Hairy Cell Leukemia

Wednesday, April 11, 2012

Molecular Biologists Discover The Cause Of A Rare Type Of Leukemia

Main Category: Lymphoma / Leukemia / Myeloma
Also Included In: Cancer / Oncology; Blood / Hematology; Biology / Biochemistry
Article Date: 10 Jun 2011 - 8:00 PDT

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During the annual meeting of the European Hematology Association (EHA) professor E. Tiacci from the Institute of Hematology in Perugia, Italy will present groundbreaking news on the cause of a specific, rare subtype of leukemia, so-called hairy cell leukemia.

"With modern genetic technology we have discovered that in 46 out of 46 patients with hairy cell leukemia one specific gene in the DNA in the cell nucleus (BRAF gene) has undergone an irreversible change (called a "mutation"). Through this a cascade of events occurs which lead to continuous division/proliferation of the malignant cells in hairy cell leukemia"", says Tiacci.

Hairy cell leukemia is characterized by the piling up of leukemic cells in the bone marrow with a lack of production of normal blood cells, which might be life-threatening, as well as a large spleen, which may lead to significant complaints to the patients involved. Consequence of this discovery may be that specific drugs / molecules may be developed that inhibit the activated, mutated BRAF gene, thereby preventing continuous stimulation of leukemic cells to divide. Indeed, the first laboratory results employing these specific inhibitors show promising results.

About the EHA Annual Congress

After 15 congresses and constantly increasing number of delegates, the 16th Congress of EHA will take place in London. Hematology is a specialty that covers everything to do with blood: its origin in the bone marrow, diseases (in the production) of blood and their treatments. The latest data on research and developments within the wide spectrum of hematology are presented. The Congress is aimed at health professionals working in or interested in the field of hematology. The scientific program topics range from stem cell physiology and development, to leukemia; myeloma; lymphoma; diagnosis and treatment; red blood cells; white blood cells and platelet disorders; hemophilia; thrombosis and bleeding disorders as well as transfusion and stem cell transplantation. Last year the congress in Barcelona welcomed over 9,000 participants.

Source:
European Hematology Association

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Chronic Lymphocytic Leukemia and Hairy-Cell Leukemia

Chronic lymphocytic leukemia (CLL) is a clonal malignancy that results from expansion of the mature lymphocyte compartment. This expansion is a consequence of prolonged cell survival, rather than increased cellular proliferation. The affected lymphocytes are of B-cell lineage. Previously some cases were diagnosed as T-cell CLL but we now view those diseases as distinct entities and do not refer to them as CLL.

CLL is the most common leukemia in adults in Western countries, accounting for approximately 25% to 30% of all leukemias. The proportion of cases diagnosed with the early stages of the disease (Rai stage 0) has risen from 10% to 50%, probably because of earlier diagnosis (routine automated blood counts).

The incidence of CLL in the general population is 4.2:100,000 population, with an estimated death rate of 1.1:100,000 population. It was estimated that there will be 14,570 patients diagnosed with CLL in 2011 in the United States.

The male-to-female ratio is 2:1. There is little change with age, as the male-to-female ratio is 2.1:1 for patients < 65 years old, compared with 1.9:1 for those = 65 years old.

The median age at diagnosis is 72 years, and 70% of patients are > 65 years of age at diagnosis. CLL is rarely seen in younger patients, with < 2% being younger than 45 at the time of diagnosis.

In the American population, the incidence of CLL is similar in different races. However, the incidence is much lower in Asia (Japan, Korea, and China), Latin America, and Africa than in the United States and Western Europe.

The etiology of CLL is unclear. However, some factors associated with CLL have been identified.

There is a familial risk for CLL, with family members of patients with CLL having a twofold to sevenfold higher risk of developing the disease. CLL with a familial association tends to occur in younger individuals with subsequent generations, perhaps because of increased screening. Association with certain human lymphocyte antigen (HLA) patterns has not been consistent, and ongoing studies are attempting to identify susceptibility genes for CLL.

