scholarly journals Leukopheresis therapy of leukemic reticuloendotheliosis (hairy cell leukemia)

Blood ◽  
1979 ◽  
Vol 54 (3) ◽  
pp. 747-749 ◽  
Author(s):  
JW Fay ◽  
JO Moore ◽  
GL Logue ◽  
AT Huang
Keyword(s):  
Lymph Node ◽  
Lymph Nodes ◽  
Hairy Cell Leukemia ◽  
Alternative Therapy ◽  
Leukemia Cell ◽  
Skin Lesions ◽  
Leukemia Cells ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Cell Counts

Abstract Intensive leukopheresis has been valuable in the short-term palliation of chronic lymphocytic and granulocytic leukemias. A 47-yr-old man with refractory leukemic reticuloendotheliosis (hairy cell leukemia) manifested by anemia, thrombocytopenia, elevated peripheral leukemia cell counts, generalized lymph node enlargement, and leukemic infiltrative skin disease was treated with serial leukopheresis. Removal of approximately 7 X 10(11) peripheral leukemia cells resulted in marked clinical and hematologic improvement with resolution of enlarged lymph nodes and clearing of skin infiltrates. At the time of this reporting, more than 400 wk since the last leukopheresis, the patient continues to do well. The improvement in all blood counts, reduction in lymph node size, and clearing of skin lesions paralleled the reduction of peripheral leukemia cell load by leukopheresis, suggesting mobilization of leukemia cells from marrow, lymph nodes, and skin. Removal of large numbers of leukemia cells in hairy cell leukemia has the potential of achieving sustained clinical improvement and may be a useful alternative therapy for these patients.

scholarly journals Leukopheresis therapy of leukemic reticuloendotheliosis (hairy cell leukemia)

Blood ◽  
1979 ◽  
Vol 54 (3) ◽  
pp. 747-749
Author(s):  
JW Fay ◽  
JO Moore ◽  
GL Logue ◽  
AT Huang
Keyword(s):  
Lymph Node ◽  
Lymph Nodes ◽  
Hairy Cell Leukemia ◽  
Alternative Therapy ◽  
Leukemia Cell ◽  
Skin Lesions ◽  
Leukemia Cells ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Cell Counts

Intensive leukopheresis has been valuable in the short-term palliation of chronic lymphocytic and granulocytic leukemias. A 47-yr-old man with refractory leukemic reticuloendotheliosis (hairy cell leukemia) manifested by anemia, thrombocytopenia, elevated peripheral leukemia cell counts, generalized lymph node enlargement, and leukemic infiltrative skin disease was treated with serial leukopheresis. Removal of approximately 7 X 10(11) peripheral leukemia cells resulted in marked clinical and hematologic improvement with resolution of enlarged lymph nodes and clearing of skin infiltrates. At the time of this reporting, more than 400 wk since the last leukopheresis, the patient continues to do well. The improvement in all blood counts, reduction in lymph node size, and clearing of skin lesions paralleled the reduction of peripheral leukemia cell load by leukopheresis, suggesting mobilization of leukemia cells from marrow, lymph nodes, and skin. Removal of large numbers of leukemia cells in hairy cell leukemia has the potential of achieving sustained clinical improvement and may be a useful alternative therapy for these patients.

Specific skin lesions in hairy cell leukemia at presentation: case report and review of literature

2009 ◽  
Vol 27 (2) ◽  
pp. 559-561 ◽  
Author(s):  
N. Čolović ◽  
M. Peruničić ◽  
V. Jurišić ◽  
M. Čolović
Keyword(s):  
Case Report ◽  
Hairy Cell Leukemia ◽  
Skin Lesions ◽  
Hairy Cell ◽  
Review Of Literature ◽  
Cell Leukemia

scholarly journals Polyclonal B-Cell Lymphocytosis With Features Resembling Hairy Cell Leukemia-Japanese Variant

Blood ◽  
1997 ◽  
Vol 89 (6) ◽  
pp. 2008-2014 ◽  
Author(s):  
Takashi Machii ◽  
Mitsuhiro Yamaguchi ◽  
Ryoichi Inoue ◽  
Yukihiro Tokumine ◽  
Hirohiko Kuratsune ◽  
...  
Keyword(s):  
B Cells ◽  
B Cell ◽  
Hairy Cell Leukemia ◽  
Leukemia Cells ◽  
Tartrate Resistant Acid Phosphatase ◽  
Variant Form ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Persistent Lymphocytosis ◽  
Biopsy Specimens

