Citizendia

Hairy cell leukemia
Classification and external resources
ICD-10C91.4
ICD-9202.4
ICD-O:M9940/3
DiseasesDB5589
eMedicinemed/937 
MeSHD007943

Hairy cell leukemia is a mature B cell neoplasm. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books It is usually classified as a sub-type of chronic lymphoid leukemia for convenience. Chronic lymphocytic leukemia (also known as "chronic lymphoid leukemia" or "CLL" is a type of Leukemia, or cancer of the white blood cells ( Lymphocytes It is uncommon, representing about 2% of all leukemias, or less than a total of 2000 new cases diagnosed each year in North America and Western Europe combined.

Originally known as histiocytic leukemia, malignant reticulosis, or lymphoid myelofibrosis in publications dating back to the 1920s, this disease was formally named leukemic reticuloendotheliosis and its characterization significantly advanced by Bertha Bouroncle, M. D. and her colleagues at the Ohio State University College of Medicine in 1958. Its common name, which was coined in 1966[1], is derived from the appearance of the cells under a microscope.

Contents

Classification

Two variants have been described: Hairy cell leukemia-variant[1], which usually is diagnosed in men and a Japanese variant. The non-Japanese variant is more difficult to treat than either 'classic' HCL or the Japanese variant HCL.

Hairy cell leukemia-variant, or HCL-V, is usually described as a prolymphocytic variant of hairy cell leukemia. [2] It was first formally described in 1980 by a paper from the University of Cambridge's Hayhoe lab. [3] About 10% of HCL patients have this variant form of the disease, representing about 60-75 new HCL-V patients each year in the U. S. While classic HCL primarily affects men, HCL-V is somewhat more evenly divided between males and females. [4] While the disease can appear at any age, the median age at diagnosis is over 70. [5]

Similar to B-PLL in Chronic Lymphocytic Leukemia, HCL-V is a more aggressive disease. B-cell prolymphocytic leukemia is a more aggressive but still treatable form of Chronic lymphocytic leukemia. Chronic lymphocytic leukemia (also known as "chronic lymphoid leukemia" or "CLL" is a type of Leukemia, or cancer of the white blood cells ( Lymphocytes It is less likely to be treated successfully than classic HCL and remissions tend to be shorter. Many treatment approaches, such as Interferon-alpha, CHOP and common alkylating agents like cyclophosphamide provide very little benefit. Interferons ( IFN s are natural Proteins produced by the cells of the Immune system of most Vertebrates in response to challenges by foreign agents Cyclophosphamide (the generic name for Cytoxan Neosar Revimmune also known as cytophosphane is a Nitrogen mustard alkylating agent, from the oxazophorines group [4] Pentostatin and cladribine provide some benefit to many HCL-V patients, but with shorter remissions and lower response rates compared to classic HCL. More than half of patients respond partially to splenectomy. [4]

In terms of B cell development, the prolymphocytes are less developed than lymphocyte cells or plasma cells, but are still more developed than their lymphoblastic precursors. A lymphocyte is a type of White blood cell in the Vertebrate Immune system.

HCL-V differs from classic HCL principally in these respects:

The lack of CD25, which is part of the receptor for a key immunoregulating hormone, may explain why HCL-V cases are normally resistant to treatment by immune system hormones. [8]

HCL-V, which has a high proportion of hairy cells without a functional p53 tumor suppressor gene, is somewhat more likely to transform into a higher-grade disease, with Daniel Catovsky suggesting a typical transformation rate of 5% in the U. p53 (also known as protein 53 or tumor protein 53) is a Transcription factor encoded by the TP53 gene K. , which is similar to the Richter's transformation rate for SLVL and CLL[4] and reporting 6% in one group of patients. Richter's transformation, or Richter's syndrome (RS is a rare complication of B cell Chronic lymphocytic leukemia (CLL or Hairy cell leukemia (HCL [9] Among HCL-V patients, the most aggressive cases normally have the least amount of p53 gene activity. [10] Hairy cells without the p53 gene tend, over time, to displace the less aggressive p53+ hairy cells.

