Case Presentation:
A 45-year-old Chinese Indian woman presented in Feb 2015 with complaints of ‘persistent’ back pain and a recent history of total hysterectomy for an ovarian mass diagnosed as ovarian plasmacytoid neoplasm. She had no other localizing symptoms, no B symptoms and informed us of a family history of breast cancer (mother). Examination revealed bilateral (B/L) breast lumps (3cms max). CT thorax and abdomen showed additional lesions including few small left axillary lymph nodes, nodular lesions in bilateral adrenal glands (4.2cm max) and altered signals in L1 vertebrae with an associated paravertebral soft tissue mass (5.3cm max) compressing the conus. HIV test (by ELISA) was negative. Biopsy of the breast lumps showed lymphoproliferative neoplasm with plasmacytoid histology [positive for CD138, EBER-ISH, and kappa light chain restriction] s/o Plasmablastic Lymphoma. Bone marrow (BM) aspirate showed 6% plasma cell with no atypical cells. Cytogenetics on Marrow was FISH 17p deleted in 8% plasma cells. Baseline Protein electrophoresis was not done. She received local RT for pain control in the dorso-lumbar region. With a curative intent, she was treated with 6 cycles of dose-adjusted EPOCH (daEPOCH). After 6 cycles of daEPOCH PET CT showed complete metabolic response (cMR). This was consolidated with BEAM high dose chemotherapy and autologous hematopoietic stem cell transplant (AHCT) in Aug 2015. Follow up PET-CT 3 months after AHCT continued to be in cMR. On regular follow-up, in Jan 2017, she relapsed with B/L breast lumps (6cms max) \soutand confirmed by repeat biopsy (CD138 and CD 38 positive). PET CT showed an additional lytic lesion in the seventh rib. BM and CSF were uninvolved. cMYC rearrangement was negative. She received salvage DHAP chemotherapy from Feb 2017. PET-CT post 3 cycles, showed reduction in size of breast lumps but progression with new lesions in ribs. She was switched to a Bortezomib and Lenalidomide (VR) regimen from May 2017. Daratumumab(D) was added from July 2017. After 4 cycles of combination DaraVR, PET-CT showed mild residual metabolic activity in bilateral breast lesions. She received local radiotherapy (RT) to B/L breast (45 Gy in 25# over 5 weeks). Post RT, PET-CT showed cMR. This was followed by a haploidentical allogenic hematopoietic cell transplant (AlloHCT) using T-cell replete peripheral blood stem cells from her haploidentical sibling brother in Dec 2017. Conditioning regimen used was myeloablative Fludarabine-Busulfan. Graft versus host disease (GVHD) prophylaxis consisted of a combination of post-transplant cyclophosphamide, tacrolimus and mycophenolate (PTCy+Tac+MMF). Peri-transplant period was uneventful. She had complete engraftment by Day +15. In April 2018, by Day + 94, she developed Skin and Gut GVHD for which she received Methylprednisolone 1 mg/kg along with oral budesonide, and Tacrolimus (oral) was continued. GVHD settled thereafter. She had a relapse of skin GVHD on Day +189 which settled with steroid re-challenge. Her immunosuppression was later tapered and stopped by 12 months post-transplant. A PET CT repeated in Oct 2019 showed cMR. At last follow up in March 2020, she remained disease free after more than 2 years of AlloHCT.