HAIRY CELL LEUKEMIA

Dear friends,

Wishing you a very happy and prosperous New Year 2021.

Hairy cell leukemia (HCL) is a chronic B- cell lymphoid malignancy that has always fascinated hematologists and pathologists. In this post, we shall see about the history of hairy cell leukemia with landmark papers.

Year	Description	Landmark papers
1952	Reticular cell leukemia: A clinical hematologic entity- described 17 patients with typical features of HCL presented at a meeting at American Society for Clinical Pathology.	Rosenthal
1958	Classical description of the disease as Leukaemic Reticulo-endotheliosis and the benefits of splenectomy.	Bouroncle et al
1966	Introduction of the term ‘Hairy cell’	Schrek and Donelly
1966	Demonstration of the characteristic bone marrow fibrosis in HCL	Duhamel and Guerra
1971	Showed that hairy cells stained with Tartrate-resistant acid phosphatase (TRAP) Isoenzyme 5	Yam et al
1972	Electron microscopy description of ribosome-lamella complex (RLC)	Katayama, Li and Yam
1974	Pattern of bone marrow histology described	Burke et al
1974	B- cell nature of NHLs confirmed	Catovsky et al
1978	Prognosis- Median OS four years	Golomb, Catovsky
1979	Splenectomy in 26 patients: 86% OS at five years in responders	Mintz and Golomb
1982	Clinical staging system: prognostic groups based on anemia and splenomegaly	Jansen and Hermans
1983	The genetic B-cell origin of HCL	Korsmeyer et al
1984	The membrane phenotype of HCL	Melo et al
1984	Results of treatment of HCL with interferon –alpha	Quesada et al
1987	UK experience with alpha interferon	Worman, Catovsky , Cawley et al.
1987	Results of treatment of HCL with pentostatin	Spiers et al.
1990	Results of treatment with 2CDA (2-chloro deoxy adenosine) (Cladribine)	Piro et al
1990	CD 103 expression in HCL	Mulligan et al
1995	Randomized trial of pentostatin vs interferon in HCL	Grever et al
2004	Annexin A1 expression in HCL	Falini et al
2009	Long-term follow up of 233 HCL patients treated with pentostatin or CDA	Else et al
2011	Demonstrated BRAF V 600 mutation in 100 % cases	Tiacci et al
2015	Clinical trials demonstrating effectiveness of BRAF inhibitors	Tiacci et al
2015	Clonal mutations of CDKN1B (p27), a cell cycle inhibitor, in 16 % of cases	Dietrich et al
2016	CDA + R (Rituximab)  vs CDA alone, showing benefit of addition of rituximab	Chihara D et al
2018	Pivotal trial of moxetumomab pasudotox in HCL	Kreitman et al

 

Electron microscopic picture of Hairy cells within splenic cord. Picture taken from Hairy cell leukemia (Leukemic reticuloendotheliosis). II. Ultrastructure of the spleen. Jerome S. Burkem, Brucem Ackaym. Cancer,1976.

There has been remarkable progress made over years in the diagnosis and management of HCL with the median overall survival of just around four years, in 1978 to a median relapse free survival of 16 years now. We shall see in detail in subsequent posts about the treatment of hairy cell leukemia.

Wishing you all again a very happy, prosperous and successful new year 2021.

References:

Hairy cell leukemia: From Hairy Beginnings to a BRAF New World. British Society for hematology. 60^th anniversary special issue.  November 2020.

 

                                                                                   Written by Dr.Priyavadhana B