Hairy cell leukemia-Japanese variant or HCL-J. There is also a Japanese variant, which is more easily treated.

Symptoms

In hairy cell leukemia, the broken "hairy cells" build up in the bone marrow, which means that the bone marrow has difficulty producing enough normal cells: white blood cells to fight infections, red blood cells to carry oxygen, and platelets to stop bleeding. Red blood cells are the most common type of Blood cell and the Vertebrate body's principal means of delivering Oxygen to the body tissues via the Blood Platelets, or Thrombocytes, are small cytoplasmic bodies derived from cells They circulate in the Blood of Mammals and are involved Consequently, patients usually present with infection, anemia-related fatigue, and/or easy bleeding. An infection is the detrimental Colonization of a host Organism by a foreign Species. Anemia ( AmE) or anæmia/anaemia ( BrE) (from the Ancient Greek grc-Latn anaîmia, meaning “without blood” is defined as a qualitative [11]

Most symptoms are often vague, such as "persistent fatigue" or "not feeling well. " Some of the leukemic cells may gather in the spleen and cause it to swell; this can have the side effect of making the person feel full even when he or she has not eaten much. The spleen is an organ found in all Vertebrate animals In humans the spleen is located in the abdomen of the body where it functions in the destruction of redundant Red

Hairy cell leukemia is commonly diagnosed after a routine blood count shows unexpectedly low numbers for one or more kinds of blood cells, or after unexplained bruises or unexplained infections, such as repeated bouts of pneumonia in an otherwise apparently healthy patient.

Platelet function may be somewhat impaired in HCL patients, although this does not appear to have any significant practical effect. [12] It may result in somewhat more mild bruises than would otherwise be expected for a given platelet count or a mildly increased bleeding time for a minor cut. It is likely the result of producing slightly abnormal platelets in the overstressed bone marrow tissue.

Patients with a high tumor burden may also have somewhat reduced levels of cholesterol,[13] especially in patients with an enlarged spleen. [14] Cholesterol levels return to more normal values with successful treatment of HCL.

Cause

The cause is unknown, but it is generally accepted that it is not caused by tobacco, ionizing radiation, pesticides, or industrial chemicals other than possibly diesel. [15] Farming and gardening appear to increase the risk in some studies. [16] The possibility that HCL is caused by a random accident during routine cell division can not be ruled out.

Diagnosis

The diagnostic path may have begun with a simple test like a complete blood count (also called a full blood count), but this is not adequate to diagnose HCL. A complete blood count ( CBC) also known as full blood count ( FBC) or full blood exam ( FBE) or blood panel, is A CBC normally shows low counts for white blood cells, red blood cells, and platelets in HCL patients. However, if large numbers of hairy cells are in the blood stream, then normal or even high lymphocyte counts may be found. Lymphocytosis is an increase in the number or proportion of Lymphocytes in the blood usually detected when a Complete blood count is routinely obtained

Most patients require a bone marrow biopsy for proper diagnosis. Bone marrow examination refers to the pathologic analysis of samples of Bone marrow obtained by bone marrow biopsy (often called a Trephine biopsy The bone marrow biopsy is used to confirm the presence of HCL and also the absence of any secondary disease. Abnormal white blood cells bearing hair-like projections from the cytoplasm are seen on blood film examination or bone marrow biopsy. A Blood Film or Peripheral Blood Smear is a slide made from a drop of Blood, that allows the cells to be examined microscopically The diagnosis can be confirmed by viewing the cells with a special stain, known as TRAP, or tartrate resistant acid phosphatase. Tartrate resistant acid phosphatase is a Glycosylated monomeric metalloenzyme expressed in mammals