There is no documented association of CLL with exposure to radiation, alkylating agents, or known leukemogenic chemicals. However, exposure to some chemicals used in agriculture may increase the risk of developing CLL.

Associations between CLL and several viruses, including human T-cell lymphotrophic viruses I and II (HTLV-I and HTLV-II) and Epstein-Barr virus, have been suggested. However, no conclusive evidence of a causal relationship exists. Adult T-cell leukemia/lymphoma, a T-cell disorder that can resemble CLL, is caused by HTLV-I.

Recent studies suggest that over 4% of the population over 40 years of age harbors a population of clonal B cells with the phenotype of CLL or another B-cell malignancy, a condition now called monoclonal B-cell lymphocytosis (MBL). These asymptomatic individuals have no clinical evidence of disease and do not fulfill diagnostic criteria for CLL. All cases of CLL appear to be preceded by MBL, but most patients with MBL will not develop a hematologic malignancy.

In one study, 5.1% of patients > age 62 in the general population had monoclonal CLL-phenotype B cells. These asymptomatic individuals did not have lymphocytosis or clinical evidence of disease and did not fulfill diagnostic criteria for CLL. Whether or not these individuals will eventually develop diagnostic criteria or symptomatic disease is unknown. In that same study, patients with lymphocytosis (> 4,000 lymphocytes/µL) who developed CLL requiring treatment developed it at the rate of 1.1% per year.

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Tuesday, April 10, 2012

By Mayo Clinic staff

Hairy cell leukemia is a rare, slow-growing cancer of the blood in which your bone marrow makes too many B cells (lymphocytes), a type of white blood cell that fights infection. These excess B cells are abnormal and look "hairy" under a microscope. As the number of leukemia cells increases, fewer healthy white blood cells, red blood cells and platelets are produced.

Hairy cell leukemia affects more men than women, and it occurs most commonly in middle-aged or older adults.

Doctors aren't sure what causes hairy cell leukemia, and there is no cure. Hairy cell leukemia is considered a chronic disease because it may never completely disappear, although treatment can lead to a remission for years.

References Abeloff MD. Abeloff's Clinical Oncology. 4th ed. Philadelphia, Pa.: Churchill Livingstone; 2008:2309.Hoffman R, et al. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa.: Churchill Livingstone Elsevier; 2009. http://www.mdconsult.com/books/about.do?about=true&eid=4-u1.0-B978-0-443-06715-0..X5001-8--TOP&isbn=978-0-443-06715-0&uniqId=230100505-56. Accessed Feb. 2, 2012.Lichtman MA, et al. Williams Hematology. 8th ed. New York, N.Y.: The McGraw-Hill Companies; 2010. http://www.accessmedicine.com/resourceTOC.aspx?resourceID=69. Accessed Feb. 2, 2012.Hairy cell leukemia treatment (PDQ): Patient version. National Cancer Institute. http://www.cancer.gov/cancertopics/pdq/treatment/hairy-cell-leukemia/patient. Accessed Feb. 2, 2012.Grever MR, et al. Modern strategies for hairy cell leukemia. Journal of Clinical Oncology. 2011;29:583.Rituxan (prescribing information). South San Francisco, Calif.: Genentech Inc.; 2009. http://www.rituxan.com/index.html. Accessed Feb. 2, 2012.Integrative medicine & complementary and alternative therapies as part of blood cancer care. The Leukemia & Lymphoma Society. http://www.lls.org/#/resourcecenter/freeeducationmaterials/treatment/integrativemedandcam. Accessed Feb. 2, 2012.

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Monday, April 9, 2012

Gene Mutation Linked to Leukemia

(Ivanhoe Newswire) -- A new study reveals a single mutation, known as BRAF V600E, occurred in every patient in a group with hairy cell leukemia, suggesting this mutation could have implications for treating the disease.

