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

Article adapted by Medical News Today from original press release.
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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

Contact: Liz Savage
Journal of the National Cancer Institute

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

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Published: April 13, 2011

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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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