It is also possible to definitively diagnose hairy cell leukemia through a flow cytometry blood test, which identifies characteristic proteins on the cell surfaces. Flow cytometry is a technique for counting examining and sorting microscopic particles suspended in a stream of fluid These cancerous cells are larger than normal and positive for CD19, CD20, CD22, CD11c, CD25, CD103, and FMC7. CD19 ( C luster of D ifferentiation 19) is a human Protein encoded by the Gene. CD20 is a non- Glycosylated phosphoprotein expressed on the surface of all mature B-cells. CD22 or Cluster of differentiation -22 is a molecule belonging to the SIGLEC family of Lectins Generally speaking CD22 is a regulatory molecule that prevents CD11c, also known as Integrin alpha X (complement component 3 receptor 4 subunit ( ITGAX) is a human Gene. The interleukin-2 receptor (IL-2R is heterotrimeric protein expressed on the surface of certain immune cells such as Lymphocytes that binds and responds to Integrin alpha E ( ITGAE) also known as CD103 ( C luster of D ifferentiation 103) is an Integrin. CD20 is a non- Glycosylated phosphoprotein expressed on the surface of all mature B-cells. [17] Hairy cell leukemia-variant (HCL-V), which shares some characteristics with B cell prolymphocytic leukemia (B-PLL), does not show CD25 (also called the Interleukin-2 receptor, alpha). As this is relatively new and expensive technology, its adoption by physicians is not uniform, despite the advantages of comfort, simplicity, and safety for the patient when compared to a bone marrow biopsy.

Because a patient could have more than one similar disease, it is also necessary to rule out the presence of leukemias and lymphomas such as SMZL or B-PLL. Splenic marginal zone lymphoma (SMZL is a Lymphoma comprised of B-cells that replace the normal architecture of the White pulp of the Spleen B-cell prolymphocytic leukemia is a more aggressive but still treatable form of Chronic lymphocytic leukemia. The presence of these diseases is easily checked during a flow cytometry test, where they characteristically show different results. [18] Careful review of bone marrow biopsy samples is also reliable for this purpose.

On physical exam, patients may display massive splenomegaly. This is less likely among patients who are diagnosed through routine blood work, when the disease is at an early stage. Enlarged lymph nodes appear in a few patients. Lymphadenopathy is a term meaning "disease of the Lymph nodes.

The differential diagnoses include: several kinds of anemia, including myelophthisis and aplastic anemia,[19] and most kinds of blood neoplasms, including hypoplastic myelodysplastic syndrome, atypical chronic lymphocytic leukemia, B-cell prolymphocytic leukemia, or idiopathic myelofibrosis. Anemia ( AmE) or anæmia/anaemia ( BrE) (from the Ancient Greek grc-Latn anaîmia, meaning “without blood” is defined as a qualitative The myelodysplastic syndromes (MDS formerly known as "preleukemia" are a diverse collection of hematological conditions united by ineffective production (or B-cell prolymphocytic leukemia is a more aggressive but still treatable form of Chronic lymphocytic leukemia. Myelofibrosis, also known as myeloid metaplasia, chronic idiopathic myelofibrosis, and primary myelofibrosis, is a disorder of the Bone marrow [20]

Pathology of hairy cells

Hairy cells are nearly mature, activated clonal cells with signs of VH gene differentiation. [21] They may be related to memory cells. Memory B cells are a B cell sub-type that are formed following primary infection

While there are few genomic imbalances in the hairy cells, the expression of genes is dysregulated in a complex and specific pattern. Comparative genomic hybridization ( CGH) or Chromosomal Microarray Analysis ( CMA) is a molecular- Cytogenetic method for the analysis of Gene expression is the process by which inheritable information from a Gene, such as the DNA sequence, is made into a functional Gene product, such The cells underexpress 3p24, 3p21, 3q13. 3-q22, 4p16, 11q23, 14q22-q24, 15q21-q22, 15q24-q25, and 17q22-q24 and overexpress 13q31 and Xq13. 3-q21. [22] It has not yet been demonstrated that any of these changes have any practical significance to the patient.

