Chronic myelogenous leukemia

Chronic myelogenous leukemia (CML) 

Updated: 04/20/2021

© Jun Wang, MD, PhD

General features
  • Most common chronic myeloproliferative neoplasm
  • Clonal proliferation of pluripotent granular stem cells
  • All ages affected, median age 50-60
Clinical presentations
  • Insidious onset with anemia, fatigue, weight loss
  • Extramedullary hematopoiesis causing massive hepatosplenomegaly and lymphadenopathy
Clinical course
  • Chronic phase: Usually for 3-4 years, < 10% blasts in the bone marrow, readily controlled with oral medications
  • Accelerated phase: 10-19% blasts in blood or marrow, >20% basophils in blood, persistent platelet count <100K or >1000K, increasing splenomegaly or white blood cell count while on therapy, marked myelofibrosis, additional cytogenetics abnormalities, lack of response to treatment
  • Blast transformation (phase): > 20% or more blasts in marrow or blood, often death shortly
Key pathogenesis
  • Translocation resulted abnormal activation of ABL: non-receptor tyrosine kinase in nuclei, regulating cell division, adhesion, differentiation
Key Laboratory findings
  • Leukocytosis with left shift
  • Basophilia
  • Monocytosis, usually <3%
  • Low leukocyte alkaline phosphatase (LAP) score
  • Thrombocytosis
Key morphological features
Differential diagnosis
  • Acute myeloid leukemia (AML): Blasts > 20% or cytogenetic findings
  • Chronic myelomonocytic leukemia: High monocytic count
  • Chronic neutrophilic leukemia
    • Sustained proliferation of mature neutrophils
    • May be asympatomatic
    • >80% combined segmented neutrophils or bands, <10% immature granulocytes and <1% myeloblasts in peripheral blood or <5% in marrow
    • Normal morphology of monocytic and megakaryocytic series
    • CSF3R mutation in 80% pts
    • NO PDGFRA, PDGFRB, FGFR1 nor BCR-ABL
  • Thrombocythemia: No or little granulocyte or erythroid proliferation
  • Leukemoid reactions: Toxic granulation, a HIGH LAP score, an obvious cause for the neutrophilia

Genetic abnormalities
  • t(9;22) (q34;q11), Philadelphia chromosome: BCR-ABL


Back to myeloproliferative neoplasms
Back to myeloid neoplasms
Back to contents

Comments

Post a Comment

Popular posts from this blog

Contents

Pathology notes Jun Wang, MD, PhD 1. General information Frequently discussed syndromes Terms of dermatology Terms of hematology 2. Primary immunodeficiency disorders 3. Hemodynamic abnormalities Shock Thrombosis and embolism Bleeding disorders 4. Benign white cell disorders 5. Hematopoietic neoplasms Lymphoid neoplasms Myeloid neoplasms Histiocytic neoplasms: Langerhans cell histiocytosis 6. Cardiovascular system Pulmonary hypertension Cardiovascular system tumors 7. Respiratory tract tumors 8. Gastrointestinal tract Congenital disorders Infectious gastroenteritis Oral cavity Esophagus Stomach Intestinal tumors Salivary glands Exocrine pancreas and gallbladder 9. Urinary system Kidney masses Lower urinary tract 10. Male reproductive system 11. Female genital tract 12. Breast 13. Endocrine organs Pituitary gland Adrenal gland Thyroid gland Parathyroid gland Endocrine pancreas Multiple endocrine neoplasms 14. B
Read more

Female genital tract

Pathology of female genital tract   Updated: 01/23/2024 © Jun Wang, MD, PhD Key anatomic features Opened channel, pathological basis for pelvic inflammatory disease Cervix: exocervix, endocervix, transformation zone Uterus: fundus, body Fallopian tube: intramural, isthmus, ampulla, infundibulum, fimbriae Placenta: umbilical cord, fetal membrane, placenta Key histologic features Stratified squamous epithelium from vulva to exocervix Simple columnar epithelium from endocervix to fimbriae Uterine corpus Endometrium: Glands, stroma Myometrium Perimetrium: Mesothelium/peritoneum Ovaries: three components Epithelium (mesothelium) Stroma Germ cells (follicles) Placenta: Cytotrophoblast, syncytiotrophoblast, intermediate trophoblast Infectious/inflammatory diseases Diseases of vagina and vulva Molluscum contagiosum Lichen sclerosus Paget disease Sarcoma botryoides Squamous cell carcinoma Diseases of cervix Cervical intraepithelial neopla
Read more

Neoplasms of respiratory tract

Neoplasms of respiratory tract   Updated: 11/14/2022 © Jun Wang, MD, PhD Anatomy Nasal cavity Nasopharynx Oropharynx Laryngopharynx Larynx Trachea and bronchi Lungs Histology Squamous mucosa Respiratory mucosa May have abundant lymphoid tissue in pharynx Type I and Type II pneumocytes Key risk factors Human papillomavirus: Squamous cell carcinoma, squamous cell papilloma Epstein Barr virus: Nasopharyngeal carcinoma Smoking Tumors of nasal cavity and nasopharynx Olfactory neuroblastoma Nasopharyngeal angiofibroma Sinonasal papilloma NUT midline carcinoma Nasopharyngeal carcinoma Tumors or tumor like lesions of larynx Vocal cord polyp Squamous papilloma Squamous cell carcinoma Branchial pouch/cleft anomalies Branchial cyst Thyroglossal duct cyst Neoplasms of lung Squamous cell carcinoma Adenocarcinoma Large cell carcinoma Small cell carcinoma Carcinoid Lymphangioleiomyomatosis Neoplasms of pleura Mesothelioma
Read more