Professional Documents
Culture Documents
Hairy Cell Leukmia
Hairy Cell Leukmia
in nutshell
Ahmed Allam A.H. Mohamed.
Assiatant Lecturer, Clinical Oncology
and Nuclear med. Depart.
Assiut University Hospitals
• Hairy cell leukemia is a chronic Lymphoprolifrative
disorder.
• In 1958, Bouroncle et al. used “leukemic
reticuloendotheliosis” to describe the clinical entity now
recognizable as HCL.*
• Eight years later (1966), Schrek and Donnelly also reported
on the same disease and commented on “peculiar cells” that
had numerous short villi and were arbitrarily called “hairy
cells” on phase contrast microscopy. “Hairy cell leukemia”
gained popular and official recognition.**
Bouroncle BA, Wiseman BK, Doan CA. Leukemic reticuloendotheliosis. Blood 1958.
Schrek R, Donnelly WJ. “Hairy” cells in blood in lymphoreticular neoplastic disease and “ flagellated” cells of normal lymph nodes. Blood 1966.
WHO 2008: mature B-cell neoplasms.
Revised guidelines for the diagnosis and management of hairy cell leukaemia and hairy cell leukaemia variant,BJH, 2011
Flow Cytometry:
• either peripheral blood or aspirated bone marrow, flow
cytometric studies can be very helpful.
• Markers of B cells, such as CD19, CD20 and CD22, are
always positive
• Negative for CD5, CD10, CD23
• Strong postive CD11c, FMC7, CD25, CD103
Revised guidelines for the diagnosis and management of hairy cell leukaemia and hairy cell leukaemia variant,BJH, 2011
Clinical features
• Patients may be asymptomatic and the disease is
identified because a full blood count is taken for
an unrelated reason.
• Symptoms related to Cytopenias:
Up until mid‐1980s
• Splenectomy: overall survival 4‐6 yrs
• IFNα: 80% response, rare CRs
Saven A, Burian C, Koziol JA, et al. Long-term follow-up of patients with hairy cell leukemia after cladribine treatment.
Blood1998
PURINE ANALOGS: Cladribine cont’d
Cladribine has been delivered in a variety of ways
• a normalization of cytopenias
• a minimum 50% improvement in both
organomegaly and bone marrow infiltration
• No circulating hairy cells.
Treatment at relapse
• The majority of relapsed patients achieve second
remission when re-treated with either
pentostatin or cladribine.
• Choice of agent at relapse may depend on the
duration of first remission: if short, i.e. <1 year,
use the alternative agent; if longer (>1 years)
retreat using the same agent
Rituximab
• Rituximab has been an effective salvage therapy for
relapsed and/or refractory HCL
• Study treated 15 relapsed and/or refractory patients
with rituximab 350mg/m2 for eight consecutive
weeks. OR rate was 80%, including eight CRs, two
CRs with minimal residual disease, and two PRs.*
• A retrospective study evaluated eight patients who
had relapsed HCL to prior purine analog therapy. All
eight patients received salvage therapy with
rituximab in combination (either sequential or
concomitant) with a purine analog. The OR rate was
100%, including 87.5% CRs**
Thomas DA, O’Brien S, Bueso-Ramo C, et al. Rituximab in relapsed or refractory hairy cell leukemia. Blood2003
ElseM, OsujiN, Forconi F, et al. The role of rituximab in combination with pentostatin or cladribine for the treatment of recurrent/refractory
hairy cell leukemia. Cancer 2007
interferon alpha
• The role of interferon alpha is nowadays limited
to patients who present with severe
pancytopenia and for whom there is a pressing
need for cell count recovery as quickly as
possible.
• A regimen of 3 mega-units three times a week
will gradually improve blood counts and
facilitate the subsequent use of either nucleoside
analogue.
BL-22 recombinant immunotoxin
• BL-22 is a recombinant immunotoxin composed of an
anti- CD22 variable domain fused to a fragment of
pseudomonas exotoxin.
“The art of medicine consists of amusing the patient while nature cures the disease.”
Voltaire
Paint: hygeia( ancient greek Goddess of good health, cleanliness, and sanitation) for
Gustave Klimt