The development of cutaneous macroglobulinosis may precede a diagnosis of Waldenström macroglobulinemia, according to a case report described in JAAD Case Reports.

To date, only 8 cases of cutaneous macroglobulinosis in a patient with a history of Waldenström macroglobulinemia have been reported, according to the study authors.

The latest case report described a woman aged 56 years, who was initially diagnosed with lymphoplasmacytic lymphoma and later confirmed to have Waldenström macroglobulinemia. 

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At the time of her diagnosis of lymphoplasmacytic lymphoma, no skin lesions were present, and she was treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). However, she developed hyperviscosity syndrome, neuropathy, and elevated immunoglobulin (Ig) M — that is, an IgM level of 280,200 g/dL — leading to her discontinuation of treatment after 4 cycles and a diagnosis of Waldenström macroglobulinemia. 

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Next, the patient was treated with bendamustine-rituximab, but she had to stop treatment due to severe neutropenia. 

The patient went on to receive ibrutinib as her third line of treatment, with an IgM level of 4096 g/dL. However, 1 month after starting ibrutinib, she developed skin lesions on her face, chest, and abdomen. Ulcerated hyaline deposits were detected in the skin lesions and found to be cutaneous IgM deposits. 

The patient eventually achieved a partial response, and at that time, her IgM level was 2242.28 g/dL. She was also diagnosed as having type 1 cryoglobulinemia. 

In the fourth-line setting, the patient was treated with rituximab, ifosfamide, carboplatin and etoposide with concurrent ibrutinib, but she discontinued due to pancytopenia and septic shock. 

The patient is currently waiting for approval to be treated with tisagenlecleucel.

The study authors explained that the “temporal relationship” between cutaneous macroglobulinosis and Waldenström macroglobulinemia “varies” because patients can develop cutaneous macroglobulinosis before, concurrent with, or — in this case — after diagnosis of the underlying plasma cell dyscrasia. 

“Hence, diagnosis of CM [cutaneous macroglobulinosis] can permit the diagnosis of a latent plasma cell dyscrasia before any other indicative information becomes available,” the study authors asserted.

Disclosure: Dr Alencar has received honoraria for consulting from Genentech and Celgene. For a full list of disclosures, please refer to the original abstract.


  1. Fayne R, Rosenberg M, White K, et al. Disseminated cutaneous immunoglobulin M macroglobulinosis associated with cryoglobulinemia and minimal residual disease of Waldenström macroglobulinemia. JAAD Case Rep. 2019;5(10):918-922.