Case PresentationCase Presentation

Patient’s HistoryPatient’s History A. Y. female patient 4 years old A. Y. female patient 4 years old

is living in Cairois living in Cairo Presented outside NCI by Presented outside NCI by Persistent feverPersistent fever PallorPallor Outside NCI CBC: 55% blastsOutside NCI CBC: 55% blasts

ClinicallyClinically Fair general conditionFair general condition PallorPallor Symptoms of increased ICTSymptoms of increased ICT No other neurological deficit No other neurological deficit No organomegaly or No organomegaly or

lymphadinopathylymphadinopathy Chest, heart, & abdominal Chest, heart, & abdominal

examination: free examination: free

InvestigationsInvestigations CBCCBC: TLC 7500, ANC 500, Hb 6, Plt : TLC 7500, ANC 500, Hb 6, Plt

6500065000 BM aspirationBM aspiration: AML M3 : AML M3 IPTIPT: myeloid CD 33, 13 MPO +ve: myeloid CD 33, 13 MPO +ve CytogeneticsCytogenetics: 46 XX t 15-17: 46 XX t 15-17 CSFCSF: ++ve: ++ve Brain MRIBrain MRI: normal: normal Chest x-ray & abdominal USChest x-ray & abdominal US: free: free

TreatmentTreatment InductionInduction:: ADR 45 mg/ m2 (d 1, 2, & 3)ADR 45 mg/ m2 (d 1, 2, & 3) ATRA 45 mg/ m2 till remissionATRA 45 mg/ m2 till remission Intra thecal weeklyIntra thecal weekly Consolidation:Consolidation: 2 courses of ADR 45 mg/ m2 (d 2 courses of ADR 45 mg/ m2 (d

1, 2, & 3)1, 2, & 3)

She was planned to receive She was planned to receive craniospinal craniospinal radiotherapyradiotherapy

& to be put on & to be put on maintenance maintenance chemotherapychemotherapy

MTX 20 mg /m2 weeklyMTX 20 mg /m2 weekly 6 MP 75 mg / m2 6 MP 75 mg / m2 ATRA 45 mg /m2 for 2 weeks ATRA 45 mg /m2 for 2 weeks

every 12 weeksevery 12 weeks Radiotherapy was postponed Radiotherapy was postponed

due to poor chest conditiondue to poor chest condition

Chest condition

•Fever •Respiratory distress Gr III•Neutropenia•Chest x-ray: pleural effusion

Malignant effusionATRA SyndromeChest Infection

Stopped chemotherapyPlural effusion cytology:

-ve

Chest Infection•Broad spectrum antibiotic•Follow up chest x-ray: encysted pleural effusion•CT chest: pleural based mass with central breaking down•Sputum C&S: Many cndidal colonies•Amphotercin B was added

•Follow up chest CT: no appreciable changes•Another sputum C&S: gram +ve cocci only sensitive to vancomycin•Vancomycin was added for 2 weeks•Follow up chest CT: marked improvement

Re evaluation after 2 weeks

After improvement of the After improvement of the chestchest

condition, the patient condition, the patient received received

Craniospinal irradiationCraniospinal irradiation Continued maintenance Continued maintenance

chemotherapychemotherapy

Seven months later, the patient had Seven months later, the patient had Hematological relapseHematological relapse o CBC: TLC 16000, Hb 9.6, Plt CBC: TLC 16000, Hb 9.6, Plt

26000,26000, blasts 57% blasts 57% o BM aspirate: BM aspirate: AML M3 in relapseAML M3 in relapse 20% blasts20% blastsCNS manifestationCNS manifestation

•Fever & headache•Convulsions•Aphasia•Blurring of vision •Neck stiffness•Behavioral changes

Leukemic infiltrationSuspected due to•Previous history of CNS infiltration• hematological relapse

CNS InfectionSuspected due to:•Fever &• neck stiffness

CNS disease

Empirical ttt by Brain dehydrating measures

•Salvage chemotherapy•Intra thecal injections

•Anti bacterial•Acyclovir &

Results of InvestigationResults of InvestigationEmpirical treatment was Empirical treatment was continuedcontinued

until the collection of data fromuntil the collection of data from Radiological investigations:Radiological investigations: MRI revealed: Lt tempro-parieto-MRI revealed: Lt tempro-parieto-

occipital infiltration with lepto-occipital infiltration with lepto-meningeal distribution & gyral meningeal distribution & gyral edemaedema

Laboratory investigationLaboratory investigation • CSF analysis:CSF analysis: High TLCHigh TLC High protein contentHigh protein content Normal sugar & Chloride contentNormal sugar & Chloride content• CSF C & S:CSF C & S: +ve for HSV type I by monoclonal +ve for HSV type I by monoclonal

antibodiesantibodies -ve for bacterial & fungal growth -ve for bacterial & fungal growth

Revaluation by BM aspirate Revaluation by BM aspirate revealedrevealed

Complete RemissionComplete RemissionCNS condition markedly CNS condition markedly

improvedimproved

Plan of future Plan of future treatmenttreatment

RT PCR for t 15-17

+ve

Allogenic BMT

-ve

Auto BMT