Treatment

Several treatments are available, and successful control of the disease is common.

Not everyone needs treatment. Treatment is usually given when the symptoms of the disease interfere with the patient's everyday life, or when white blood cell or platelet counts decline to dangerously low levels, such as an absolute neutrophil count below one thousand cells per microliter (1. 0 K/uL). Not all patients need treatment immediately upon diagnosis, and about 10% of patients will never need treatment.

Treatment delays are less important than in solid tumors. Unlike most cancers, treatment success does not depend on treating the disease at an early stage. Because delays do not affect treatment success, there are no standards for how quickly a patient should receive treatment. However, waiting too long can cause its own problems, such as an infection that might have been avoided by proper treatment to restore immune system function. Also, having a higher number of hairy cells at the time of treatment can make certain side effects somewhat worse, as some side effects are primarily caused by the body's natural response to the dying hairy cells. This can result in the hospitalization of a patient whose treatment would otherwise be carried out entirely at his hematologist's office.

Single-drug treatment is normal. Unlike most cancers, only one drug is normally given to a patient at a time. While monotherapy is normal, combination therapy -- typically using one first-line therapy and one second-line therapy -- is being studied in current clinical trials and is used more frequently for refractory cases. It is unclear whether combining rituximab with cladribine or pentostatin will produce any practical benefit to the patient. [2] Combination therapy is almost never used with a new patient. Because the success rates with purine analog monotherapy are already so high, the additional benefit from immediate treatment with a second drug in a treatment-naïve patient is very low. For example, one round of either cladribine or pentostatin gives the median first-time patient a ten-year remission; the addition of rituximab, which gives the median patient only three or four years, is reasonably expected to provide no additional value for this easily treated patient. In a more difficult case, however, the benefit from the first drug may be substantially reduced and therefore a combination may provide some benefit.

First-line therapy: purine analog chemotherapy

Cladribine (2CDA) and pentostatin (DCF) are the two most common first-line therapies. Cladribine is a drug commonly used to treat Hairy cell leukemia (leukemic reticuloendotheliosis Pentostatin (Deoxycoformycin is an anticancer Chemotherapeutic drug Cladribine is a kind of mild chemotherapy that can be administered by injection under the skin, by infusion over a couple of hours into a vein, or by a pump worn by the patient that provides a slow drip into a vein, 24 hours a day for 7 days. Most patients receive cladribine by IV infusion once a day for five to seven days, but more patients are being given the option of taking this drug once a week for six weeks. The different dosing schedules used with cladribine are approximately equally effective and equally safe. [23] Relatively few patients have significant side effects other than fatigue and a high fever caused by the cancer cells dying, although complications like infection and acute kidney failure have been seen.

Pentostatin is chemically similar to cladribine, and has a similar success rate and side effect profile, but it is always given over a much longer period of time, usually one dose by IV infusion every two weeks for three to six months.

(A third related chemical, fludarabine, is not used for hairy cell leukemia, despite being chemically similar. Fludarabine (marketed as fludarabine phosphate under the trade name Fludara) is a Chemotherapy drug used in the treatment of hematological malignancies )

During the weeks following treatment the patient's immune system is severely weakened, but his bone marrow will begin to produce normal blood cells again. An immune system is a collection of mechanisms within an Organism that protects against Disease by identifying and killing Pathogens and Tumor Bone marrow is the flexible tissue found in the hollow interior of Bones In adults marrow in large bones produces new Blood cells It constitutes 4% of Treatment often results in long-term remission. About 85% of patients achieve a complete response from treatment with either cladribine or pentostatin, and another 10% receive some benefit from these drugs, although there is no permanent cure for this disease. If the cancer cells return, the treatment may be repeated and should again result in remission, although the odds of success decline with repeated treatment. [24] Remission lengths vary significantly, from one year to more than twenty years. The median patient can expect a treatment-free interval of about ten years.

It does not seem to matter which drug a patient receives. A patient who is not successfully treated with one of these two drugs has a reduced chance of being successfully treated with the other. However, there are other options.

