UI - 11892443
AU - Medini Manai Z; Meddeb B; Lakhal R; Ben Abid H; Bel HadjAli Z; Ben
Othman T; Hafsia A
[Hairy cell leukemia: report of 8 cases]
SO - Tunis Med 2001 Dec;79(12):681-5
AD - Service d'hematologie Hopital Aziza Othmana, Tunis.
Hairy cell leukemia haemopathy is a rare lymphoid haemopathy type B. 8
cases are reported and diagnosed at Hopital Aziza Othmana over a period
of 20 years between 1979 and 1999, 7 men and one women. The mean age of
the patients is 51 years, with externe ages from 42 to 81 years. 4
patients consulted for an infections and, or anaemia syndrome. The
disease was revealed due to the presence of an isolated splenomegaly in
other cases. At the clinical examination, the spleen is hypertrophied in
7 patients out of 8. Pancytopenia is observed in 50% of the patients.
Only one patient has presented a moderated hyperleukocytosis at
11,000/mm3 related to the presence of moving on tricholeukocytes. The
myelogramme is pocr. It allowed to mention the diagnosis in 6 cases out
of 8. Bone Marrow biopsy revealed a diffuse infiltration by TCL with a
reticulinic fibrosis in all patients. 4 patients out of 8 have been
splenectomized. Cytopenies have been corrected in all patients. Only one
patient has been treated by alpha Interferon for 3 years with a partial
hematological response. A relapse was observed once the Interferon was
stopped. With the introduction of new drugs such purine analogues. The
HCL treatment has been revolutionarized thanks to the improvement of the
rate of complete response (from 10% to 80% of CR). If splenectomy is
still observed in HCL for splenomegalic and or severe cytopenia, our
findings could be improved thanks to new purine analogues.
UI - 11801472
AU - Palomera L; Domingo JM; Sola C; Azaceta G; Calvo MT; Gutierrez M
Cladribine (2-chlorodeoxyadenosine) therapy in hairy cell leukemia
variant. A report of three cases.
SO - Haematologica 2002 Jan;87(1):107-8
AD - Department of Hematology, University Clinical Hospital, C/ San Juan
Bosco, 15 50009 Zaragoza, Spain. firstname.lastname@example.org
UI - 11849216
AU - Bourguin-Plonquet A; Rouard H; Roudot-Thoraval F; Bellanger C; Marquet
J; Delfau-Larue MH; Divine M; Farcet JP
Severe decrease in peripheral blood dendritic cells in hairy cell
SO - Br J Haematol 2002 Mar;116(3):595-7
AD - Service d'Immunologie Biologique, Hopital Henri Mondor, Creteil, France.
Clinical studies in hairy cell leukaemia (HCL) have linked the frequent
occurrence of infections due to intracellular pathogens and a profound
monocytopenia. More recently, dendritic cells (DC), a subset of which
are related to monocytes, were shown to be the professional
antigen-presenting cells which stimulate the adaptive immune response.
Using membrane markers and flow cytometry, we determined in peripheral
blood whether various DC subsets and monocytes were impaired in HCL.
Lymphoid and myeloid DC were virtually absent in five HCL patients with
active disease. After treatment, both DC and monocytes recovered slowly.
The decrease in DC suggests that defective antigen presentation could
affect susceptibility to intracellular pathogens in HCL.
UI - 10792402
AU - Grey MR; Flanagan NG; Kelsey PR
Severe skin rash in two consecutive patients treated with
2-chlorodeoxyadenosine for hairy cell leukaemia at a single institution.
SO - Clin Lab Haematol 2000 Apr;22(2):111-3
AD - Haematology/Oncology Unit, Blackpool Victoria Hospital NHS Trust,
Although hairy cell leukaemia was first described 40 years ago, it is
only in the last decade that newer therapeutic agents have enabled
effective treatment. The purine nucleoside analogue,
2-chlorodeoxyadenosine (2-CdA) is currently considered as first-line
therapy with a very high rate of complete remission. Although adverse
events with 2-CdA are increasingly recognized, severe cutaneous
reactions have been reported rarely. We describe two consecutive
patients treated with 2-CdA for hairy cell leukaemia who both suffered
extremely severe cutaneous reactions, one of which was life-threatening.
