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Theml, Color Atlas of Hematology - part 7

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Tuổi khởi phát: Bất cứ tuổi nào. Inicidence cao điểm khoảng 50 năm. Kết quả lâm sàng: Dần dần phát triển mệt mỏi, thiếu máu, trong một số trường hợp rõ rệt splenomegaly; không bị sốt. CBC: tăng bạch cầu và một sự chuyển dịch còn lại trong các dòng bạch cầu hạt, có thể Hb, thrombocytes. | 116 Abnormalities of the White Cell Series Characteristics of CML Age of onset Any age. Peak inicidence about 50 years. Clinical findings Slowly developing fatigue anemia in some cases palpable splenomegaly no fever. CBC Leukocytosis and a left shift in the granulocyte series possibly Hb ị thrombocytes ị or ị . Advanced diagnostics Bone marrow cytogenetics and molecular genetics Philadelphia chromosome and BCR-ABL rearrangement . Differential diagnosis Reactive leukocytoses alkaline phosphatase trigger other myeloproliferative disorders bone marrow cytogenetics alkaline phosphatase . Course therapy Chronic progression. Acute transformation after years. New curative drugs are currently under development. Evaluate the possibility of a bone marrow transplant up to age approx. 60 years . Steps in the Diagnosis of Chronic Myeloid Leukemia Left-shift leukocytosis in conjunction with usually low-grade anemia thrombocytopenia or thrombocytosis which often correlates with the migration of small megakaryocyte nuclei into the blood stream and clinical splenomegaly is typical of CML. LDH and uric acid concentrations are elevated as a result of the increased cell turnover. The average typical cell composition is as follows in a series analyzed by Spiers about 2 myeloblasts 3 promyelocytes 24 myelocytes 8 metamyelocytes 57 band and segmented neutrophilic granulocytes 3 basophils 2 eosinophils 3 lymphocytes and 1 monocytes. In almost all cases of CML the hematopoietic cells display a marker chromosome an anomalously configured chromosome 22 Philadelphia chromosome . The translocation responsible for the Philadelphia chromosome corresponds to a special fusion gene BCR-ABL that can be determined by polymerase chain reaction PCR and fluorescence in situ hybridization FISH . Theml Color Atlas of Hematology 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. Left shift as far as myeloblasts proliferation of eosinophils and basophils suggest chronic .