JSH Practical Guidelines for Hematological Malignancies, 2018: I. Leukemia-4. Chronic myeloid leukemia (CML)/myeloprolif
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GUIDELINE
JSH Practical Guidelines for Hematological Malignancies, 2018: I. Leukemia‑4. Chronic myeloid leukemia (CML)/myeloproliferative neoplasms (MPN) Kazuya Shimoda1 · Naoto Takahashi2 · Keita Kirito3 · Noriyoshi Iriyama4 · Tatsuya Kawaguchi5 · Masahiro Kizaki6 Received: 13 July 2020 / Accepted: 13 July 2020 © Japanese Society of Hematology 2020
Overview
Chronic myeloid leukemia (CML)
Myeloproliferative neoplasms (MPN), a group of diseases that develop through neoplastic transformation at the level of hematopoietic stem cells, are characterized by marked proliferation of myeloid cells (i.e., granulocytes, erythroblasts, and megakaryocytes). 1 The category of MPN includes chronic myeloid leukemia (CML), chronic neutrophilic leukemia (CNL), polycythemia vera (PV), primary myelofibrosis (PMF), essential thrombocythemia (ET), chronic eosinophilic leukemia (CEL), and MPN, unclassifiable. Early-stage MPN exhibit hyperplasia of bone marrow cells with capacity for differentiation and increased peripheral granulocytes, red blood cells (RBCs), and platelets. Physical findings include splenomegaly and hepatomegaly. MPN produce few subjective symptoms in their early stage, but progress in stages along with general symptoms. They ultimately progress to myelofibrosis or loss of maturation potential through transformation (blast crisis). A different treatment approach is used for CML from those for other types of MPN. These guidelines cover treatments for CML, PV, ET, and PMF.
Staging of CML
* Kazuya Shimoda [email protected]‑u.ac.jp
1
Department of Gastroenterology and Hematology, University of Miyazaki, Miyazaki, Japan
Naoto Takahashi [email protected]‑u.ac.jp
2
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Akita, Japan
Keita Kirito [email protected]
3
Department of Hematology and Oncology, University of Yamanashi, Yamanashi, Japan
Noriyoshi Iriyama iriyama.noriyoshi@nihon‑u.ac.jp
4
Tatsuya Kawaguchi tatsu@kumamoto‑u.ac.jp
Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan
5
Masahiro Kizaki makizaki@saitama‑med.ac.jp
Department of Medical Technology, Kumamoto Health Science University, Kumamoto, Japan
6
Department of Hematology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
Chronic myeloid leukemia (CML) is a type of leukemia that arises from abnormalities in pluripotent hematopoietic stem cells and is characterized by the presence of the Philadelphia (Ph) chromosome formed by the t(9;22) (q34;q11) translocation. This translocation results in the constitutive activation of BCR–ABL1 tyrosine kinase encoded and produced by the BCR–ABL1 fusion gene on the Ph chromosome. This contributes to the proliferation of leukemic cells and initiates the progression of the disease through three stages.1 Most cases of CML (85%) are diagnosed during the chronic phase (CP; approximately 3 to 5 years after diagnosis), in which patients have eleva
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