Journal List > Korean J Hematol > v.43(4) > 1032793

Yang, Jung, So, Lim, Han, Park, Kim, Hur, Lee, Shim, and Park: A Case of Combined POEMS Syndrome and Multiple Myeloma with Positive M protein Only on Immunofixation

Abstract

POEMS syndrome is a plasma cell proliferative disorder is characterized by the presence of peripheral neuropathy (P), organomegaly (O), monoclonal gammopathy (M), endocrinopathy or edema (E) and skin change (S). It can be rarely related to multiple myeloma. A 48-year-old man was admitted to our hospital due to paresthesia of both inguinal areas and weakness of both lower extremities. He had a history of Castleman's disease, and showed features of polyneuropathy, multiple osteoblastic lesions, hepatosplenomegaly, pretibial pitting edema, and papilledema. The serum and urine electrophoresis were negative, but urine immunofixagion could detect monoclonal protein. Plasmacytoma was confirmed through the biopsy for the osteoblastic lesions. We present a case of combined POEMS syndrome and multiple myeloma with positive M protein only on immunofixation in order to share our experience with physicians and specialists.

REFERENCES

1). Dispenzieri A., Kyle RA. Neurological aspects of multiple myeloma and related disorders. Best Pract Res Clin Haematol. 2005. 18:673–88.
crossref
2). Soubrier MJ., Dubost JJ., Sauvezie BJ. POEMS syndrome: a study of 25 cases and a review of the literature. French study group on POEMS syndrome. Am J Med. 1994. 97:543–53.
3). Bélec L., Mohamed AS., Authier FJ, et al. Human herpesvirus 8 infection in patients with POEMS syndrome-associated multicentric Castleman's disease. Blood. 1999. 93:3643–53.
4). Tokashiki T., Hashiguchi T., Arimura K., Eiraku N., Maruyama I., Osame M. Predictive value of serial platelet count and VEGF determination for the management of DIC in the Crow-Fukase (POEMS) syndrome. Intern Med. 2003. 42:1240–3.
crossref
5). Dispenzieri A. POEMS syndrome. Blood Rev. 2007. 21:285–99.
crossref
6). Rajkumar SV., Dispenzieri A., Fonseca R, et al. Thalidomide for previously untreated indolent or smoldering multiple myeloma. Leukemia. 2001. 15:1274–6.
crossref
7). Doster DR., Folds J., Gabriel DA. Nonsecretory multiple myeloma. Arch Pathol Lab Med. 1988. 112:147–50.
8). Tominaga N., Katagiri S., Hamaguchi Y, et al. Plasma cell leukaemia of non-producer type with missing light chain gene rearrangement. Br J Haematol. 1988. 69:213–8.
crossref
9). CognéM. Guglielmi P. Exon skipping without splice site mutation accounting for abnormal immunoglobulin chains in nonsecretory human myeloma. Eur J Immunol. 1993. 23:1289–93.
10). Matsuzaki H., Yoshida M., Akahoshi Y., Kuwahara K., Satou T., Takatsuki K. Pseudo-nonsecretory multiple myeloma with light chain deposition disease. Acta Haematol. 1991. 85:164–8.
crossref
11). Singhal S., Stein R., Vickrey E., Mehta J. The serum-free light chain assay cannot replace 24-hour urine protein estimation in patients with plasma cell dyscrasias. Blood. 2007. 109:3611–2.
crossref
12). Drayson M., Tang LX., Drew R., Mead GP., Carr-Smith H., Bradwell AR. Serum free light-chain measurements for identifying and monitoring patients with nonsecretory multiple myeloma. Blood. 2001. 97:2900–2.
crossref
13). Iwashita H., Ohnishi A., Asada M., Kanazawa Y., Kuroiwa Y. Polyneuropathy, skin hyperpigmentation, edema, and hypertrichosis in localized osteosclerotic myeloma. Neurology. 1977. 27:675–81.
crossref
14). Dispenzieri A., Moreno-Aspitia A., Suarez GA, et al. Peripheral blood stem cell transplantation in 16 patients with POEMS syndrome, and a review of the literature. Blood. 2004. 104:3400–7.
crossref
15). Wilson JA., Jacobs A. ABO discrepancy in a multiple myeloma patient: a case study. Clin Lab Sci. 2002.

Fig. 1
Pelvic MRI showed multifocal osteoblastic lesions at pelvic bone, sacrum, and L5 (white arrows).
kjh-43-253f1.tif
Fig. 2
Eccentrically placed nuclei of neoplastic plasma cells with basophilic cytoplasm and a sequestrum (black arrow) (Bone biopsy, H&E stain, ×400, A) and positive staining for λ chain (×400, B).
kjh-43-253f2.tif
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