Abstract:Unusual clinical course Background: Plasma cell myeloma is a neoplastic plasma cell disorder that usually presents after the fifth decade of life; it is rarely described in younger population especially under 30 years of age. However, there are conflicting reports in the literature about the clinical behavior and overall survival in younger age groups. In approximately 2% of plasma cell myeloma, the morphology of the neoplastic cells is highly pleomorphic, quite anaplastic, and may resemble metastatic tumor ce… Show more
“…Therefore, a new line of chemotherapy [cisplatin, doxorubicin, etoposide, cyclophosphamide] combined with pomalidomide and Carfilzomib was started for two cycles the patient planned for ASCT. 1, cases [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. Upon an extensive review of English literature, a total of 22 cases of PCNs (including the three cases reported here) showed aberrant co-expression of Tcell associated markers.…”
Section: Results Results Of Patients Diagnosed In Our Centermentioning
confidence: 99%
“…16 Moreover, anaplastic myeloma variant, in which the malignant PCs are highly pleomorphic, has been reported more common in younger patients with a predisposition for the extramedullary site and poor prognosis. 14,17 This morphologic variant may present initially at diagnosis 16 or as a feature of disease progression. 18 Aberrant expression of T-cell antigens has also been rarely described in cases with the plasmablastic transformation of PCM, and it has been postulated that EBV may stimulate T-cell antigen expression in B-lineage neoplasms.…”
Section: Discussion Aberrant Expression Of Differentiation Markers Of a Different Cell Lineage On The Malignantmentioning
Background:
Plasma cell neoplasms can show aberrant expression of a different lineage-related antigens, however, co-expression of T-cell associated markers on malignant plasma cells is extremely rare.
Material and methods: This is a report of clinicopathologic characteristics of three myeloma patients with emergent plasmablastic morphology and aberrant acquisition of T‐cell associated markers. An extensive literature search for similar cases was conducted and the relevant pathologic, clinical and prognostic characteristics were summarized.
Results: A total of 22 cases of plasma cell neoplasm, showed aberrant co-expression of T-cell markers. We found an evident association between aberrant expression of T-cell markers on malignant plasma cells and extramedullary involvement, aggressive morphologic features, high proliferative index ki67 >90%, aggressive clinical course, adverse outcome with short survival.
Conclusion:
Due to rarity of this aberrant phenotype and scarcity of the published data, the precise causative mechanism and its clinical implications have not yet been elucidated.
“…Therefore, a new line of chemotherapy [cisplatin, doxorubicin, etoposide, cyclophosphamide] combined with pomalidomide and Carfilzomib was started for two cycles the patient planned for ASCT. 1, cases [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. Upon an extensive review of English literature, a total of 22 cases of PCNs (including the three cases reported here) showed aberrant co-expression of Tcell associated markers.…”
Section: Results Results Of Patients Diagnosed In Our Centermentioning
confidence: 99%
“…16 Moreover, anaplastic myeloma variant, in which the malignant PCs are highly pleomorphic, has been reported more common in younger patients with a predisposition for the extramedullary site and poor prognosis. 14,17 This morphologic variant may present initially at diagnosis 16 or as a feature of disease progression. 18 Aberrant expression of T-cell antigens has also been rarely described in cases with the plasmablastic transformation of PCM, and it has been postulated that EBV may stimulate T-cell antigen expression in B-lineage neoplasms.…”
Section: Discussion Aberrant Expression Of Differentiation Markers Of a Different Cell Lineage On The Malignantmentioning
Background:
Plasma cell neoplasms can show aberrant expression of a different lineage-related antigens, however, co-expression of T-cell associated markers on malignant plasma cells is extremely rare.
Material and methods: This is a report of clinicopathologic characteristics of three myeloma patients with emergent plasmablastic morphology and aberrant acquisition of T‐cell associated markers. An extensive literature search for similar cases was conducted and the relevant pathologic, clinical and prognostic characteristics were summarized.
