A 27 year old male presented with pain in the left scapular region x 1 months. On examination 2 x 2 cm left axillary node was noted. No hepatosplenomegaly and no other palpable peripheral nodes were seen.
Relevant Blood parameters were as follows:-
USG guided Biopsy was performed: Impression
• Hematolymphoid neoplasm, high grade
Features are intermediate between Plasmablastic myeloma and Plasmablastic lymphoma.
IHC: The atypical cells are positive for CD138, MUM-1 and CD45 while negative for PanCK, CD20, cyclin D1, TdT and S 100. [Note: EBER-ISH is negative]
PET/CT: Findings: FDG avid destructive lytic lesion with soft tissue component noted involving the left 5th rib measuring 7.1×7.5×5.6cm (SUVmax 13.61) + Multiple Hypodense splenic lesions + supra and infra diaphragmatic adenopathy + no Bone marrow uptake.
|FDG avid destructive lytic lesion with soft tissue component noted involving the left 5th rib measuring 7.1×7.5×5.6cm (SUVmax 13.61)||Multiple FDG avid hypodense lesions are noted involving the spleen largest measuring 6.0×6.9cm (SUVmax 10.23)|
So what is your final diagnosis at this time point ?
- Plasmablastic myeloma or Plasmablastic lymphoma.
Plasmacytoma and plasmablastic lymphoma have the same immunophenotype, other than EBV and are generally separated based on clinical grounds.
|Plasmablastic Lymphoma|| Plasmablastic M
|Plasmablastic morphology||A Subset of atypical cells are plasmablastic|
|Often HIV+ or otherwise immunosuppressed||Usually not immunosuppressed|
|Often in oral cavity or mucosal areas of head||Extraosseous sites overlap|
|~60% EBV+||EBV negative|
|Myeloma like clinical presentation such as renal failure, presence of M Band is note seen.||Myeloma like clinical presentation such as renal failure, presence of M Band is seen.|
|M Band Negative||Presence of M Band points towards myeloma diagnosis.|
|Bone marrow is mostly uninvolved||Usually bone marrow is involved except in cases of plasmablastic plasmacytoma.|
So in this cases SPEP/SFLC/SIF was sent and bone marrow was performed.
Monoclonal Band: Negative (Remarks Polyclonal, IgA and IgG raised)
Immunofixation (Qualitative): No cross reactivity seen.
BMA = NO CLONAL PLASMA CELLS PC < 5%
So based on above clinical scenario a diagnosis of Plasmablastic lymphoma was made although EBV was negative (IHC EBER-ISH negative).
Treatment: Plasmablastic Lymphoma
Plasmablastic lymphoma (PBL) is a rare and aggressive CD20-negative lymphoma associated with poor outcomes. Multiple studies have shown median survival times of 12–18 months. Several case reports and small case series have suggested an increased response rate in patients treated with bortezomib alone or in combination, especially the combination of bortezomib and DA-EPOCH. Bortezomib can be administered at 1·3 mg/m2 SQ or IV weekly or twice weekly with each cycle of EPOCH (etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin).
So presently preferred approach for treatment of plasmablastic lymphomas is Bortezomib 1.3 mg/m2 on (Day 1,4,8,11) + DA EPOCH (21 day cycle).