According to the National Cancer Institute, hairy cell leukemia (HCL) is a cancer of the blood and bone marrow. It is a rare type of leukemia that slowly worsens or does not change at all. The disease is called "hairy" cell leukemia because the leukemia cells look "hairy" when viewed under a microscope.

The researchers found the BRAF V600E mutation appeared in 47 consecutive patients with HCL compared to zero of the 195 patients with other types of leukemias and lymphomas.

In-vitro studies showed that incubation of BRAF-mutated primary hairy cell leukemia cells with a specific inhibitor of BRAF let to a decrease in levels of phosphorylated ERK and MEK -- two known downstream targets of BRAF kinase.

"The BRAF V600E mutation was present in all patients with HCL who were evaluated. This finding may have implications for the pathogenesis, diagnosis, and targeted therapy of HCL," the study authors concluded.

SOURCE: New England Journal of Medicine, June 2011

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Review: Modern strategies for hairy cell leukaemia

In this review on hairy cell leukaemia, the following topics are discussed:

• Enormous progress through clinical investigation

• Establishing the correct diagnosis and initiating therapy

• Management decisions

• Importance of minimal residual disease

• Relapse and unresponsive disease

• Potential new avenues for therapeutic research

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Sunday, April 8, 2012

Study Examines Second Cancer Risk Among Hairy Cell Leukemia Survivors

Main Category: Lymphoma / Leukemia / Myeloma
Also Included In: Cancer / Oncology
Article Date: 09 Feb 2007 - 17:00 PDT

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Survivors of a rare cancer called hairy cell leukemia are at an increased risk of Hodgkin lymphoma, non-Hodgkin lymphoma, and thyroid cancer, and at an increased risk of death from leukemia and lymphoma, according to a new study. However, the absolute risk of those second cancers is small.

Hairy cell leukemia is a malignancy of a type of white blood cell called B lymphocytes. It accounts for about 2 percent of all leukemias. Better treatments have improved the prognosis for this disease, but the treatments may be associated with an increased risk of second cancers. To determine the risk of second cancers and to examine the causes of death among these patients, Michie Hisada, M.D., Sc.D., and colleagues at the National Cancer Institute examined the records for 3,104 patients with hairy cell leukemia who survived for at least two months after diagnosis.

They found that hairy cell leukemia survivors had a 6.6-fold increased risk of Hodgkin lymphoma, a 5-fold increased risk of non-Hodgkin lymphoma, a 3.6-fold increased risk of thyroid cancer, but a decreased risk of lung cancer compared with the general population. Among survivors, there was a lower risk of death from cardiovascular and cerebrovascular diseases than among the general population. "… Extrapolating from our results, among 10,000 hairy cell leukemia patients, a total excess of about 34 cancers - 21 non-Hodgkin lymphomas, 2 Hodgkin lymphomas, and 7 solid tumors (including two thyroid cancers) - might be observed per year," the authors write.

"Future studies should address the influence of such factors as changes in treatment regimens, immunologic impairment, natural history, diagnostic misclassification, and tobacco use on risk of second cancers in hairy cell leukemia patients," the authors conclude.

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Note: The Journal of the National Cancer Institute is published by Oxford University Press and is not affiliated with the National Cancer Institute. Attribution to the Journal of the National Cancer Institute is requested in all news coverage. Visit the Journal online at http://jncicancerspectrum.oxfordjournals.org/.

Other highlights in the February 7 JNCI

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AAN: Case Report Links Cladribine to PML (CME/CE)

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Published: April 13, 2011

Reviewed by Michael J. Olek, DO; Director, Newport Doctors Multiple Sclerosis Clinic and Dorothy Caputo, MA, RN, BC-ADM, CDE, Nurse Planner

Note that this study was published as an abstract and presented at a conference. These data and conclusions should be considered to be preliminary until published in a peer-reviewed journal.
Note that this report discussed a preliminary single case of a patient with hairy cell leukemia treated with cladribine developing progressive multifocal leukoencephalopathy.

