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.