Results: A total of 22 cases of plasma cell neoplasm, showed aberrant co-expression of T-cell markers. We found an evident association between aberrant expression of T-cell markers on malignant plasma cells and extramedullary involvement, aggressive morphologic features, high proliferative index ki67 >90%, aggressive clinical course, adverse outcome with short survival.
Conclusion:
Due to rarity of this aberrant phenotype and scarcity of the published data, the precise causative mechanism and its clinical implications have not yet been elucidated.
“…They can mimic high-grade lymphoma or non-hematopoietic malignancy by morphology [ 15 ]. The cells often resemble dysplastic megakaryocytes or giant cell osteoclasts [ 16 ]. In this case, the initial panel including CD3, CD20, CD138, and AE1/3 were all negative.…”
Anaplastic myeloma (AM) is an extremely rare and aggressive histological variant of myeloma. It is characterized by extramedullary presentation in the young and has a poor prognosis. It can be a diagnostic challenge when myeloma is not suspected and even more when the immunophenotype is unexpected. We present a rare presentation of anaplastic myeloma with cardiovascular involvement. Though the patient did not have the typical clinical features of myeloma, except lytic lesion in the femur, the cardiac biopsy showed sheets of anaplastic cells, and some with multinucleation. There were also some areas with a more plasmacytoid appearance. The initial immunohistochemical panel was negative for CD3, CD20, CD138, AE1/3, and kappa. It was positive for lambda. This led to an extended panel which showed positivity for CD79a and MUM1 and negative for LMP-1, HHV-8, CD43, CD117, CD56, and CD30. Even the flow cytometry on the bone marrow showed a small population of atypical cells positive for CD38 and negative for CD138 with lambda restriction. This is an unusual case of anaplastic myeloma with cardiovascular involvement and CD138 negativity. This case highlights the need to add a panel of plasma cell markers when myeloma is suspected, and it is pertinent to read flow cytometry with caution to avoid missing atypical plasma cells which maybe CD38+/CD138−.
“…2,4,5 Penelitian oleh Elsabah dkk juga menemukan hiperkalsemia dan peningkatan kreatinin pada pasien PCM. 10 Berbeda dengan kepustakaan, pada kasus ini hanya sedikit peningkatan kreatinin, tidak ada hiperkalsemia, hipoalbuminemia maupun leukopenia dan trombositopenia. Laporan kasus oleh Gupta dkk menemukan 25% PCM dengan insufisiensi renal serta 0% hiperkalsemia dan hipoalbuminemia.21 Sejalan dengan penelitian oleh Sudandyo dkk, ditemukan hanya 4% hiperkalsemia pada pasien PCM.…”
Pendahuluan: Plasma cell myeloma merupakan keganasan tulang primer yang paling sering dijumpai dengan keluhan utama nyeri tulang belakang, dan jarang dengan gejala awal nyeri pada humerus. Diagnosis didasarkan pada temuan klinis, radiologis dan histopatologis. Laporan Kasus: Kami melaporkan kasus seorang laki-laki berusia 60 tahun datang dengan keluhan nyeri pada lengan atas kanan dan gangguan berkemih. Sebelumya pasien memiliki riwayat nyeri tulang belakang dengan dugaan fraktur kompresi torakal 12 akibat metastase. Hasil reseksi prostat transuretra (TURP) menunjukkan hiperplasia prostat. X-ray humerus memberikan hasil simple bone cyst dan CT scan dada menunjukkan chondroma.Pemeriksaan laboratorium tampak anemia ringan, normokalsemia, serta kreatinin normal. Bone survei tampak beberapa punched out lytic lession pada tengkorak, mandibula, klavikula dan panggul. Biopsi humerus dilakukan, hasil histopatologi adalah Plasma cell myeloma, well differentiated. Imunohistokimia CD138 memberikan hasil positif difus. Kesimpulan: Dalam kasus klinis non-spesifik, diagnosis yang akurat diperoleh dari patologi. Dalam hal ini, hasil histopatologi adalah sel plasma neoplastik yang difus, dibuktikan dengan penanda CD138 positif difus dan diagnosis didukung oleh anemia dan lesi litik tulang yang multipel.
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