J Pathol Transl Med.  2023 Mar;57(2):132-137. 10.4132/jptm.2023.02.07.

Unusual biclonal IgA plasma cell myeloma with aberrant expression of high-risk immunophenotypes: first report of a new diagnostic and clinical challenge

• Monroig-Rivera CA
• Cruz CNF

• KMID: 2540500
• DOI: http://doi.org/10.4132/jptm.2023.02.07

Abstract

IgA plasma cell myeloma (PCM) has been linked to molecular abnormalities that confer a higher risk for adverse patient outcomes. However, since IgA PCM only accounts for approximately 20% of all PCM, there are very few reports on high-risk IgA PCM. Moreover, no such reports are found on the more infrequent biclonal IgA PCM. Hence, we present a 65-year-old Puerto Rican female with acute abdominal pain, concomitant hypercalcemia, and acute renal failure. Protein electrophoresis with immunofixation found high IgA levels and detected a biclonal IgA gammopathy with kappa specificity. Histomorphologically, bone marrow showed numerous abnormal plasma cells (32%) replacing over 50% of the marrow stroma. Immunophenotyping analysis detected CD45-negative plasma cells aberrantly expressing CD33, CD43, OCT-2, and c-MYC. Chromosomal analysis revealed multiple abnormalities including the gain of chromosome 1q. Thus, we report on an unusual biclonal IgA PCM and the importance of timely diagnosing aggressive plasma cell neoplasms.

Keyword

Biclonal IgA; Plasma cell myeloma; Abdominal pain; High-risk phenotypes; Prognosis

Figure

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  Fig. 1. Histomorphology of biclonal IgA plasma cell myeloma. (A) Bone marrow imprint shows increased (32%) atypical plasma cells with features between immature and mature forms (“Intermediate-type” morphology); binucleation and rare plasmablasts are seen (Wright-Giemsa stain). (B) Bone marrow biopsy is hypercellular (85%–95%) with > 50% replaced by sheets of plasma cells.

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  Fig. 2. Semiquantitative, morphometric analysis of biclonal plasma cells done using immunohistochemical stains on the decalcified, formalinfixed bone marrow biopsy sample. Plasma cells express MUM1 (> 90%) (A), but aberrantly express high-risk marker CD43 (> 95%, a T-cell marker) (B), and overexpress high-risk markers OCT-2 (70%–80%) (C) and c-MYC (15%–25%) (D).

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  Fig. 3. Flow cytometry analysis of bone marrow cells detects increased clonal plasma cells (~11% of the viable cells; in black) that are positive for CD138 (A), lack CD45, and aberrantly express CD33 (a myeloid marker associated with high-risk myeloma) (B).

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  Fig. 4. Chromosome analysis revealed an abnormal complex karyotype including gain of 1q (arrow), a high-risk prognostic marker in plasma cell myeloma.

Reference

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