Abstract Polyclonal B lymphocytosis was found in four patients having clinical and hematologic features resembling those of hairy cell leukemia (HCL). All four patients were women between 37 and 67 years of age. Three patients had splenomegaly. Lymphadenopthy was absent or slight. Persistent lymphocytosis was seen in all the patients, and anemia and/or thrombopenia was observed in three of the patients. Abnormal lymphocytes have long microvilli and prominent membranous ruffles on their surfaces. Bone marrow aspirates and biopsy specimens showed increased numbers of abnormal lymphocytes with round nuclei and abundant pale cytoplasm. Although these findings were similar to those of HCL, studies of Ig gene rearrangements and expression showed the polyclonal proliferation of B cells. We called this new disease hairy B-cell lymphoproliferative disorder (HBLD). All four patients exhibited a polyclonal increase in serum IgG. The morphology of the cells in HBLD was more similar to that of leukemia cells of a variant form of HCL (HCL-Japanese variant) than to typical HCL cells. The surface IgG+, CD5−, CD11c+, CD22+, CD24−, CD25− phenotype and the weak tartrate-resistant acid phosphatase activity in the cells were identical to those of HCL cells of the Japanese variant. Our findings suggest that the B cells in HBLD are the nonmalignant counterpart of leukemic B cells in HCL-Japanese variant.

Comparative expressed sequence hybridization studies of hairy cell leukemia show uniform expression profile and imprint of spleen signature

Blood ◽  
2004 ◽  
Vol 104 (1) ◽  
pp. 250-255 ◽  
Author(s):  
Vera Vanhentenrijk ◽  
Chris De Wolf-Peeters ◽  
Iwona Wlodarska
Keyword(s):  
Lymph Node ◽  
Expression Profile ◽  
Hairy Cell Leukemia ◽  
Comparative Genomic ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Altered Expression ◽  
Marginal Zone B Cells ◽  
Genomic Imbalances ◽  
Expressed Sequence

Abstract Comparative expressed sequence hybridization (CESH) to chromosomes is a recently introduced technique that identifies chromosomal regions corresponding to a differential gene expression. This technique is analogous to comparative genomic hybridization (CGH) that detects genomic imbalances. We applied CESH for the study of hairy cell leukemia (HCL), a disorder with a largely unknown expression profile. Twelve HCL cases with spleen involvement were investigated by CESH and CGH. While the latter analysis identified only a few nonrecurrent genomic imbalances, CESH showed a consistent expression profile in all HCL cases. In addition, pairing normal spleen with normal lymph node, a “spleen signature” was established by CESH. This signature most likely reflects the expression profile of spleen-specific components, such as the sinusoidal lining cells from the red pulp and the marginal zone B cells from the white pulp. Imprint of the spleen signature was found in the HCL expression profile, suggesting that HCL may originate from a particular B-cell subset present in these splenic components. Besides pairing HCL with normal lymph node and spleen, we identified an “HCL signature” comprising several chromosome regions with altered expression. The most significantly underexpressed regions include 3p24, 3p21, 3q13.3-q22, 4p16, 11q23, 14q22-q24, 15q21-q22, 15q24-q25, and 17q22-q24; and 13q31 and Xq13.3-q21 were the most significantly overexpressed. These regions possibly harbor genes related to the biology and the pathogenesis of HCL. Their identification warrants further molecular investigations.

