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بسم الله الرحمن الرحيم. Case Presentation. Patient’s History. A. Y. female patient 4 years old is living in Cairo Presented outside NCI by Persistent fever Pallor Outside NCI CBC: 55% blasts. Clinically. Fair general condition Pallor Symptoms of increased ICT
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Patient’s History • A. Y. female patient 4 years old is living in Cairo • Presented outside NCI by • Persistent fever • Pallor • Outside NCI CBC: 55% blasts
Clinically • Fair general condition • Pallor • Symptoms of increased ICT • No other neurological deficit • No organomegaly or lymphadinopathy • Chest, heart, & abdominal examination: free
Investigations • CBC: TLC 7500, ANC 500, Hb 6, Plt 65000 • BM aspiration: AML M3 • IPT: myeloid CD 33, 13 MPO +ve • Cytogenetics: 46 XX t 15-17 • CSF: ++ve • Brain MRI: normal • Chest x-ray & abdominal US: free
Treatment • Induction: • ADR 45 mg/ m2 (d 1, 2, & 3) • ATRA 45 mg/ m2 till remission • Intra thecal weekly • Consolidation: • 2 courses of ADR 45 mg/ m2 (d 1, 2, & 3)
She was planned to receive craniospinal radiotherapy • & to be put on maintenance chemotherapy • MTX 20 mg /m2 weekly • 6 MP 75 mg / m2 • ATRA 45 mg /m2 for 2 weeks every 12 weeks • Radiotherapy was postponed due to poor chest condition
Chest condition • Fever • Respiratory distress Gr III • Neutropenia • Chest x-ray: pleural effusion Chest Infection ATRA Syndrome Malignant effusion Plural effusion cytology: -ve Stopped chemotherapy
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 Re evaluation after 2 weeks • 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
After improvement of the chest condition, the patient received • Craniospinal irradiation • Continued maintenance chemotherapy
Seven months later, the patient had • Hematological relapse • CBC: TLC 16000, Hb 9.6, Plt 26000, blasts 57% • BM aspirate: • AML M3 in relapse • 20% blasts • CNS manifestation
CNS disease • Fever & headache • Convulsions • Aphasia • Blurring of vision • Neck stiffness • Behavioral changes • Leukemic infiltration • Suspected due to • Previous history of CNS infiltration • hematological relapse • CNS Infection • Suspected due to: • Fever & • neck stiffness Empirical ttt by Brain dehydrating measures • Salvage chemotherapy • Intra thecal injections • Anti bacterial • Acyclovir &
Results of Investigation Empirical treatment was continued until the collection of data from • Radiological investigations: MRI revealed: Lt tempro-parieto-occipital infiltration with lepto-meningeal distribution & gyral edema
Laboratory investigation • CSF analysis: • High TLC • High protein content • Normal sugar & Chloride content • CSF C & S: • +ve for HSV type I by monoclonal antibodies • -ve for bacterial & fungal growth
Revaluation by BM aspirate revealed Complete Remission CNS condition markedly improved
Plan of future treatment RT PCR for t 15-17 +ve -ve Allogenic BMT Auto BMT