In this lecture we review mGODS: Monoclonal Gammopathies of Dermatological Significance1, an umbrella term that captures diseases such as Cutaneous Amyloidoses, Scleromyxedema, Acquired Cutis Laxa, Mixed Cryoglobulinemia, Nodular Amyloidoses, PEOMS syndrome, Necrobiotic Xanthogranuloma, Schnitzler’s Syndrome etc.
Specifically we will:
Provide an overview of plasma cell dyscrasias
Introduce the concept of mGODS
Review techniques to detect and identify monoclonal proteins
Review how to work up paraproteins
Provide an overview of MGUS
Here, we provide a primer for that lecture. We start with a clinical case and some discrete questions so that you can think through them ahead of the lecture. We will go through these as a group on Thursday.
Ms. S is a 82-year-old who presents for follow up for “Pruritus Without Rash”. She has been seen by two of your colleagues before, but this is the first time you are seeing her. The initial “Pruritus Without Rash” work up was notable for a normal CBC with Diff, CMP, TSH and CXR. Given that this initial work up was non-diagnostic, a second round of investigation was performed and was notable for the following: Serum iron WNL; ferritin WNL; skin biopsy not diagnostic, DIF negative; SPEP demonstrated abnormal band in gamma region, identified by IFE as IgG Kappa, and represents by densitometry 10% (730 mg/dL) of total protein.
Case 1 Clinical Questions:
What does this part of the work up mean?
SPEP demonstrated abnormal band in gamma region
Identified by IFE as IgG Kappa
Represents by densitometry 10% (730 mg/dL) of total protein
What are you next steps in management?
While referral to hematology is never the wrong answer, if you wanted to pursue additional work up, what would be your next steps?