Occurrence of T-Lymphocytes with Aberrant Immunophenotypes Suggestive of Mature T-Cell Neoplasms In Patients with Hairy Cell Leukemia

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4165-4165
Author(s):  
Wolfgang Kern ◽  
Susanne Schnittger ◽  
Claudia Haferlach ◽  
Torsten Haferlach
Keyword(s):  
T Cell ◽  
T Lymphocytes ◽  
Hairy Cell Leukemia ◽  
Initial Diagnosis ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Employment Equity ◽  
Cell Counts ◽  
Equity Ownership

Abstract Abstract 4165 Mature T-Cell Neoplasms (MTN) comprise a heterogeneous group of diseases with largely varying clinical courses ranging from indolent cases which are asymptomatic for years to aggressive cases requiring immediate therapy. The incidence of MTN is increasing with age, however, particularly due to the large group of cases with very indolent clinical course MTN are considered underdiagnosed to a significant portion. Published data indicates that T-lymphocytes with aberrant immunophenotype, i.e. double-positive cell expressing both CD4 and CD8, are present in healthy subjects, however, their frequency in general amounts to clearly less than one percent of total leukocytes. With increasing age, these T-lymphocytes with aberrant immunophenotype are detected in more subjects and at higher frequencies suggesting a higher incidence of mature T-cell neoplasms or at least of pre-malignant conditions in these cases. The diagnosis of hairy cell leukemia has not yet been linked to a higher frequency of these T-lymphocytes with aberrant immunophenotype or of MTN. Following the identification of various cases with both a diagnosis of hairy cell leukemia and the presence of T-lymphocytes with aberrant immunophenotype in our laboratory we hypothesized that both of these conditions co-occur at a higher rate than would be expected by chance. We therefore retrospectively evaluated multiparameter immunophenotyping results of 338 patients diagnosed with hairy cell leukemia (newly diagnosed or during follow-up) between August 2005 and July 2010 for the presence of an increased percentage (more than 1%) of T-lymphocytes with an aberrant immunophenotype. We identified 31 such patients, i.e. 9.2% of all patients with hairy cell leukemia. 17 were identified at initial diagnosis and 14 during follow-up after therapy for hairy cell leukemia. The patients` ages ranged from 43.4 to 89.3 years (median, 62.1 years), 21 were male. The aberrant immunophenotype comprised the coexpression of CD3, CD4, and CD8 in all cases and in addition of CD56 in 25/31 (80.6%) cases. The median values and ranges for blood cell counts amounted to: WBC, 2.6 ×10e9/l, 1.0–24.6 ×10e9/l; hemoglobin, 12.7 g/dl, 7.3–16.5 g/dl; thrombocytes, 116 ×10e9/l, 24–258 ×10e9/l. The percentage of T-lymphocytes with an aberrant immunophenotype (compared to all leukocytes) ranged from 1% to 22% (median, 4%); the respective concentrations ranged from 0.013 ×10e9/l to 0.984 ×10e9/l (median, 0.122 ×10e9/l). The concentrations of T-lymphocytes with an aberrant immunophenotype tended to be higher in cases at follow-up as compared to those at initial diagnosis, although this difference was not significant (mean±SD, 0.266±0.275 ×10e9/l vs. 0.144±0.119 ×10e9/l). In four of the 31 patients with T-lymphocytes with an aberrant immunophenotype molecular genetic analysis of T-cell receptor (TCR) rearrangement was performed. In 3/4 patients both TCR beta and gamma were found rearranged and in one of these also TCR delta was rearranged, however, in 1/4 patient no TCR rearrangement was present. This data indicates that T-lymphocytes with aberrant immunophenotypes are present in patients with hairy cell leukemia much more often and at higher concentrations than in the general population. This data therefore suggests that the incidence of mature T-cell neoplasms may be higher in hairy cell leukemia patients. Clinical symptoms and findings like cytopenia and splenomegaly therefore may not be attributable to hairy cell leukemia alone which may have significant therapeutic implications. It is suggested to monitor for T-lymphocytes with aberrant immunophenotypes in patients with hairy cell leukemia and to perform analysis for TCR rearrangements in positive cases. Disclosures: Kern: MLL Munich Leukemia Laboratory: Employment, Equity Ownership. Schnittger:MLL Munich Leukemia Laboratory: Employment, Equity Ownership. Haferlach:MLL Munich Leukemia Laboratory: Employment, Equity Ownership. Haferlach:MLL Munich Leukemia Laboratory: Employment, Equity Ownership.

scholarly journals VH4-34+ hairy cell leukemia, a new variant with poor prognosis despite standard therapy

