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    Acute Lymphoblastic Leukemia

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    15. Alen Jacob
    The document discusses a case of a 7-year-old boy diagnosed with Acute Lymphoblastic Leukemia (ALL), detailing his symptoms, examination findings, and laboratory results that confirmed the diagnosis. It outlines the characteristics, types, etiology, and clinical manifestations of leukemia, emphasizing the prevalence of ALL in children and its genetic predispositions. Additionally, it provides information on prognosis based on various factors such as age, sex, and cytogenetic abnormalities.

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    Acute Lymphoblastic Leukemia

    Uploaded by

    15. Alen Jacob
    1 views19 pages
    The document discusses a case of a 7-year-old boy diagnosed with Acute Lymphoblastic Leukemia (ALL), detailing his symptoms, examination findings, and laboratory results that confirmed the diagnosis. It outlines the characteristics, types, etiology, and clinical manifestations of leukemia, emphasizing the prevalence of ALL in children and its genetic predispositions. Additionally, it provides information on prognosis based on various factors such as age, sex, and cytogenetic abnormalities.

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    The document discusses a case of a 7-year-old boy diagnosed with Acute Lymphoblastic Leukemia (ALL), detailing his symptoms, examination findings, and laboratory results that confirmed the diagnosis. It outlines the characteristics, types, etiology, and clinical manifestations of leukemia, emphasizing the prevalence of ALL in children and its genetic predispositions. Additionally, it provides information on prognosis based on various factors such as age, sex, and cytogenetic abnormalities.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    1 views19 pages

    Acute Lymphoblastic Leukemia

    Uploaded by

    15. Alen Jacob
    The document discusses a case of a 7-year-old boy diagnosed with Acute Lymphoblastic Leukemia (ALL), detailing his symptoms, examination findings, and laboratory results that confirmed the diagnosis. It outlines the characteristics, types, etiology, and clinical manifestations of leukemia, emphasizing the prevalence of ALL in children and its genetic predispositions. Additionally, it provides information on prognosis based on various factors such as age, sex, and cytogenetic abnormalities.

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    ACUTE

    LYMPHOBLASTIC
    LEUKEMIA
    • Jibin ,7year old male.
    • when he was 5 years of age ,he presented with complaints of loss of
    appetite for 3 weeks,fever for 1 week right side otalgia for 4 days and
    bilateral lid sweeking for 4 days
    • On examination,he was dull lookinG,pallor present,bilateral cervical
    lymphadenopathy bilateral eye lid swelling.
    • Liver palpable 4 cm below right coastal margin and spleen palpable 3
    cm below left coastal margin both testis were normal
    • Investigations
    • Hematology:HB-2.7g/dl,TC-2.05L/mm3,DLC-N9L27 Abnormal cells-
    64%,PLC-2.01L/mm3
    • Biochemistry-s.LDH-548U/L
    • Imageology-no mediastinal swelling
    • Peripheral smear -45% blasts,only 1-2% myeloperoxidase positive
    • Bone marrow aspiration:B-ALL,78% BLASTS
    • CYTOGENETICS:BCR-ABL FUSION POSITIVE
    • CSF:smear shows occasional large cell having scanty cytoplasm with
    coarse chromatin and few lymphocytes and monocytes(cns 3)
    • STARTED ON MODIFIED BFM ALL PROTOCOL
    • End of induction phase-MRD POSITIVE,end of consolidation phase-
    MRD -negative
    • He Is Currently On Maintainence Phase On Drugs
    T.DASATINIB,T.6MERCAPTOPURINE,T.METHOTREXATE
    Leukemia
    • Most common malignant neoplasm in childhood(31%)
    • It’s a group of malignant diseases in which genetic abnormalities in a
    hematopoietic cells give rise to an unregulated clonal proliferation of
    cells
    • These cells have increased rate of proliferation and decreased rate of
    spontaneous apoptosis
    • It results in disruption of normal marrow function and results in
    marrow failure
    TYPES
    • Acute Lymphoblastic leukemia (77%)
    • Acute myelogenous leukemia (11%)
    • Chronic lymphoblastic leukeMIa (2-3%)
    • Juvenile myelomonocytic leukemia (1-2%)
    LYMPHOBLASTS MYELOBLASTS
    SIZE 2-3 TIMES SIZE OF LYMPHOCYTE 3-5 TIMES THE SIZE OF
    LYMPHOCYTE
    CYTOPLASM SCANTY,BLUE,AGRANULAR SCANTY TO MODERATE,GRAY WITH
    CYTOPLASMIC GRANULES AND
    AUER RODS
    NUCLEAR CHROMATIN UNIFORM COARSE CHROMATIN UNIFORM FINE CHROMATIN
    NUCLEOLI INCONSPICUOUS NUCLEOLI 3-5 PROMINENT NUCLEOLI
    CYTOCHEMISTRY PAS-BLOCKS OF POSITIVITY MPO AND SUDAN BLACK POSITIVE
    ACCOMPANYING CELLS LYMPHOCYTES PROMYELOCYTES,MYELOCYTES ,ME
    TMYELOCYTES,BAND FORMS
    ACUTE LYMPHOBLASTIC
    LEUKEMIA
    • Most Common Childhood Malignancy
    • More common boys than in girls
    • More common in children with certain chromosomal abnormalities
    such as Down Syndrome, Li-Fraumenni, Bloom syndrome, Ataxia
    Telengectasia
    Etiology
    • They are thought to be caused by post conception somatic pathogenic
    gene variants in lymphoid cells
    • In some cases in utero events in the initiation of malignant process
    are evidenced by identification of specific fusion gene sequences in
    archived neonatal blood spots
    • Additional genetic modifications are required for disease expression
    as there is along lag period
    • Exposure to medical diagnostic radiation
    • EBV infections
    PREDISPOSING FACTORS
    GENETIC SUSCEPTIBILITY
    • Congenital syndromes-Down syndrome,Fanconi anemia,Ataxia
    Telengectasia,bloom Syndrome
    • Inherited gene variants-ARID5B,IKZF1,CEBPE,CDKN2A
    • Constituitional Robertsonian Translocation Between Chromosomes 15
    AND 21
    • Single Gene Polymorphisms:rs12402181 In miR-3117,rs6255571442 in
    miR3689d2