Second-line therapy: immunotherapy

If a patient is resistant to either cladribine or pentostatin, then second-line therapy is pursued.

Monoclonal antibodies The most common treatment for cladribine-resistant disease is infusing monoclonal antibodies that destroy cancerous B cells. Rituximab is by far the most commonly used. Rituximab, sold under the trade names Rituxan, MabThera and Reditux, is a chimeric Monoclonal antibody against the protein CD20 Most patients receive one IV infusion over several hours each week for four to eight weeks. A 2003 publication found two partial and ten complete responses out of 15 patients with relapsed disease, for a total of 80% responding. [25] The median patient (including non-responders) did not require further treatment for more than three years. This eight-dose study had a higher response rate than a four-dose study at Scripps, which achieved only 25% response rate. [26] Rituximab has successfully induced a complete response in Hairy Cell-Variant. [27]

Rituximab's major side effect is serum sickness, commonly described as an "allergic reaction", which can be severe, especially on the first infusion. Serum sickness is primarily caused by the antibodies clumping during infusion and triggering the complement cascade. The complement system is a Biochemical cascade that helps clear Pathogens from an organism Although most patients find that side effects are adequately controlled by anti-allergy drugs, some severe, and even fatal, reactions have occurred. Consequently, the first dose is always given in a hospital setting, although subsequent infusions may be given in a physician's office. Remissions are usually shorter than with the preferred first-line drugs, but hematologic remissions of several years' duration are not uncommon.

Other B cell-destroying monoclonal antibodies such as Alemtuzumab, Ibritumomab tiuxetan and I-131 Tositumomab may be considered for refractory cases. Alemtuzumab (marketed as Campath, MabCampath or Campath-1H) is a Monoclonal antibody used in the treatment of Chronic lymphocytic leukemia Ibritumomab tiuxetan, also sold under the trade name Zevalin, is a monoclonal antibody Radioimmunotherapy treatment for some forms of B cell Tositumomab is a Monoclonal antibody derived from immortalized mouse cells

Interferon-alpha Interferon-alpha is an immune system hormone that is very helpful to a relatively small number of patients, and somewhat helpful to most patients. Interferons ( IFN s are natural Proteins produced by the cells of the Immune system of most Vertebrates in response to challenges by foreign agents In about 65% of patients,[28] the drug helps stabilize the disease or produce a slow, minor improvement for a partial response. [29]

The typical dosing schedule injects at least 3 million units of Interferon-alpha (not pegylated versions) three times a week, although the original protocol began with six months of daily injections.

Some patients tolerate IFN-alpha very well after the first couple of weeks, while others find that its characteristic flu-like symptoms persist. Perhaps as many as 40% of patients develop a level of depression. In the fields of Psychology and Psychiatry, the terms depression or depressed refer to both expected and pathologically chronic or severe It is possible that, by maintaining a steadier level of the hormone in the body, that daily injections might cause fewer side effects in selected patients. Drinking at least two liters of water each day, while avoiding caffeine and alcohol, can reduce many of the side effects.

A drop in blood counts is usually seen during the first one to two months of treatment. Most patients find that their blood counts get worse for a few weeks immediately after starting treatment, although some patients find their blood counts begin to improve within just two weeks. [30]

It typically takes six months to figure out whether this therapy is useful. Common criteria for treatment success include:

If it is well-tolerated, patients usually take the hormone for 12 to 18 months. An attempt may be made then to end the treatment, but most patients discover that they need to continue taking the drug for it to be successful. These patients often continue taking this drug indefinitely, until either the disease becomes resistant to this hormone, or the body produces an immune system response that limits the drug's ability to function. A few patients are able to achieve a sustained clinical remission after taking this drug for six months to one year. This may be more likely when IFN-alpha has been initiated shortly after another therapy. Interferon-alpha is considered the drug of choice for pregnant women with active HCL, although it carries some risks, such as the potential for decreased blood flow to the placenta.

