markers (CD11c, CD22, CD25, CD103, CD123)
Bone marrow biopsy: Histological confirmation of hairy cell infiltration
Molecular genetics: Detection of the BRAF V600E mutation to confirm the diagnosis
Imaging (ultrasound, CT scan): Assessment of spleen and lymph node enlargement

Treatment

Not all patients require immediate treatment. In asymptomatic cases, an active surveillance approach (watch and wait) is initially appropriate.

First-Line Therapy

The standard treatment for hairy cell leukemia involves purine analogues, particularly:

Cladribine (2-CdA): A single treatment cycle achieves complete remission in approximately 80 to 90% of patients.
Pentostatin (DCF): Equally effective and used as an alternative.

Further Treatment Options

Rituximab: A monoclonal antibody targeting CD20, used in relapsed disease or in combination with purine analogues.
BRAF inhibitors (e.g., vemurafenib): Targeted therapy for patients with relapsed or refractory disease.
Splenectomy: Surgical removal of the spleen, now rarely performed and reserved for specific clinical situations.

Prognosis

The overall prognosis for hairy cell leukemia is excellent. Most patients achieve long-term remission following treatment. The 10-year survival rate exceeds 90%. Relapses can occur but generally respond well to retreatment.

References

Grever M. R. et al. - Consensus guidelines for the diagnosis and management of hairy cell leukemia. Blood, 129(5):553-560, 2017. DOI: 10.1182/blood-2016-01-689422
Tiacci E. et al. - BRAF mutations in hairy-cell leukemia. New England Journal of Medicine, 364(24):2305-2315, 2011. DOI: 10.1056/NEJMoa1014209
National Cancer Institute (NCI) - Hairy Cell Leukemia Treatment (PDQ). www.cancer.gov, accessed 2024.

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