Leukemia-II (Chronic Leukemia) PDF

Leukemia-II 02/01/2025 1 Chronic Leukemia 02/01/2025 2  Intended learning objectives  Describe types of chronic leukemia's.  Describe chronic lymphocytic leukemia.  Describe chronic myelocytic leukemia.  Describe Philadelphia chromosome. ...

Leukemia-II 02/01/2025 1 Chronic Leukemia 02/01/2025 2  Intended learning objectives  Describe types of chronic leukemia's.  Describe chronic lymphocytic leukemia.  Describe chronic myelocytic leukemia.  Describe Philadelphia chromosome.  Chronic Leukemia Chronic lymphocytic leukemia (CLL). Chronic myelocytic leukemia (CML). Chronic Lymphocytic Leukemia (CLL) 02/01/2025 5  Definition Monoclonal disorder characterized by a progressive accumulation of functionally incompetent mature B lymphocytes.  The cells accumulate in the bone marrow, blood, liver, spleen and lymph nodes. 02/01/2025 6  Epidemiology  The median age at diagnosis is 60 years.  2:1 male predominance. Clinical features  Generalized lymphadenopathy: enlargement of cervical, axillary or inguinal lymph nodes  Hepato-splenomegaly occurs in 50-60% of cases.  Features of anemia may be present.  Bruising or purpura may occur due to thrombocytopenia.  Hypogammaglobinemia leads to increased risk of bacterial infections. 02/01/2025 7 CERVICAL LYMPHADENOPATHY  Criteria for diagnosis (National Comprehensive Cancer Network (NCCN) 1. Peripheral blood lymphocyte count of ≥5000/μl. ( Normal lymphocyte count- 1000–3000/μl) 2. Small, mature lymphocytes with high N/C, round to oval nuclei and clumped chromatin. 3. Clonality confirmed by flow cytometry. 4. Immunophenotyping- cells express CD19, CD20, CD23.  Laboratory findings 1. Peripheral blood:  WBCs: ↑↑↑ >100.000, predominantly lymphocytes.  RBCs: anaemia  Platelets: thrombocytopenia. 2. Bone marrow: shows mature B lymphocytic replacement of normal marrow elements. 3. Immunophenotyping: cells express CD19, CD20, CD23, CD5. 02/01/2025 10  Characteristic peripheral smear  Small mature B lymphocytosis with ‘’smudge cells’’.  Prognosis Depends on the stage of the disease.  Two staging systems are used.  1. Binet staging system  2. Rai-Sawitsky staging system. Chronic Myeloid leukemia (CML) 02/01/2025 13  Definition Neoplastic proliferation of the granulocytic cell line without the loss of their capacity to differentiate. Characterized by the presence of Philadelphia chromosome. 02/01/2025 14  Epidemiology  CML is primarily a disease of adults but also occurs in children and adolescents.  The peak incidence is in the fifth to sixth decades of life.  Stages of CML Three stages  Chronic stage.  Accelerated stage.  Acute blastic crisis. Chronic stage 85% patients present in chronic stage.  Chronic stage  85% patients present in chronic stage.  Features of anemia may include pallor, dyspnea and tachycardia.  Low-grade fever, Fatigue, weight loss, loss of energy and night sweats.  Abdominal fullness due to splenomegaly (markedly enlarged)  Left upper quadrant abdominal pain from spleen infarction  Hepatomegaly.  Easy bruisability and spontaneous bleeding.  Gout or renal impairment may be seen because of hyperuricemia (elevated levels of uric acid) due to high cell turnover.  Laboratory findings 1. Peripheral blood:  WBCs: markedly raised. >100,000/dl.  Basophilia (important finding) and eosinophilia.  RBCs: anaemia  Platelets: thrombocytosis. 02/01/2025 18  Peripheral smear in CML  Manymature neutrophils, few metamyelocytes, and a myelocyte 2. Bone marrow:  Hypercellular with markedly increased myeloid series of cells.  Increased basophils, eosinophils and megakaryocytes. 3. Cytogenetic analysis. For detection of Philadelphia chromosome. Philadelphia chromosome Results form reciprocal translocation between chromosome 9 and 22 i.e t(9,22) (q34;q11). Ph. chromosome refers to shortened chromosome 22.  Prognosis The natural history is one of slow progression; even without treatment, the median survival is about 3 years.  REFERENCES  Robbins and Cotran Pathologic Basis of Disease, 9th edition, 2014 ( Kumar, Abbas, Aster)  Robbins Basic Pathology 10th edition, 2017 ( Kumar, Abbas, Aster) AMBOSS https://next.amboss.com/us/article/jT0_q2? q=CHRONIC%20leukemia#0N0e-T  Case 1  A 72-year-old man comes to the physician because of fatigue and a 5-kg (11-lb) weight loss over the past 6 months despite a good appetite. He takes no medications. He does not smoke or use illicit drugs. Physical examination shows hepatosplenomegaly and diffuse, nontender lymphadenopathy. Laboratory studies show a hemoglobin concentration of 11 g/dL and a leukocyte count of 16,000/mm3. A photomicrograph of a peripheral blood smear is shown. Which of the following is the most likely diagnosis? A. Chronic myelogenous leukemia B. Acute myelogenous leukemia C. Hodgkin lymphoma D. Chronic lymphocytic leukemia  Case 2 A 64-year-old woman comes to the physician because of a 7-month history of abdominal discomfort, fatigue, and a 6.8-kg (15- lb) weight loss. Physical examination shows generalized pallor and splenomegaly. Laboratory studies show anemia with pronounced leukocytosis and thrombocytosis. Cytogenetic analysis shows a BCR-ABL fusion gene. Which of the following is the most likely diagnosis? A. Acute lymphoblastic leukemia B. Chronic lymphocytic leukemia C. Chronic myeloid leukemia D. Acute promyelocytic leukemia THANK YOU