HONOLULU -- A patient treated with cladribine for hairy cell leukemia went on to develop progressive multifocal leukoencephalopathy (PML), suggesting that the drug may pose a similar risk when used in multiple sclerosis (MS), French researchers reported here.

Cladribine, which suppresses lymphocyte proliferation and activity, is sometimes used off label in MS. Its manufacturer, German drug firm Merck KGaA, has been seeking regulatory approval to market the drug for the condition -- so far unsuccessfully, as both the FDA and its European counterpart have turned it down.

Marc Aletti, MD, and colleagues at the St. Anne French Military Hospital in Toulon, France, reported the leukemia patient's PML case here at the American Academy of Neurology's annual meeting.

The patient was about 81 when diagnosed with PML. He had initially presented with hairy cell leukemia in 2006 with moderate neutropenia and thrombocytopenia.

Both then and in 2007, he received courses of cladribine treatment at 0.12 mg/kg/day for five days. In 2009, he also received pentostatin at 4 mg/m2 for 15 days.

Symptoms of PML developed in May of 2010, including sleepiness and memory problems. These worsened rapidly over a three-week period, leading to the diagnosis. JC virus genetic matter was identified in his cerebrospinal fluid, helping to confirm the diagnosis.

Extremely low CD4- and CD8-positive cell counts -- 67 and 28 per mm3, respectively -- were found in his peripheral blood, along with moderate neutropenia and thrombocytopenia. There were no circulating tricholeukocytes and no evidence of HIV infection.

The man eventually recovered following eight months of cidofovir treatment, Aletti and colleagues reported.

PML is well known to occur with leukemias that depress normal leukocyte counts severely, but has been rare in association with hairy cell leukemia, Aletti and colleagues noted.

As a result, the researchers suspected that cladribine may have contributed to development of PML in this case.

"Cladribine may cause deep and prolonged lymphopenia, similar to an AIDS-like immune state, which potentially increases the risk of PML," according to their poster presentation.

They emphasized that the patient in this case did not have MS and that PML has not been reported in patients treated with cladribine for MS.

But Aletti and colleagues argued that "the risk for opportunistic infections in this situation" warrants clinicians' attention.

On the other hand, Robert Fox, MD, a neurologist at the Cleveland Clinic, told MedPage Today that he wasn't especially concerned about the report.

"Leukemia patients get PML," he said, pointing out that the rate is the important issue. He noted that rituximab (Rituxan) has been linked to PML in rheumatoid arthritis patients, but at a far smaller rate than has been seen with natalizumab (Tysabri) in MS patients.

The study had no external funding.

Study authors reported that they had no relevant financial interests.

Fox reported consulting or speaker fees from Biogen Idec, Genentech, and Teva, and research funding from Biogen Idec and Genentech.

Primary source: Neurology
Source reference:
Aletti M, et al "Progressive multifocal leukoencephalopathy after cladribine treatment for hairy cell leukemia" Neurology 2011; 76:A28.

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Saturday, April 7, 2012

Single Mutation Key to Hairy Cell Leukemia (CME/CE)

By Charles Bankhead, Staff Writer, MedPage Today

Published: June 11, 2011

Reviewed by Robert Jasmer, MD; Associate Clinical Professor of Medicine, University of California, San Francisco and Dorothy Caputo, MA, RN, BC-ADM, CDE, Nurse Planner

Explain that a single mutation (BRAF V600E) occurred in every patient in a cohort with hairy cell leukemia, suggesting that targeting the mutation could have major implications for treating the disease.
Note that the method used was genome-wide massively parallel sequencing of tumor and normal cells from the same patient to identify recurrent somatic mutations.

A single mutation occurred in every patient in a cohort with hairy cell leukemia, suggesting that targeting the mutation could have major implications for treating the disease, Italian investigators reported.

The same BRAF V600E mutation appeared in 47 consecutive patients with hairy cell leukemia versus none of 195 patients with other types of peripheral B-cell lymphomas and leukemias.