Interferon-alpha works by sensitizing the hairy cells to the killing effect of the immune system hormone TNF-alpha, whose production it promotes. [31] IFN-alpha works best on classic hairy cells that are not protectively adhered to vitronectin or fibronectin, which suggests that patients who encounter less fibrous tissue in their bone marrow biopsies may be more likely to respond to Interferon-alpha therapy. It also explains why non-adhered hairy cells, such as those in the bloodstream, disappear during IFN-alpha treatment well before reductions are seen in adhered hairy cells, such as those in the bone marrow and spleen. [31]

Experimental therapies

Three immunotoxin drugs are in Phase II trials at the NIH's National Cancer Institute in the U. "NIH" redirects here For other meanings of NIH see NIH (disambiguation. The National Cancer Institute (NCI is part of the United States Federal government's National Institutes of Health. S. : BL22[32], HA22[33] and LMB-2. BL22, also called CAT-3888 or GCR-3888 is an Immunotoxin which attaches to and upon internalization kills B cells It has completed a Phase I clinical [34]

All of these protein-based drugs combine part of an anti-B cell antibody with a bacterial toxin to kill the cells on internalization. BL22 and HA22 attack a common protein called CD22, which is present on hairy cells and healthy B cells. LMB-2 attacks a protein called CD25, which is not present in HCL-variant, so LMB-2 is only useful for patients with HCL-classic or the Japanese variant.

All three of these therapies are available only at the National Cancer Institute in Bethesda, Maryland, USA. While initial results are generally favorable, it is likely to be a number of years before these drugs are available on the market. For the latest on HA22 ( AKA CAT-8015) visit http://www.cancer.gov/search/viewclinicaltrials.aspx?version=healthprofessional&cdrid=562490

Other treatment options

Splenectomy can produce long-term remissions in patients whose spleens seem to be heavily involved, but its success rate is noticeably lower than cladribine or pentostatin. A splenectomy is a procedure that involves the removal of the Spleen by operative means Splenectomies are also performed for patients whose persistently enlarged spleens cause significant discomfort or in patients whose persistently low platelet counts suggest Idiopathic thrombocytopenic purpura. Idiopathic thrombocytopenic purpura (ITP is the condition of having a low Platelet count ( Thrombocytopenia) of no known cause ( Idiopathic)

Bone marrow transplants are usually shunned in this highly treatable disease because of the inherent risks in the procedure. They may be considered for refractory cases in younger, otherwise healthy individuals. "Mini-transplants" are possible.

Patients with anemia or thrombocytopenia may also receive red blood cells and platelets through blood transfusions. Anemia ( AmE) or anæmia/anaemia ( BrE) (from the Ancient Greek grc-Latn anaîmia, meaning “without blood” is defined as a qualitative Thrombocytopenia (or -paenia, or thrombopenia in short is the presence of relatively few Platelets in Blood. Blood transfusion is the process of transferring Blood or blood-based products from one person into the Circulatory system of another Blood transfusions are always irradiated to remove white blood cells and thereby reduce the risk of graft-versus-host disease. Graft-versus-host disease (GVHD is a common complication of allogeneic bone marrow transplantation in which functional immune cells in the transplanted marrow recognize Patients may also receive a hormone to stimulate production of red blood cells. These treatments may be medically necessary, but do not kill the hairy cells. Medical necessity is a United States Legal doctrine, related to activities which may be justified as reasonable necessary and/or appropriate based on evidence-based

Patients with low neutrophil counts may be given filgrastim or a similar hormone to stimulate production of white blood cells. Neutrophil granulocytes, generally referred to as neutrophils, are the most abundant type of White blood cells in humans and form an essential part of the Filgrastim is a Granulocyte colony-stimulating factor (G-CSF analog used to stimulate the proliferation and differentiation of Granulocytes It is produced by However, a 1999 study indicates that routine administration of this expensive injected drug has no practical value for HCL patients after cladribine administration. [35] In this study, patients who received filgrastim were just as likely to experience a high fever and to be admitted to the hospital as those who did not, even though the drug artificially inflated their white blood cell counts. This study leaves open the possibility that filgrastim may still be appropriate for patients who have symptoms of infection, or at times other than shortly after cladribine treatment.