UI - 10552943
AU - Kreitman RJ; Wilson WH; Robbins D; Margulies I; Stetler-Stevenson M;
Waldmann TA; Pastan I
Responses in refractory hairy cell leukemia to a recombinant
SO - Blood 1999 Nov 15;94(10):3340-8
AD - Laboratory of Molecular Biology, the Metabolism Branch, National Cancer
Institute, National Institutes of Health, Bethesda, MD.
We report major responses in 4 of 4 patients with hairy cell leukemia
(HCL) who have recently been treated on a phase I trial with the
recombinant immunotoxin LMB-2. The immunotoxin, designed to target
CD25(+) malignancies, is composed of the Fv portion of the anti-Tac
(anti-CD25) antibody, fused to a 38-kD truncated form of Pseudomonas
exotoxin A, and has previously been called anti-Tac(Fv)-PE38. All 4 HCL
patients were resistant to standard and salvage therapies for HCL,
including 2-chlorodeoxyadenosine (CdA) and interferon alpha, and all
patients responded to LMB-2 after a single cycle. One patient treated
with 2 cycles had a complete remission (CR), with regression of HCL
cells from the blood and marrow and resolution of splenomegaly and
pancytopenia. As is typical for patients in CR after treatment with CdA,
minimal residual disease was detectable by flow cytometry of the bone
marrow aspirate. This patient has not relapsed after 11 months. Three
other patients had 98% to 99.8% reductions in malignant circulating
cells. These results represent a proof of principal that targeted
therapy with recombinant Fv-containing proteins can be clinically
useful. LMB-2 may be an effective new therapy for patients with
chemotherapy-resistant CD25(+) HCL.
UI - 11755467
AU - Khalaf W; Maina C; Byers J; Harvey W
Interferon-alpha 2b and vesnarinone influence levels of tumor necrosis
factor-alpha, apoptosis, or interleukin 6 in ESKOL, a hairy cell
leukemic cell line. A potential cytokine and oncogene relationship
regulating apoptosis is suggested.
SO - Leuk Res 2002 Feb;26(2):169-77
AD - Department of Microbiology and Immunology, Indiana University School of
Medicine, Indianapolis, IN 46222, USA.
The in vitro effects of interferon-alpha (IFN) on levels of secreted
interleukin 6 (IL-6), tumor necrosis factor-alpha (TNF alpha), and
nuclear matrix proteins (NMP) were examined in ESKOL, a B-lymphoblastoid
cell line resembling hairy cell leukemia (HCL). IFN enhances
differentiation in ESKOL, decreases TNF alpha levels, decreases
apoptosis, increases IL-6 levels, and down regulates the expression of
several oncogenes. Vesnarinone (Ves), a TNF alpha repressor, lowers
TNF-alpha and decreases apoptosis in the same cell line. ESKOL exhibits
enhanced apoptosis and reduced B-cell lymphomas (Bcl-2) levels over
WIL-2. IL-6 and TNFalpha have been shown to decrease and increase
apoptosis in B-cells respectively; however, treatment of ESKOL with
these cytokines had no significant effect on apoptosis. We suggest that
IFN decreases apoptosis by mechanisms involving enhanced IL-6 and Bcl-2
levels, decreased TNF alpha and the down regulation of apoptotic
oncogenes, including c-myc.
UI - 11839386
AU - Tallman MS
Understanding the action of interferon in hairy cell leukemia: the past
SO - Leuk Res 2002 Apr;26(4):407-8
AD - Northwestern University Medical School, Robert H. Lurie Comprehensive
Cancer Center, Chicago, IL, USA.
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