Blood ◽  
2009 ◽  
Vol 114 (21) ◽  
pp. 4687-4695 ◽  
Author(s):  
Evgeny Arons ◽  
Tara Suntum ◽  
Maryalice Stetler-Stevenson ◽  
Robert J. Kreitman
Keyword(s):  
Hairy Cell Leukemia ◽  
Poor Prognosis ◽  
Single Agent ◽  
Progression Free Survival ◽  
Poor Response ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Molecular Features ◽  
Cell Counts ◽  
New Variant

Abstract Hairy cell leukemia variant (HCLv) presents with high disease burden, lack of typical antigens like CD25, and poor response to standard treatments like cladribine. Occasionally, patients with classic HCL respond poorly. Clinical and molecular features of HCL and HCLv has not been compared. Rearrangements expressing immunoglobulin VH chain were sequenced, including 22 from 20 patients with HCLv and 63 from 62 patients with classic HCL. Most patients were seeking relapsed/refractory trials, representing a poor-prognosis population. VH4-34, a gene commonly used in autoimmune disorders, was observed in 8 (40%) HCLv and 6 (10%) classic (P = .004) HCL patients. Compared with 71 VH4-34− rearrangements, 14 VH4-34+ rearrangements were more frequently (P < .001) unmutated, defined as greater than 98% homologous to germline sequence. VH4-34+ patients had greater white blood cell counts at diagnosis (P = .002), lower response rate (P < .001) and progression-free survival (P = .007) after initial cladribine, and shorter overall survival from diagnosis (P < .001). Response and survival were more closely related to VH4-34 status than to whether or not patients had HCLv. VH4-34+ HCL is an important disorder that only partly overlaps with the previously described HCLv. Response to initial single-agent cladribine therapy is suboptimal; these patients should be considered for alternative approaches, including antibody-related therapy.

scholarly journals Hairy Cell Leukemia: An Autopsy Study

2003 ◽  
Vol 46 (4) ◽  
pp. 175-177 ◽  
Author(s):  
Karel Dědič
Keyword(s):  
Lymph Nodes ◽  
Cause Of Death ◽  
Hairy Cell Leukemia ◽  
Liver Involvement ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Autopsy Study ◽  
Massive Splenomegaly ◽  
Hematopoietic System ◽  
Leukemic Infiltration

Autopsy study from 21 patients with hairy cell leukemia was performed. All patients had the expected widespread involvement of the hematopoietic system. Leukemic infiltration of lymph nodes was detected in 12 cases. Liver involvement was present in 19 patients, leukemic infiltration ranged from focal portal and sinusoidal infiltration to massive infiltration that effaced the hepatic architecture. Twelve patients showed leukemic infiltration of the spleen, remaining 9 patients underwent previous splenectomy for massive splenomegaly. We also found leukemic infiltration of kidneys in four cases, two patients showed leukemic involvement of the lungs. The cause of death was related to impaired immunity (sepsis, bronchopneumonia, etc.) in the majority (73 %) of cases.

Analysis of Toxicity and Efficacy of Subcutaneous Cladribine at Reduced or Standard Doses (Five Versus Seven Consecutive Days) In Patients with Hairy Cell Leukemia (HCL) In the ICGHCL2004 Protocol by the Italian Cooperative Group on Hcl

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 701-701 ◽  
Author(s):  
Francesco Forconi ◽  
Emanuele Cencini ◽  
Francesco Zaja ◽  
Tamara Intermesoli ◽  
Francesca Fiore ◽  
...  
Keyword(s):  
Total Dose ◽  
Hairy Cell Leukemia ◽  
Hematological Toxicity ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Economic Implications ◽  
Lower Toxicity ◽  
Cell Counts ◽  
Second Tumors

