Case 2
A 19-year-old male presented to us with fever and abdominal pain for 2 days duration. His hemogram showed haemoglobin - 10.6g/dL, white blood cell count 44.2 x 109/L, platelet count 90 x 109/L with 88% blasts in differential count. Bone marrow examination showed hypercellular marrow with 87% blasts. Flow cytometry analysis showed CD7, CD38 positive, heterogenous dim to moderate cCD3 and CD5. Dim expression for CD33. Negative expression was seen for CD1a, CD2, CD3, CD4, CD8, CD10, CD13, CD15, CD16, CD19, CD34, c CD41, CD56, cCD 79a, cMPO and cTdT suggestive of ETP ALL. Molecular markers were negative and karyotype was normal. Baseline cerebrospinal fluid evaluation was normal. He was started on BFM 95 regimen. Day 8 blast count was 12.51 x 109/L. He continued to have blast in peripheral blood until day 22 of therapy. Hence, he was started on oral venetoclax x 7 days (100 mg day 1, 200 mg day2, 300 mg day3, and 100 mg with oral posaconazole from day 4 to day 7) in addition to continuation of BFM regimen. He had cytopenias for 2 weeks after completion of therapy and post induction bone marrow evaluation on D 49 of therapy showed morphological remission with positive MRD (0.99%). He received HR1 protocol of BFM 95 with oral venetoclax (100 mg for 7 days along with oral posaconazole). Post HR1 bone marrow showed < 1 % blasts with negative MRD (<0.01%). Patient did not have a matched sibling donor. Matched unrelated donor search showed 6 fully matched donors in India. However, none of the donors were willing for donation in the setting of corona virus pandemic. He underwent haploidentical allogeneic stem cell transplant with father as donor. Conditioning was done with fludarabine 30 mg/m2 x 3 days on D-6, D-5 and D-4 followed by total body irradiation – 200 Gy twice a day x 3 days on D-3, D-2 and D-1. GVHD prophylaxis was with tacrolimus, MMF and post-transplant cyclophosphamide. Neutrophil engraftment was on day+ 13 and platelet engraftment was on day +10. He had persistently low tacrolimus trough levels and developed Grade IV acute GVHD of gut on D+ 25 of transplant. He responded to IV methylprednisolone. Tacrolimus was changed to cyclosporine. Chimerism analysis at D+ 30 showed 99.14% donor. Day +60 bone marrow evaluation showed morphological remission and negative MRD (<0.01%). At present he has completed 1 year 10-month follow-up and is doing well on Ruxolinitib for liver and gastrointestinal GVHD.