In vitro studies showed that incubation of BRAF-mutated primary hairy cell leukemia cells with a specific inhibitor of BRAF led to a marked decrease in levels of two known downstream targets of BRAF kinase, according to an article published online in the New England Journal of Medicine.

"The BRAF V600E mutant is a potential therapeutic target in patients with hairy cell leukemia who do not have a response (or have a suboptimal response) to initial therapy with purine analogs, as well as in patients with repeated relapses or unacceptable toxic effects," Brunangelo Falini, MD, of the University of Perugia, and co-authors wrote in conclusion.

"Notably, BRAF V600E inhibitors have shown remarkable activity in patients with BRAF-mutated metastatic melanoma. These results, along with our in vitro finding that a specific active BRAF inhibitor causes MEK and ERK dephosphorylation in primary hairy cell leukemia cells, warrant the clinical testing of active BRAF inhibitors."

The findings were reported simultaneously at the European Hematology Association meeting.

Despite advances in the diagnosis and treatment of hairy cell leukemia over the past 50 years, little progress has occurred toward elucidation of the underlying genetic alterations associated with disease.

Gene-expression profiling has failed to pinpoint any recurrent genetic alterations, nor has genome-wide single-nucleotide polymorphism genotyping, the authors wrote in the introduction to their findings.

Falini and colleagues used a more powerful approach to examining the genetic basis of cancer: genome-wide massively parallel sequencing of tumor and normal cells from the same patient. They sought to identify recurrent somatic mutations in protein-coding genes, with the goal of gaining more insight into the origin of the disease and identifying new options for diagnosis and treatment.

Investigators obtained leukemic and nonleukemic genomic DNA from a single patient with hairy cell leukemia. By use of massively parallel sequencing, candidate somatic mutations were identified. Researchers used polymerase-chain-reaction (PCR) amplification and direct DNA sequencing to validate the findings from the index patient.

Falini and colleagues performed whole-exome sequencing of genomic DNA from the index patient and identified five unique variants specific to tumor DNA, the best known of which was BRAF.

The amino acid substitution at position 600 of the BRAF protein (V600E) is associated with several solid tumors. At the recent American Society of Clinical Oncology meeting, treatment with a BRAF inhibitor led to unprecedented activity in patients with metastatic melanoma.

With the finding from the index patient, Falini and colleagues screened DNA samples for 47 additional patients with hairy cell leukemia and the 195 patients with other leukemias and lymphomas. They identified the V600E mutation in samples from all of the patients with hairy cell leukemia but none of the patients with other hematologic malignancies.

Working from the understanding that V600E constitutively activates BRAF kinase activity, Falini and colleagues evaluated the phosphorylation status of MEK and ERK. The former is the kinase target immediately downstream from BRAF and the latter is the kinase phosphorylated by MEK.

Using antibodies specific to phosphorylated MEK and ERK, the investigators confirmed the presence of the two kinases in DNA specimens from five patients with BRAF-mutated hairy cell leukemia. Introduction of the BRAF inhibitor PLX-4720 led to marked decrease in phosphorylated MEK and ERK, whereas vehicle-treated samples retained MEK and ERK phosphorylation.

Falini had no relevant disclosures. Co-author Robin Foa disclosed relationships with Roche, Bristol-Myers Squibb, GlaxoSmithKline, and Celgene.

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TrovaGene, Inc., Announces Worldwide Exclusive Licensing of Assay for Hairy Cell Leukemia

SAN DIEGO--(Healthcare Sales & Marketing Network)-- TrovaGene, Inc. (Pink Sheets:TROV.pk ), a developer of transrenal molecular diagnostics to facilitate personalized medicine, has signed a worldwide exclusive license for an assay that detects Hairy Cell Leukemia (HCL).

A recent discovery, published in the June 16, 2011, New England Journal of Medicine by Dr. Brunangelo Falini and colleagues, showed that a specific mutation in the BRAF gene was present in all patients with HCL in the study. TrovaGene holds exclusive rights to the discovery and will offer nonexclusive licenses for its diagnostic application. The results of such a test will also help physicians to monitor effectiveness of treatment and disease relapse.

