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Acute leukemia. Mohammed Al-matrafi . Leukemia. Malignancy of leucocytes precursors Appearance of abnormal cells in BM, peripheral blood, infiltration in LN, Liver spleen etc. Childhood Leukemia. Types: Based on clinical presentation Acute leukemia 95\% Chronic leukemia 5\%

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acute leukemia

Acute leukemia

Mohammed Al-matrafi

leukemia
Leukemia
  • Malignancy of leucocytes precursors
  • Appearance of abnormal cells in BM, peripheral blood, infiltration in LN, Liver spleen etc.
childhood leukemia
Childhood Leukemia

Types:

Based on clinical presentation

  • Acute leukemia 95%
  • Chronic leukemia 5%

Based on type of predominant leukemic cells

  • Acute leukemia:

   Acute lymphoblastic leukemia- 85%

  Acute myeloid leukemia- 15%

childhood leukemia1
Childhood leukemia

Prevalence:

  • Most common malignancy in children
  • 30% of all pediatric malignancies
  • Average incidence 4/100,000 children
  • Peak age:

ALL : 4 years

AML: Same from birth –10 years

etiology
Etiology

Unknown:

Genetic predisposition

Viral infection

Cong. immune deficiency

Ionizing radiation

Certain toxic chemicals

genetics
Genetics
  • At risk:

Trisomy 21 {15 times}

Fanconi aplastic anemia

Ataxia telengectasia

Siblings of patient with ALL {2-3 times}

Identical twins{ concordance of ALL}

clinical presentation of all
Clinical presentation ofALL
  • SYMPTOMS:

Usually < 4 weeks history at diagnosis

Fatigue/malaise

Fever/infection

Extremity, joint or bone pain

Bleeding manifestations

CNS symptoms (Increased ICP)

Weight loss

Others: DIC, Chloroma {AML}

clinical presentation of all1
Clinical Presentation of ALL
  • SIGNS:

Pallor

Hepatomegaly

Spleenomegaly

Lymphadenopathy

Petechie

Bony tenderness

diagnosis
Diagnosis
  • Peripheral blood :

CBC : Normal, increased, decreased

> 100,000 bad prognosis

Anemia

Neutropenia

Blast cells

Thrombocytopenia

slide21
L1

L2

L3

diagnosis1
:Diagnosis
  • Bone marrow (BM)

Morphology:

>25% blast cells in marrow

(normal <5%)

other investigations
Other investigations
  • Uric acid high
  • LDH high
bone marrow aspirate
Bone marrow aspirate
  • Morphological classification:
  • Cytochemical analysis:
  • Immune phenotyping:
  • Cytogenetic:
  • Molecular studies:
morphology
Morphology
  • FAB classification:

{ depending on size,cytoplasm,nucleus}

L1

L2

L3

L1: commonest and has good prognosis

immune phenotype
Immune phenotype
  • T cell leukemia
  • B cell leukemia
  • Non T cell non B cell leukemia
cytogenetic studies
Cytogenetic studies
  • Higher ploidy

{ >50 chromosomes}: good prognosis

  • Diploidy or hyperdiploidy:

{ 47-50 chromosomes} Intermediate prognosis

  • Haploid cell:

worst prognosis

differential diagnosis
Differential diagnosis

Non malignant conditions like:

  • Juvenile Rheumatoid Arthritis / other connective disorders
  • Infectious Mononucleosis
  • Aplastic Anemia
  • Idiopathic Thrombocytopenic Purpura {ITP}
treatment of all
Treatment of ALL

BASED ON:

  • Lineage (B or T)
  • Cytogenetic abnormalities
  • Patient’s age and other risk factors
  • White blood cell count (WBC)
supportive measures
Supportive measures
  • Hydration
  • Treatment of infection
  • Correction of electrolyte disturbances
  • Blood product transfusion
  • Psychological support
  • Treatment of tumor lysis syndrome
treatment of all1
Treatment of ALL

REQUIRES:

  • Intensive systemic multi agent chemotherapy
  • Repetitive intrathecal chemotherapy
  • Cranial irradiation when necessary in older children
  • Bone marrow transplant in special circumstances
  • Treatment continued for  3 years
treatment
Treatment
  • Induction phase: 4 weeks {3-4 drugs}

vincristine, prednisilone, L-asparaginase etc

  • CNS prophylaxis:

Intrathecal methotrexate

Cranial irradiation

  • Consolidation phase:2-4 weeks

{For prevention of relapse}

  • Maintenance phase:{2-5 years}
sanctuaries areas
Sanctuaries areas
  • Relatively impermeable to the medications:
  • Sites of relapse:

2 sites:

CNS

Testis

prognostic factors contd
Prognostic factors {contd.}
  • Morphology, histochemistry, cytogenetic

L1; good prognosis

  • Response to induction therapy

Rapid- good prognosis

Slow- poor prognosis

  • B cell leukemia: worst prognosis
prognostic factors
Prognostic factors
  • Demographic

Age: <2year,>10year poor prognosis

Race: Black poor prognosis

Sex: Male poor prognosis

  • Leukemic burden

WBC: >50,000 poor prognosis

Mediastinal LN: poor prognosis

CNS involvement. at diagnosis: poor prognosis

outcome
Outcome
  • Relapse sites:

Bone marrow

CNS

Testis in males

  • Disease free for 5 years after diagnosis:

overall 60-70%, in standard risk group 80%

  • Relapse: Allogenic bone marrow transplant
bone marrow transplant
Bone marrow transplant
  • Very high risk cases
  • Following relapse
slide39
In a blood stem cell transplant, the patient is first given a pre-transplant treatment of chemotherapy and/or radiation therapy to destroy the patient's leukemia cells and immune system.

Blood stem cells are then put into the patient's blood to restore the patient's immune system and blood production.

acute myelogenous leukemia
Acute myelogenous leukemia
  • FAB classification:

M1,M2,M3,M4,M5,M6,M7

M3 { promyelocytic} may present with DIC

  • Disease free survival with

chemotherapy 30 %

BMT 50-60 %

slide41
Questions
  • Commonest childhood malignancy
  • Types of Ac leukemia
  • Peak age
  • Etiology
  • At risk Patients
  • Symptoms
  • Signs
  • Diagnosis: PBS and bone marrow changes
  • D/D
  • Sanctuary areas
  • Prognostic factors: eg: age <1year,female ,white races ,WBC > 100,000, mediatinal mass, CNS invol

L1 type , Rapid response to induction therapy

  • Relapse site
  • BMT indication
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