Keywords: hairy cell leukaemia, incidence, antigen, biological advances, new drugs, clinical phase. Hairy cell leukaemia hcl is chronic b cell lymphoid melanoma with typical immunophenotype having cd19, cd20, and cd22, cd11c, cd25, and cd103 immunoglobulin. First complete description of hairy cell leukaemia was initiated in the year 1958 by bouroncle et.tal. A hairy cell variant hcl v account for 10% 20% of the hairy cell leukaemia.it was first acknowledged by cawley et.at and later was illustrated as promphocytic leukaemia.

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Hairy cell leukaemia hcl is considered as heterogeneous diseases according to study conducted by graca m. Tnf alpha is produced by hairy cell which in turn suppressed the normal production of healthy blood cell by the bone marrow. Hairy cell leukemia hcl is a chronic lymphoid leukemia, originally described in 1958 by bouroncle and colleagues 1, 2 and named after the hairlike cytoplasmic projections seen on the surface of the abnormal b cells see the image below. Woermann, md, division of instructional media, institute for medical education, university of bern, switzerland. A critical images slideshow, to help detect chronic leukemias and determine the specific type present.

The abnormal cell in hairy cell leukemia is a clonal b cell lymphocyte see image below. This cell infiltrates the patient's reticuloendothelial system and interferes with bone marrow function, resulting in bone marrow failure or pancytopenia. 3, 4 the hairy cell also infiltrates the liver and spleen, resulting in organomegaly. Recently, new dna scanning techniques using whole exome sequencing identified 5 missense somatic clonal mutations, including a heterozygous mutation in braf that results in the braf v600e variant protein, which is oncogenic for other tumors as well, was identified in all hairy cell leukemia patients and may be responsible for the pathogenesis, diagnosis, and targeted therapy of hairy cell leukemia. 5 the etiology of hairy cell leukemia has not been determined, although some investigators suggest that exposures to benzene, organophosphorus insecticides, or other solvents may be related to disease development. This hypothesis has not been confirmed by other reports, although a french study that evaluated occupational exposure to pesticides and lymphoid neoplasms among men appears to support the hypothesis that occupational pesticide exposures may not only be involved in hairy cell leukemia, hodgkin lymphoma, and multiple myeloma, but also may play a role in non hodgkin lymphoma.

Exposure to radiation, agricultural chemicals, and wood dust, and a previous history of infectious mononucleosis have been suggested as etiologic associations in previous reports. Overexpression of cyclin d1 protein, an important cell cycle regulator, has been observed in hairy cell leukemia and may play a role in the molecular pathogenesis of the disease. Accumulation of hairy cells in the bone marrow, liver, and spleen, with very little lymph node involvement, is characteristic of hairy cell leukemia. This pattern probably results from the expression of the integrin receptor alpha4 beta1 by the hairy cells and the interaction of the receptor with the vascular adhesion molecule 1 vcam 1 found in splenic and hepatic endothelia, bone marrow, and splenic stroma. G schmidt wolf 1 and i g h schmidt wolf 1

1 center for integrated oncology cio , bonn, germany 2 kantonsspital graub x0fc nden, chur, switzerland correspondence: professor dr igh schmidt wolf, center for integrated oncology cio , university of bonn, sigmund freud stra x0df e 25, bonn 53105, germany.

E mail: picasso@uni bonn.de received 12 december 2013 accepted 23 december 2013 data for this review were identified by searches of medline, pubmed and references from relevant articles using the search terms x02018 hairy cell leukemia x02019 hcl. Representing approximately 2 x025 of all leukemias, hcl is a chronic b cell lymphoproliferative disorder. 1 in the following, historical and recent treatment options, their results as well as new insights in the pathophysiology and treatment recommendations are reviewed.

splenic irradiation and splenectomy

splenomegaly is one of the most common clinical manifestations of hcl up to 96 x025 of the patients , 2 often getting symptomatic in the form of epigastric discomfort or pain. 3 splenectomy was developed as the first effective therapeutic approach in the history of the treatment of hcl and was considered as treatment of choice until 1984, 4. 5 resulting in overall response rates orr of 60 x02013 100 x025 as documented in eight major reports.

8 patients with hcl benefit from splenectomy compared with non splenectomized patients 5 and respond better and faster to the following therapy. 9 it is recommended to wait at least 6 months after splenectomy before initiating further treatment approaches to reach full benefits of splenectomy. 10 nevertheless, splenectomy does not improve bone marrow infiltration and fibrosis, 5 and positive influences of splenectomy with regard to long term results are reported to be uncertain. 10 despite the fact that more effective chemotherapeutics are available in the treatment of hcl today, splenectomy still remains a valid treatment option in certain cases. X03e 10 thinsp cm below costal margin but low bone marrow involvement can be an indication for splenectomy as first line treatment.

10 in cases of unknown splenomegaly, splenectomy is not only a therapeutic but also a helpful diagnostic instrument to prove the typical transformation of spleen histology. 11 splenectomy can also be an alternative intervention for patients with relapsed or refractory disease. 2 owing to the fact that hcl is less common in women at childbearing age and thus rare in pregnancy, there are no standardized therapy regimens in this configuration. Then, laparoscopic splenectomy should be considered as a therapeutic option to protect the fetus from toxic effects of chemotherapy, although there are only few data available with regard to the use of chemotherapy during pregnancy. 8 although splenic irradiation is enumerated together with androgens, lithium, corticosteroids, chlorambucil or cyclophosphamide as unsatisfying treatment approaches before the introduction of interferon x003b1 ifn x003b1 , 14 lavrenkov et al. 3 reported the successful use of palliative splenic irradiation to reduce splenic size and to improve anemia and thrombocytopenia in two patients with symptomatic splenomegaly in hcl. In conclusion, splenectomy has clearly lost its therapeutic importance after the introduction of ifn x003b1 and later on of purine analogs pa. Nevertheless, it should still be remembered as an alternative or additional option, especially in symptomatic or refractory x02f relapsed disease.

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ifn x003b1

initially described as an anti viral agent in 1957, it took almost 30 years until the approval of ifn x003b1 as the first recombinant cytokine for the treatment of malignancies. 15 quesada et al. 16 showed that ifn x003b1 can improve and is even able to normalize peripheral blood counts in patients with hcl. 5 the main therapeutical effects on patients with hcl are not only the reduction of cytopenia and the elimination of hc from the blood but also the reduction of bone marrow fibrosis. 17

purine analogs

pentostatin 2 x02032 deoxycoformycin, dcf

although early approaches were carried out in refractory cll, 18 pentostatin, a product of streptomyces antibioticus and a pharmacologic inhibitor of adenosine deaminase ada , 1. 19 it is not only well tolerated but also leads to durable complete remission rates of x03e 75 x025.

18 with the establishment of pa in the therapy of hcl, prognosis was improved significantly. 20 the irreversible inhibition of ada 9 causes an intracellular accumulation of cytotoxic deoxyadenosine triphosphate metabolites inter alia in hc, 21 leading to the inhibition of the repair system of spontaneously formed dna breaks. 22 consequences are the activation of p53 and the release of cytochrome c from mitochondria, initiating apoptosis.