?We are pleased to enter into this collaboration with Dr. Falini and his colleagues as we continue to build our franchise in the diagnosis and treatment of leukemia and lymphoma,? said Dr. Tom Adams, Chairman of TrovaGene. ?This new test for the diagnosis of Hairy Cell Leukemia (HCL) based on the identification of a specific BRAF gene mutation is unique in that it represents an objective, reproducible, specific and sensitive DNA-based test for the diagnosis of HCL. It also provides an immediate therapeutic indication for the use of available anti-B-RAF drugs.?

HCL is a cancer of the bone marrow resulting in accumulation of abnormal B lymphocytes in the blood. There are about 2,000 new cases of HCL diagnosed annually in the U.S. and Europe. Most patients are successfully treated with cladribine or pentostatin with 80% of patients achieving a complete response. Patients who relapse often respond successfully to retreatment. Monitoring for relapse is typically performed by routine complete blood count (CBC) but may include bone marrow testing. The name stems from the hairy appearance of the abnormal B lymphocytes under a microscope, visible in about 85% of HCL cases.

About TrovaGene, Inc.

Headquartered in San Diego, California, TrovaGene has focused on development of tests using its patented technology to detect transrenal DNA and RNA, short nucleic acid fragments from normal and diseased cell death that cross the kidney barrier and can be detected in urine.

TrovaGene has a dominant patent position as relates to transrenal molecular testing. It has U.S. and European patent applications and issued patents that cover testing for HPV and other infectious diseases, cancer, transplantation, prenatal and genetic testing. In addition, it owns worldwide rights to nucleophosmin-1 (NPM1), an informative biomarker for acute myeloid leukemia (AML).

TrovaGene is currently in the process of auditing its financial statements and preparing a Form 10 registration statement so that it can report on a current basis with the Securities and Exchange Commission. A filing is anticipated during the third quarter of 2011. More complete current information about TrovaGene will be contained in the filing.

Certain statements in this press release are forward-looking within the meaning of the Private Securities Litigation Reform Act of 1995. These statements may be identified by the use of forward-looking words such as "anticipate," "believe," "forecast," "estimated" and "intend," among others. These forward-looking statements are based on TrovaGene's current expectations and actual results could differ materially. There are a number of factors that could cause actual events to differ materially from those indicated by such forward-looking statements. These factors include, but are not limited to, substantial competition; our ability to continue as a going concern; our need for additional financing; uncertainties of patent protection and litigation; uncertainties of government or third party payer reimbursement; limited sales and marketing efforts and dependence upon third parties; and risks related to failure to obtain FDA clearances or approvals and noncompliance with FDA regulations. As with any medical diagnostic tests under development, there are significant risks in the development, regulatory approval and commercialization of new products. There are no guarantees that future clinical trials discussed in this press release will be completed or successful or that any product will receive regulatory approval for any indication or prove to be commercially successful. TrovaGene does not undertake an obligation to update or revise any forward-looking statement.

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Friday, April 6, 2012

50 Years Of Hairy-Cell Leukemia Research To Be Observed

Public release date: 5-Dec-2008
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Contact: Eileen Scahill
Eileen.Scahill@osumc.edu
614-390-9832
Ohio State University Medical Center

COLUMBUS, Ohio ? In 1958, Ohio State University cancer researcher Dr. Bertha Bouroncle first identified a deadly disease now known as hairy-cell leukemia, a once fatal disease that can now be effectively treated.

Now, 50 years later researchers from across the globe are gathering for a symposium titled "50 years of Enormous Progress in Hairy Cell Leukemia: A Celebration of Clinical Research with Remaining Unanswered Questions."

The free Friday Satellite Symposium, which precedes the 50th American Society of Hematology Annual Meeting, will be held from 12:30 to 4:30 p.m. PST Friday (12/5) at the Moscone Convention Center West, 888 Howard St., San Francisco, Level 3, Room 3000/3002/3004.