    Environmental factors -pesticide exposure,ionizing


    radiations,childhood infections,alkylating agents
    CLASSIFICATION
    • MORHOLOGY
    • PHENOTYPE CLASSIFICATION
    • CYTOGENETIC and MOLECULAR GENETIC
    FAB classification
    CYTOLOGIC FEATURES L1(80-85%) L2(15%) L3(1-2%)
    CELL SIZE SMALL CELLS LARGE HETEROGENOUS LERGE,HOMOGENOUS
    PREDOMIBNATE,HOMOGEN
    OS
    AMOUNT OF CYTOPLASM SCANTY MODERATELY ABUNDANT MODERATELY ABUNDANT
    NUCLEOLI SMALL INCONSPICUOUS ONE OR MORE OFTEN ONE OR MORE PROMINENT
    LARGE
    NUCLEAR CHROMATIN HOMOGENOUS VARIABLE HETEROGENOUS STIPPLED HOMOGENOUS
    NUCLEAR SHAPE REGULAR OCCASSIONAL IRREGULAR CELLS REGULAR OVAL TO ROUND
    CELLS INDENTATION
    CYTOPLASMIC BASOPHILIA VARIABLE VARIABLE INTENSELY BASOPHILIC
    CYTOPLASMI VARIABLE VARIABLE PROMINENT
    VACUOLATION
    WHO classification
    • B-lymphoblastic leukemia/lymphoma,NOS
    • B-lymphoblastic leukemia/lymphoma,WITH HIGH HYPERPLOIDY
    • B-lymphoblastic leukemia/lymphoma,WITH HYPOPLOIDY
    • B-lymphoblastic leukemia/lymphoma,WITH iAMP21
    • B-lymphoblastic leukemia/lymphoma,WITH BCR::ABL1 fusion
    • B-lymphoblastic leukemia/lymphoma,WITH BCR::ABL1 fusion LIKE FEATURES
    • B-lymphoblastic leukemia/lymphoma,WITH KMT2A rearrangement
    • B-lymphoblastic leukemia/lymphoma ,WITH EVT6::RUTNX1 FUSION
    • B-lymphoblastic leukemia/lymphoma WITH TCF3::PBX1 FUSION
    • B-lymphoblastic leukemia/lymphoma,WITH IGH::IL3 FUSION
    • B-lymphoblastic leukemia/lymphoma,WITH OTHER DEFINED GENETIC ABNORMALITIES
    Phenotype classification
    • B-lymphoblastic ALL(85%)
    • T-lymphoblastic ALL(15%)
    • Burkitt leukemia-derived from mature B cells
    Molecular genetic
    Clinical manifestations
    • Anorexia,fatigue,malaise,irritability
    • Intermittent low grade fever
    • Severe Bone or joint pain in extremeties
    • As the disease progresses,pallor,exercise intolerance,oral or mucosal
    bleeding,epistaxis
    • Organ infiltration-lymphadenopathy,hepatosplenomegaly,testicular
    enlargement,CNS involvement (cranioneuropathies, headache,
    seizures)
    • PHYSICAL EXAMINATION-pallor,listlessness,purpuric and petechial skin
    lesions,mucous membrane hemorrhage
    • Lymphadenopathy,splenomegaly,hepatomegaly
    • Bonetenderness,swelling,deep bone pain
    • Signs of raised ICP-papilledema,retinal hemorrhage,cranial nerve palsies
    • Respiratory distress-anemia/large anterior mediastinal mass(tcell>bcell)
    • Superior Vena Caval syndrome-facial edema and plethora,throbbing
    headache,dilated neck veins
    B lymphoblastic leukemia
    • Most common immunophenotype
    • 1-10 years
    • Median leukocyte count->33000
    • Hepatosplenomegaly
    • CNS involvement-5%,CNS leukemia if>5WBC/Ul,blasts present
    • Testicular involvement
    PROGNOSIS
    UNFAVOURABLE PROGNOSIS FAVOURABLE PROGNOSIS

    AGE LESS THAN 2 AND MORE THAN 10 2-10

    SEX Males FEMALES

    Total WBCcount High(>50000 cells/mm3) LOW

    Blasts count in peripheral blood Greater than 1 lakh <100000cells/mm3

    Meningeal involvement Present Absent

    Cytogenetic abnormalities T(9;22) Hyperploidy and trisomy of


    chromosomes 4,7,10 and t(12:21)
    time required for clearing blasts >1weektime required for clearing ,<1 week
    from blood blasts from blood

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