Although hairy cells are technically long-lived, instead of rapidly dividing, some late-stage patients are treated with broad-spectrum chemotherapy agents such as methotrexate that are effective at killing rapidly dividing cells. This is not typically attempted unless all other options have been exhausted and it is typically unsuccessful.

Prognosis

More than 95% of new patients are treated well or at least adequately by cladribine or pentostatin. [3] A majority of new patients can expect a disease-free remission time span of about ten years, or sometimes much longer after taking one of these drugs just once. If re-treatment is necessary in the future, the drugs are normally effective again, although the average length of remission is somewhat shorter in subsequent treatments.

How soon after treatment a patient feels "normal" again depends on several factors, including:

With appropriate treatment, the overall projected lifespan for patients is normal or near-normal. In all patients, the first two years after diagnosis have the highest risk for fatal outcome; generally, surviving five years predicts good control of the disease. After five years' clinical remission, patients with normal blood counts can often qualify for private life insurance with some companies. [4]

Despite decade-long remissions and years of living very normal lives after treatment, hairy cell leukemia is officially considered an incurable disease. Relapses have happened even after more than twenty years of continuous remission. Patients will require lifelong monitoring and should be aware that the disease can recur even after decades of good health.

HCL patients are also at a slightly higher than average risk for developing a second kind of cancer at some point during their lives (including before their HCL diagnosis).

Worldwide, approximately 300 HCL patients per year are expected to die. [5] Some of these patients were diagnosed with HCL due to a serious illness that prevented them from receiving initial treatment in time; many others died after living a normal lifespan and experiencing years of good control of the disease. Perhaps as many as five out of six HCL patients die from some other cause.

Accurately measuring survival for patients with the variant form of the disease (HCL-V) is complicated by the relatively high median age (70 years old) at diagnosis. However, HCL-V patients routinely survive for more than 10 years, and younger patients can likely expect a long life.

People who have hairy cell leukemia are never considered 'cured' and should have regular follow-up examinations after their treatment is over. Most physicians insist on seeing patients at least once a year for the rest of the patient's life, and getting blood counts twice a year. Regular follow-up care ensures that patients are carefully monitored, any changes in health are discussed, and new or recurrent cancer can be detected and treated as soon as possible. Between regularly scheduled appointments, people who have hairy cell leukemia should report any health problems, especially viral or bacterial infections, as soon as they appear.

Patients with HCL are more likely than average to develop another neoplastic disease, such as colon cancer or lung cancer. This appears to relate best to the number of hairy cells, and not to different forms of treatment. [36] On average, patients might reasonably expect to have as much as double the risk of developing another cancer, with a peak about two years after HCL diagnosis and falling steadily after that, assuming that the HCL was successfully treated. Aggressive surveillance and prevention efforts are generally warranted, although the lifetime odds of developing a second cancer after HCL diagnosis are still less than 50%.

Prevention/Screening

Because the cause is unknown, no effective preventive measures can be taken.

Because the disease is rare, routine screening is not cost-effective.

Epidemiology

This disease is rare, with fewer than 1 in 10,000 people being diagnosed with HCL during their lives.

Most patients are white males over the age of 50, although it has been diagnosed in at least one teenager (PMID 11554237). Men are four to five times more likely to develop hairy cell leukemia than women. [37] It does not appear to be hereditary, although occasional familial cases have been reported,[38] usually showing a common HLA type.

References

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  32. ^ Clinical trial NCT00074048
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  34. ^ Clinical trial NCT00337311
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External links

See also

Annexin A1 (or Lipocortin I) is a human Protein encoded by the ANXA1 Gene.
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