Hairy cell leukaemia

• What is Hairy Cell Leukaemia
• Statistics on Hairy Cell Leukaemia
• Risk Factors for Hairy Cell Leukaemia
• Progression of Hairy Cell Leukaemia
• Symptoms of Hairy Cell Leukaemia
• Clinical Examination of Hairy Cell Leukaemia
• How is Hairy Cell Leukaemia Diagnosed?
• Prognosis of Hairy Cell Leukaemia
• How is Hairy Cell Leukaemia Treated?
• Hairy Cell Leukaemia References

What is Hairy Cell Leukaemia

Hairy Cell Leukaemia arises from cells located in the bone marrow. The bone marrow is the soft, spongy tissue located in the centre of most long bones and is responsible for the production of the cellular components of blood; namely white cells, red cells and platelets.
Lymphoblasts and myeloblasts are found in the bone marrow and are normal “immature” (precursor) cells of white and red blood cells. The development of these lymphoblasts is influenced by many factors within the body (endogenous factors) such as proteins and cytokines to differentiate into mature white and red blood cells.
Leukaemia occurs when there is abnormally high production (proliferation) of lymphoblasts and myeloblasts in the bone marrow.
It may secondarily affect the blood, lymph nodes, liver, spleen, bones, joints, central nervous system, testes and skin.

Statistics on Hairy Cell Leukaemia

Hairy cell leukaemia is rare and accounts for approximately 2% of all leukaemias. It occurs mostly in males with a ratio of 6:1. Most patients diagnosed are above the age of 45 and the peak incidence occurs between the fifth and sixth decades.

Risk Factors for Hairy Cell Leukaemia

No clear predisposing factors for this disease have been established. There has been the occasional report of recurrence within a family, however, this is rare.

Progression of Hairy Cell Leukaemia

Hairy cell leukaemia leads to gradual infiltration of the bone marrow and spleen by the abnormal cells. This can lead to bone marrow failure and an enlarged spleen.

How is Hairy Cell Leukaemia Diagnosed?

A full blood count usually reveals a reduction in all cellular components of the blood (pancytopaenia). Anaemia may be a feature as well as elevated liver enzymes.

Prognosis of Hairy Cell Leukaemia

There is a 90% response rate with the use of current therapies and the median survival over 4 years.

How is Hairy Cell Leukaemia Treated?

A class of drugs by the name of purine analogues have had promising effects in the treatment of Hairy Cell Leukaemia. These 3 drugs are 2-Deoxycoformycin, 2-Chlorodeoxyadenosine and Interferon-alpha.
A splenectomy (removal of the spleen) may be necessary.

Hairy Cell Leukaemia References

1. Cotran RS, Kumar V, Collins T. Robbins Pathological Basis of Disease Sixth Ed. WB Saunders Company 1999.

 

• Fauci AS et al (1998), Harrison’s Principles of Internal Medicine 14th Edition, McGraw-Hill.
• Haslett C, Chilvers E, Boon N, Colledge N (Editors) (2002), Davidson’s Principles and Practice of Medicine 19th Edition, Churchill Livingstone.
• Kumar P, Clark M. Clinical Medicine. Fourth Ed. WB Saunders, 1998.
• Souhami RL, Tannock I, Hohenberger P, Horiot J-C. Oxford Textbook of Oncology.Oxford University Press. 2002.