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| CASE REPORT |
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| Year : 2022 | Volume
: 13
| Issue : 4 | Page : 277-279 |
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Dysplastic transformation of plasma cell myeloma
Laila Alsuhaibani1, Tarek Owaidah2
1 Department of Haematopathology, Hematology Unit, Pathology and Clinical Laboratory Medicine Administration, King Fahad Medical City, Riyadh, Saudi Arabia
2 Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center, Hematology Unit, Riyadh, Saudi Arabia
| Date of Submission | 22-May-2021 |
| Date of Acceptance | 11-Nov-2021 |
| Date of Web Publication | 18-Oct-2022 |
Correspondence Address:
Dr. Laila Alsuhaibani
Hematology Unit, Pathology and Clinical Laboratory Medicine Administration, King Fahad Medical City, Riyadh
Saudi Arabia
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/joah.joah_64_21
Rare cases of plasma cell myeloma with morphological variants have been reported. Here we report a case of a plasma cell myeloma with a very unusual presentation diagnosed based on flow cytometry, immunohistochemistry, and cytogenetic studies.
Keywords: Dysplastic plasma cell morphology, FISH, immunophenotype
How to cite this article:
Alsuhaibani L, Owaidah T. Dysplastic transformation of plasma cell myeloma. J Appl Hematol 2022;13:277-9 |
| Introduction | |  |
Plasma cell myeloma is a malignant proliferation of plasma cells in bone marrow associated with an M-protein in serum and/or urine. Bone marrow examination is essential to establish the diagnosis of multiple myeloma in association with other clinical and laboratory features. In bone marrow examination, most cases show the mature pattern of neoplastic plasma cells. Atypical morphologic features have been reported more commonly in plasma cell leukemia. These include crystal-storing histiocytes, Burkitt-like cells, blastoid cells, and monocytoid plasma cells with cleaved nuclei.[1],[2],[3],[4] In this report, we describe a rare case of plasma cell myeloma that shows significant dysplastic changes of plasma cells with an irregular, deeply indented nuclear contour and basophilic cytoplasm.
| Case Report | | |
A75-year-old man was initially diagnosed in 2007 with IgA kappa plasma cell myeloma. The patient achieved a very good response to vincristine, doxorubicin (Adriamycin), and dexamethasone chemotherapy, and then he underwent autologous stem cell transplantation twice in 2007 and 2012. The patient had disease progression in September 2015 and was started on carfilzomib. Velcade and dexamethasone were then added, and complete remission was achieved. After a continuation of the medication, he started to show progression and was tried on thalidomide for 2 months with no response. He was then changed to Velcade again. The patient started on pomalidomide in November 2015 and showed less than a partial response but no disease progression. After that, he started on daratumumab with Velcade and dexamethasone with a good initial response and complete normalization of his IgA level. In February 2017, he started to have a slight progressive rise in his IgA level, reaching a maximum level of 30.24 g/L in November 2017.
A bone marrow examination was performed and revealed that around 37% of plasma cells in the aspirate had significant dysplastic changes, were multinucleated, and exhibited basophilic cytoplasm [Figure 1]. The bone marrow biopsy showed 100% cellularity with diffuse infiltration by mononucleated cells and some multinucleated forms [Figure 2]. Trilineage hematopoiesis was suppressed. | Figure 1: Dysplastic plasma cells having irregular, deeply indented nuclear contour with basophilic cytoplasm (Giemsa, oil immersion)
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 | Figure 2: Bone marrow biopsy tissue showing diffuse sheets of pleomorphic plasma cells with dysplastic features plasma cells (H and E, oil immersion)
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Immunohistochemistry revealed that these cells were strongly positive for the surface CD38 protein, CD138 protein, and the cytoplasmic kappa light chain protein. However, flow cytometry findings showed negative CD38.
FISH study performed on bone marrow cells showed positive for IGH/CCND1 fusion (t[11;14]) and monosomy 13 (D13S319/LAMP1) which were detected in 34% and 48% of cells, respectively [Figure 3]. | Figure 3: Interphase FISH analysis of chromosomal anomalies. (a) Fusion signals for t(11;14). Probes for IGH are green and probes for CCND1 are red. Loss of one copy of chromosome 13. (b) Loss of one copy of chromosome 13
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| Discussion | | |
Unusual morphological presentation of multiple myeloma sometimes makes the diagnosis more challenging and needs ancillary studies to establish a definitive diagnosis. Morphologic features of the atypical cells in this patient initially give rise to a differential diagnosis of non-Hodgkin lymphoma, myelodysplastic syndrome, or nonhematologic malignancy; however, immunohistochemistry and flow cytometry findings assisted to make the final diagnosis of relapsed plasma cell myeloma. Immunohistochemical studies are used for quantifying plasma cells in bone marrow biopsy, confirming monotypic light chain restriction, and distinguishing plasma cell myeloma from other neoplasms. Using CD138 is helpful for quantifying plasma cells, and clonality can be determined with staining for Ig kappa and lambda light chains.[5] Immunophenotype for neoplastic plasma cells usually express CD38 and CD138 and are generally CD19 negative. However, in our case, CD38 was negative since the patient was on daratumumab, an IgG1k monoclonal antibody directed against CD38. The cells typically have monotypic cytoplasm Ig and they usually lack surface Ig. CD56 is expressed in 75%–80% of the cases. Occasionally, myeloid and monocytic markers are expressed.[6],[7],[8]
FISH study was performed on bone marrow cells, which can detect >90% of abnormalities. It showed positive for IGH/CCND1 fusion (t[11;14]) and monosomy 13 (D13S319/LAMP1). The presence of t(11;14)(q13;q32) is the most common translocation detected in multiple myeloma and is usually associated with a lymphoplasmacytic morphology.[9] The t(11;14) is recognized as a standard risk by Mayo Stratification of Myeloma and Risk-Adapted Therapy. Clinically, this type of myeloma is insignificant. Few recent studies have shown the time to progression and overall survival were not affected by t(11;14).[10] Monosomy or partial deletion of chromosome 13 (13q14) is found by FISH in nearly half of the Plasma cell myeloma (PCMs) and considered as an intermediate risk with overall survival of 4–5 years.[11],[12]
We conclude that atypical morphological features of plasma cells can be challenging at the time of diagnosis. Thus, clinical correlation, flow cytometry, immunohistochemistry, and genetic studies are essential to confirm the diagnosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]
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