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Success Story – Zeenia Zia

abeeha majid

Age 05 years, HLH, Pre B-ALL

Zeenia Zia

Contributed By: Dr. Tariq Ghafoor

Zeenia Zia was diagnosed with Histiocytosis-Lymphadenopathy Plus Syndrome (SLC29A3 mutation) and underwent Allogenic BMT on 17/10/16 with MAC (Thiotepa, Treosulphan, Fludarabine, ATG) in India. She had fits during her BMT days and required CPR due to apnoea. Her post BMT course was uneventful except Skin GVHD and CMV reactivation.

On 30/11/2018 (25 months PBMT) she presented with low grade fever and drop in blood counts. Her bone marrow aspiration and immunophenotyping confirmed the diagnosis of Pre-B ALL with negative ALL gene markers including BCR-ABL. Her CSF examination revealed no blast cells. Her donor chimerism was 40 %.

Induction chemotherapy as per UKALL2011 Regimen A protocol was started on 07/12/18. Her Day 15 marrow showed 29% blasts; her induction chemotherapy was switched to Regimen C with two doses of daunorubicin. Her Day 29 marrow showed >5% blast. Considering her resistant disease, her treatment was switched to UKALL R3 protocol with a plan to go for Allogenic BMT. UKALL R3 consolidation was given from 22/01/19 to 12/02/19. She received Phase III intensification from 25/2/19 to 02/4/19. She was started on Interim maintenance on 09th May 2019.

Craniospinal irradiation 24 Gy/12 fr were given in April and May 2019. She tolerated chemotherapy and radiation without any major problem.

Her last two Bone marrow examinations on 10/5/19 and 17/6/19 showed iatrogenic hypoplasia and no blasts.

She was fully HLA-matched with her brother and bone marrow transplant was performed on 12/7/2019 with Bu 16, Cy200 used as conditioning protocol.

Post-transplant period was unremarkable. Neutrophil engraftment was achieved on Day+12. The patient was discharged after her neutrophil recovery and was advised weekly follow up in OPD