Vnitr Lek 2015, 61(9):821-828

Myopathy in patients with Waldenström's macroglobulinemia. A case study and an overview of autoimmune expressions of type IgM monoclonal immunoglobulins

Zdeněk Adam^1,*, Jarmila Kissová¹, Luděk Pour¹, Marta Krejčí¹, Eva Ševčíková¹, Renata Koukalová², Zdeňka Čermáková³, Marta Černá⁴, Zdeněk Král¹, Jiří Mayer¹

¹ Interní hematologická a onkologická klinika LF MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Mayer, CSc.

² Oddělení nukleární medicíny, pracoviště pozitronové emisní tomografie, Masarykův onkologický ústav, Brno, prim. MUDr. Zdeněk Řehák, Ph.D.

³ Oddělení klinické biochemie FN Brno, pracoviště Bohunice, a Katedra laboratorních metod LF MU, Brno, přednosta doc. MUDr. Milan Dastych, CSc., MBA

⁴ Hematologicko transfuzní oddělení Uherskohradišťské nemocnice, a.s., Uherské Hradiště, prim. MUDr. Marta Černá

In rare cases, monoclonal immunoglobulins bind to intrinsic antigens whereby causing autoimmune damage to the body. The text describes Waldenström's macroglobulinemia which was manifested by muscle pain and anemia. The female patient had suffered from back pain for about 3 years before she came to our clinic. In the last year pain in the muscles of the upper and lower extremities developed in addition to back pain. This led to the suspicion of polymyositis. However this was not confirmed by a special examination. The patient was diagnosed with clearly established infiltration of lymphoplasmacytic lymphoma and 10.8 g/l of type IgM monoclonal immunoglobulin in the bone marrow. Serum myoglobin levels and serum CK activity were repeatedly significantly increased. Therefore the treatment with anti-CD20 monoclonal antibody (Mabthera) 375 mg/m² i. v. was started, administered once a month, with cyclophosphamide 500 mg/m² i. v. on days 1 and 15 of a 28-day cycle, and dexamethasone 20 mg from 1st through to 4th days and 15th through to 18th days of the treatment cycle. There were 8 cycles planned. Already after a 5th cycle, the disappearance of monoclonal immunoglobulin (negative immunofixation), normalisation of myoglobin and CK values and significant relief from muscle pain were achieved. The hemoglobin concentrations before treatment were significantly reduced, while they were normalised after treatment. After 5 cycles, the complete remission of Waldenström's disease was reached according to biochemical parameters, and normalisation of the serum myoglobin and creatine kinase levels was achieved.

Keywords: Mabthera; myopathy; type IgM monoclonal immunoglobulin; Waldenström's macroglobulinemia

Received: December 3, 2014; Accepted: April 20, 2015; Published: September 1, 2015  Show citation