Leukemia & Lymphoma, October 2009; 50(S1): 2–7
Historical aspects and milestones in the development of effective
treatment for hairy cell leukemia
MARTIN S. TALLMAN
1
& AARON POLLIACK
2
1
Feinberg School of Medicine, Robert H. Lurie Comprehensive Cancer Center, Northwestern University and
2
Department of
Hematology, Hadassah University Hospital and Hebrew University Medical, School, Jerusalem, Israel
Abstract
Hairy cell leukemia (HCL) is a rare B-cell lymphoproliferative disorder, but is gratifying to treat for both physicians and
patients. During the 50 years since its initial description as a clinical entity, hematologists have been fascinated by the bizarre
appearance of the malignant cell with its hair-like projections. Therapeutic strategies have evolved from splenectomy to
interfere to the purine analogues which have become the treatments of-choice and are very effective. Immunoconjugate
Therapy with BLZZ (anti-CDZZ antibody linked to truncated to Pseudomonas exotoxin) represents the newest milestone in
the development of effective treatment for hairy cell leukemia.
Keywords: Hairy cell leukemia
Introduction assigned the disease a place in the ontogeny of
lymphoproliferative disorders and provided insight
For such a rare disease, hairy cell leukemia (HCL)
into the molecular pathogenesis [6,7]. The unique
receives a lot of attention. In 2008, the American
distribution of the disease, with involvement limited
Society of Hematology celebrated the 50th anniver-
generally to the bone marrow and spleen, appears to
sary since its first meeting. In the same year, the
be attributable to specific integrins such as a4b1 and
hematologic malignancy community marked the
its ligand VCAM-1 expressed by endothelial cells,
50th anniversary of the first comprehensive descrip-
after interaction with tumor necrosis factor alpha
tion of HCL as a distinct clinical entity by Bouroncle
(TNFa) released by the hairy cells [8–10]. The
et al. in 1958 [1]. During the last 50 years, the natural
characteristic fibrosis is due to synthesis of fibronec-
history of the disease was described, the clinical
tin, the extracellular-matrix ligand for a4b1 by hairy
manifestations became apparent, and very effective
cells [11]. The explanation for the unusually indolent
treatment has emerged [2–4]. However, in some
course remains largely unknown. During the last half
ways the disease remains enigmatic.
century, this rarest of all well-described clinical
The development of effective treatment for HCL
entities, with one of the most unusual morphological
has paralleled understanding of the pathogenesis of
features, has become a highly treatable disease.
the disease. Hematopathologists can identify the
disease under the microscope and distinguish it
from other lymphoproliferative disorders [5]. The
Humble beginnings
characteristic morphologic features are usually more
apparent in the bone marrow than the peripheral Ewald [12] is credited with the first description of
blood where cells are frequently present in low HCL and referred to the disease as leukemic
numbers and the classic hair-like projections are reticuloendolethiosis in 1923. This term was also
often difficult to see. Laboratory investigators have used by Gosselin et al. 2 years before the classic
Correspondence: Martin S. Tallman, 676 N. St. Clair Street, Suite 850, Chicago, IL 60611, USA. E-mail: m-tallman@northwestern.edu
ISSN 1042-8194 print/ISSN 1029-2403 online C211 2009 Informa Healthcare USA, Inc.
DOI: 10.3109/10428190903219667
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