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.