Hairy-cell leukemia is a relatively rare form of adult chronic leukemia that affects white blood cells called B lymphocytes. This disease was once uniformly fatal, but highly successful therapies have been developed, and patients today who receive proper treatment can have a relatively normal quality of life.

Dr. Michael Grever, chairman of the department of internal medicine at Ohio State and co-leader of the Experimental Therapeutics program at The Ohio State University Comprehensive Cancer Center ? James Cancer Hospital and Solove Research Institute, is one of seven leukemia experts who will lead the meeting. The investigators also plan to establish an international association devoted to hairy-cell leukemia research.

"This seminar celebrates the progress we've made in hairy-cell leukemia, yet recognizes the work that still needs to be done," said Grever, who specializes in hematologic malignancies. "We want to show practicing oncologists that in the past 50 years, we've gone from a fatal, untreatable disease to one that can be treated effectively, allowing patients to live relatively normal lives."

Other presenters include Dr. John Cawley, department of haematology at the University of Liverpool in the U.K.; Dr. Robert J. Kreitman, clinical immunotherapy section, National Institutes of Health in Bethesda, Maryland; Francesco Lauria, division of hematology and transplantation medicine immunological services, University of Siena in Siena, Italy; Dr. Alan Saven, division of hematology and oncology, Scripps Clinic, and Ida M. and Cecil Green Cancer Center, La Jolla, Calif.; Dr. Deborah Thomas, department of leukemia, University of Texas M.D. Anderson Cancer Center in Houston; and Dr. Pier Luigi Zinzani, University of Bologna in Bologna, Italy.

Hairy-cell leukemia is rare, accounting for only about 500 new cases each year, or about 2 percent of all leukemias. As a result, hematologists may encounter this disease only a few times in their career.

Symposium attendees will learn to: Identify the clinical presentation and complications associated with hairy-cell leukemia. Recognize the correct diagnosis and distinguish this disease from numerous lymphoid malignancies. Indicate the correct time to initiate systemic therapy and select the appropriate regimen. Outline the management of care when patients relapse or fail to respond to therapy, and utilize supportive care to prevent and manage infection in the patient with hairy-cell leukemia.

"We still need to do more research," says Grever. "This is like taking the football down to the 5-yard line and not getting the touchdown."

Hairy-cell leukemia is seven times more common among elderly men than elderly women. Patients with this disease often have low platelet counts and blood counts, anemia and are at high risk for developing infections, Grever said. Symptoms may include fatigue, infections and weight loss.

The Ohio State University Comprehensive Cancer Center-James Cancer Hospital and Solove Research Institute is one of only 40 NCI-designated Comprehensive Cancer Centers in the United States and the only freestanding cancer hospital in the Midwest. Ranked among the top 20 cancer hospitals in the nation, The James is the 172-bed adult patient-care component of the cancer program at The Ohio State University.

Click here for a high-quality JPEG of Dr. Michael Grever: http://medicine.osu.edu/news/images/high_quality/grever_michael.jpg

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Thursday, April 5, 2012

Cladribine led to long-term eradication of hairy cell leukemia

Patients with hairy cell leukemia can have a long-term complete hematologic response after a one-week course of cladribine, a cytotoxic chemotherapy.

Researchers searched the Scripps Clinic cladribine hairy cell leukemia computer database for patients who were alive and in continuous complete hematologic remission after being treated with a single seven-day course of cladribine for hairy cell leukemia. They wanted to identify whether those patients assigned cladribine with long-term response had no minimal residual disease.

They identified 19 patients who had evaluable bone marrow tissue specimens. Patients received cladribine as a seven-day continuous IV infusion at 0.085 mg/kg per day to 0.1 mg/kg per day. The median time from diagnosis was 18 years.

All patients underwent biopsy and a bone marrow aspiration. Researchers performed CD20 immunostaining on all samples to identify cells characteristic of hairy cell leukemia and quantify residual disease as a percentage of marrow cellularity.