Abstract Abstract 701 Introduction: Hairy cell leukemia (HCL) is generally responsive to intravenous Cladribine (iv2CdA). Subcutaneous Cladribine (sc2CdA) is an alternative route with 100% bioavailability. In indolent non-Hodgkin lymphomas other than HCL, at the dose of 0.7 mg/kg/cycle, efficacy of sc2CdA is similar to iv2CdA and reduction to 0.5 mg/kg/cycle determines equivalent efficacy and lower toxicity. Aims: In a national multicentre clinical trial (protocol EudraCT code: ICGHCL 2004), we prospectively evaluated toxicity and efficacy of sc2CdA given 0.1mg/kg/die for 5 (total dose 0.5 mg/kg, arm A) or for 7 consecutive days (total dose 0.7 mg/kg, arm B) as a single course in classic HCL requiring first treatment. Method: Clinical data and diagnostic samples were collected from all patients for central revision of classical HCL diagnosis (WHO criteria) and for molecular analyses at the University of Siena, prior to study entry. Early grade 3–4 toxicity was assessed on days 7 to 30 after treatment, according to the 2003 NCI/CTCAE v3 criteria and occurrence of second tumors was assessed at every subsequent follow-up. Enpoints of sc2CdA efficacy were response to treatment, treatment free interval (TFI), relapse free survival (RFS), time to second tumor (TTST) and overall survival (OS). Responses to treatment were assessed on days 60 and 180 after treatment and defined according to the 1987 Consensus criteria. Complete Remissions (CR) and Partial Remissions (PR) were rated as beneficial responses (CR/PR), while minor Responses (mR) and No Responses (NR) were rated as treatment failures (mR/NR). TFI was measured as the time elapsed from sc2CdA initiation to second treatment because of new progression or failure to sc2CdA. RFS was measured in patients with a CR/PR from treatment inititation to relapse. OS was measured from sc2CdA initiation to death for any cause. Result: of 156 patients screened centrally, 148 scored as classical HCL and entered the study. Gender (male total 116/148, 78,4%; arm A: 62/77, 80,5% vs arm B: 54/71, 76,1%), age (total: median age 52 years, range 30–83; arm A: median 56, range 30–83; arm B: median 51, range 33–82), clinical and laboratory parameters prior to treatment (including spleen, hemoglobin, platelet, leucocyte and hairy cell counts) were equally balanced in the two arms. Dose reduction was required in no patients from arm A and in 2 patients from arm B due to concurrent infection. Overall hematological toxicity was no different among the two treatment arms. Requirement of platelet transfusions (total 7/148, 4,7%; arm A: 2/77, 2,6% vs arm B: 5/71, 7%), red blood cell transfusion (total 32/148; 21,6%, arm A: 15/77, 19,5% vs arm B: 17/71, 23,9%), or G-CSF (total 102/148; 68,9%, arm A: 55/77, 71,4% vs arm B: 47/71, 66,2%) was no different among the two arms. However, a significantly higher non hematological toxicity (total 28/148; 18,9%, arm A: 9/77, 11,7% vs arm B: 19/71, 26,8%) was observed in arm B (p=.019). Non haematological toxicity was mainly represented by infections or FUO (total 25/148; 16,9%) that were significantly more frequent in arm B (arm A: 8/77, 10,4% vs arm B: 17/71, 23,9%, p=.028). Higher prevalence of toxicity resulted in a higher hospitalization rate in arm B than in arm A (total 29/148; 19,6%, arm A: 9/77, 11,7% vs arm B: 20/71, 28,2%, p=.012) and one early death was experienced because of lung aspergillosis in arm B. Onehundred-forty patients (94,6%) had a beneficial response (101 CR, 68,2%; 39 PR, 26,4%) and 8 (5,4%) failed treatment (5 mR, 3,4%; 3 NR, 2%). Responses were equivalent in the two arms, with 72/77 (93,5%) beneficial responses (49/77 CR, 63,6%; 23/77 PR, 29,9%; 4/77 mR, 5,2% and 1/77 NR, 1,3%) in arm A versus 68/71 (95,8%) beneficial responses in arm B (52/71 CR, 73,2%; 16/71 PR, 22,5%; 1/71 mR, 1,4%; 2/71 NR, 2,8%). After a median follow-up of 36 months (range 12–66), 5 year TFI, RFS, TTST and OS were 67%, 71% 87% and 94%, respectively. Causes of late death were 2 cardiac events and 3 second tumors. No differences of TFI, RFS, TTST and OS were observed in the 2 arms of treatment. Conclusion: The present data indicate that overall activity of sc2CdA is similar to iv2CdA (Cheson, 1998). Furthermore, this study indicates that sc2CdA given at 25% reduced doses (0.5 mg/kg) has equivalent activity and significantly lower toxicity than sc2CdA at standard doses (0.7 mg/kg). The reduced infection rates and hospitalization rates of sc2CdA have important pharmaco-economic implications. Disclosures: No relevant conflicts of interest to declare.

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