Chronic Myeloid Leukemia (Cml)

(Chronic granulocytic leukemia, chronic myelocytic leukemia, chronic myelocytic leukemia)

Chronic myeloid leukemia (CML) is a result of malignant transformation and clonal myeloproliferation a pluripotent stem cell, and characterized by a strong overproduction of immature granulocytes. It is initially asymptomatic and slowly progressive. The first stage is chronic with nonspecific symptoms associated (weakness, loss of appetite, weight loss). The following is a accelerated phase or blast phase with pronounced clinical signs (splenomegaly, paleness, easy bruising and bleeding, fever, lymphadenopathy, and skin lesions). Peripheral blood smears, the bone marrow, the Beck punch and proof of the Philadelphia chromosome confirm the diagnosis. The treatment consists of imatinib (Editor’s note: also dasatinib, nilotinib or bosutinib), which significantly improve the response and survival. However, it is unclear how real healing can be achieved by imatinib. Myelosuppressive drugs (e.g., as hydroxyurea), stem cell transplantation or interferon alpha continue to be used.

CML accounts for about 15% of all leukemias in adulthood. It can occur at any age, although the disease before age 10 is unusual. The median age at diagnosis is between 45 and 55 years. The disease affects both sexes equally.

Chronic myeloid leukemia (CML) is a result of malignant transformation and clonal myeloproliferation a pluripotent stem cell, and characterized by a strong overproduction of immature granulocytes. It is initially asymptomatic and slowly progressive. The first stage is chronic with nonspecific symptoms associated (weakness, loss of appetite, weight loss). The following is a accelerated phase or blast phase with pronounced clinical signs (splenomegaly, paleness, easy bruising and bleeding, fever, lymphadenopathy, and skin lesions). Peripheral blood smears, the bone marrow, the Beck punch and proof of the Philadelphia chromosome confirm the diagnosis. The treatment consists of imatinib (Editor’s note: also dasatinib, nilotinib or bosutinib), which significantly improve the response and survival. However, it is unclear how real healing can be achieved by imatinib. Myelosuppressive drugs (e.g., as hydroxyurea), stem cell transplantation or interferon alpha continue to be used. CML accounts for about 15% of all leukemias in adulthood. It can occur at any age, although the disease before age 10 is unusual. The median age at diagnosis is between 45 and 55 years. The disease affects both sexes equally. Pathophysiology Most CML cases are caused by a translocation to the Philadelphia chromosome (Ph) results and approximately 95% of patients can be detected. This involves a reciprocal translocation t (9; 22), in which a piece of chromosome 9 which contains the c-abl oncogene is translocated to chromosome 22, thereby fused to the BCR gene. This fusion gene BCR-ABL plays a crucial role in the pathogenesis and expression of CML. It leads to the production of a specific tyrosine kinase. CML occurs when an abnormal pluripotent hematopoietic progenitor cell produces large amounts of granulocytes. Initially, only the bone marrow is affected, but later extramedullary manifestations occur (eg. As in the spleen and liver) on. Although the Granulozytenproduktion dominated the neoplastic clone also concerns the erythrocytes, megakaryocytes, monocytes and partly T and B cells. The normal stem cells are suppressed, but may resume its function after drug suppression of the CML clone. The CML runs in 3 phases: Chronic Phase: initial, indolent phase that can last for months or years. Accelerated phase: treatment failure, worsening of anemia, progressive thrombocytopenia or thrombocytosis, sustained or deteriorating splenomegaly, clonal development, increase of basophils in the blood and of blasts in the bone marrow or blood blast phase (blast): accumulation of blasts at extramedullary sites (e.g. B. . bone, CNS, lymph nodes, skin), Blast increase of> 20% in blood or bone marrow the blast phase leads to fulminant complications that are equivalent to those of acute leukemias, including sepsis and bleeding. In some patients, the chronic phase developed directly to the blast phase. Symptoms and signs The onset is often asymptomatic, and there are insidious non-specific symptoms (eg. As fatigue, weakness, loss of appetite and weight loss, fever, night sweats and abdominal bloating) that require clinical investigation. At the beginning pallor, bleeding, easy bruising and lymphadenopathy are rare. Common (in 60-70% of cases) occurs, however a moderate or in some cases even extreme splenomegaly. With advancing disease splenomegaly may increase and it can pallor and bleeding. Fever, lymphadenopathy and clear maculopapular lesions are prognostically unfavorable symptoms. Diagnostic blood count and peripheral blood smear examination of the bone marrow cytogenetic studies (Ph chromosome) The CML is usually diagnosed by chance as a result of a routine blood count or in the clarification of splenomegaly. The number of granulocytes is increased, however, in asymptomatic patients usually <50,000 / ul; in symptomatic patients Granulozytenwerte between 200,000 / ul and 1,000,000 / ul may lie. The platelet count is normal or slightly increased. The hemoglobin level is usually> 10 g / dl. Peripheral blood smear in chronic phase chronic myeloid leukemia with permission of the publisher. From Gorman R., K. Finiewicz In Atlas of Cancer. Edited by M. Markman and M. Kalaycio. Philadelphia, Current Medicine, 2002. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/peripheral_blood_smear_chronic-phase_chronic_myelocytic_leukemia_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual / ? professional / images / peripheral_blood_smear_chronic-phase_chronic_myelocytic_leukemia_high_de.jpg lang = en & thn = 0 ‘, title:’ Peripheral blood smear in chronic phase chronic myelogenous leukemia “, description: ‘ u003Ca id = ” v37895180 “”class = “” anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eWährend the chronic phase of chronic myeloid leukemia of the peripheral blood smear is characterized by some variations

Health Life Media Team

Leave a Reply