At a median follow-up of 16 years, nine patients (47%) appeared to have no evidence of residual disease. Of the 53% of patients with residual disease, seven (37%) had minimal residual disease and three (16%) had morphologic disease.

“While not as sensitive as clone-specific [polymerase chain reaction], the negative assays of two tests that are still highly sensitive coupled with very extended follow-up of these patients raises the possibility that a single course of cladribine can potentially cure [hairy cell leukemia],” the researchers wrote. “However, declaring cures in low-grade lymphoproliferative disorders must always be done cautiously, as very late relapses are known to occur.”

Sigal DS. Blood. 2010;doi:10.1128/blood-2009-10-251645.

Wednesday, April 4, 2012

Hairy-Cell Leukemia: 50 Years Of Research

In 1958, Ohio State University cancer researcher Dr. Bertha Bouroncle first identified a deadly disease now known as hairy-cell leukemia, a once fatal disease that can now be effectively treated.

Dr. Michael Grever, chairman of the department of internal medicine at Ohio State and co-leader of the Experimental Therapeutics program at The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute, is one of seven leukemia experts who will lead the meeting. The investigators also plan to establish an international association devoted to hairy-cell leukemia research.

Grever will discuss long-term follow-up studies of patients who were treated for hairy-cell leukemia, including studies he conducted with Bouroncle and Dr. Eric Kraut at Ohio State's Medical Center. Bouroncle spent her entire career at Ohio State, and is now a faculty emeritus in the department of internal medicine. Hairy-cell leukemia is seven times more common among elderly men than elderly women. Patients with this disease often have low platelet counts and blood counts, anemia and are at high risk for developing infections, Grever said. Symptoms may include fatigue, infections and weight loss.

Source: Eileen Scahill

Ohio State University Medical Center

Article adapted by Medical News Today from original press release.

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Tuesday, April 3, 2012

Hairy cell leukemia - Information

Hairy cell leukemia (HCL) is a rare cancer of the blood. It affects B cells, a type of white blood cell (lymphocyte). HCL is due to increase of a clonal malignant B cell that infiltrates the reticuloendothelial cells, mainly the bone marrow, resulting in bone marrow failure. Somewhere between 600 and 800 people are effected with hairy cell leukemia every year in the United States. Hairy cell leukemia affects more men than women, and it occurs most commonly in middle-aged or older adults.

Children and teenagers don't get hairy cell leukemia. This disease is observed more commonly in whites. Occasionally, hairy cell leukemia has occurred in members of the same family. However, this is uncommon and no hereditary pattern has been established. Hairy cell leukemia (HCL) is associated with gram-positive and gram-negative bacterial infections as well as atypical mycobacterial and invasive fungal infections.

HCL is related with other general immunologic disorders with scleroderma, polymyositis, polyarteritis nodosa, erythematous maculopapules, and pyoderma gangrenosum. Other rare conditions may be linked with HCL, such as acquired issue VIII antibodies, paraproteinemia, and systemic mast cell disease. The symptoms and signs of hairy cell leukemia are nonspecific and resemble those of other illnesses.

The most common symptoms and presenting complaints are weakness and tiredness due to anemia. Some symptoms and signs are pain or fullness in the upper left side of the abdomen, as a effect of an enlarged spleen, on explained weight loss, a loss of a sense of well-being and an infection accompanied by fever and chills. Hairy cells accumulate in the bone marrow and prevent the marrow from producing enough normal blood cells. People with this disease may not need treatment in the early stages.

Several treatments are available, and successful manage of the disease is common. Chemotherapy is cancer treatments that utilize drugs to prevent the growth of cancer cells, either by killing the cells or by stopping them from dividing. Biological therapy (immunotherapy) attempts to make cancer cells more recognizable to your immune system. Surgery to remove your spleen (splenectomy) was the first treatment used in hairy cell leukemia, though it's used only rarely today.