[5808]

Monoclonal gammopathy, complement deficiency, angioedema

by 5808 on Mon Apr 11, 2016 7:56 am

Hello All,

I wanted to throw a dart and see what might come from this post. My wife (early 30's) is currently being closely monitored for a monoclonal gammopathy associated with a complement deficiency / suspected acquired angioedema (AAE).

Background:

Late last year my wife presented to an immunologist for evaluation of recurrent hive-like episodes effecting various parts of her body over a 6 to 8 week period. Lab testing determined the suspected AAE associated with a small M-spike. She was referred to a local hem/onc for workup. We additionally visited with a nationally known myeloma specialist (based locally) for consult at the request of our hem/onc. Both physicians are concerned but perplexed. There is debate on best course of action. The hive-like episodes have resolved. There have been a series of subsequent GI episodes consistent with an angioedema event.

LABS

Bone Marrow: 9%

(Flagged Values:)
Free Kappa/Lambda Ratio: 0.17 (latest) / 0.20 / 0.22
Free Lambda L.C. Ser QN: 119.52 mg/L (latest) / 118.10 mg/L / 80.00 mg/L
Free Kappa L.C. Ser QN: 20.42 mg/L (latest) / 23.56 mg/L / 17.22 mg/L

SPE M Protein: 1.46 g/dL (latest) / 1.42 / 1.22 / 1.12
SPE Gamma: 2.16 g/dL (latest) / 2.21 / 2.06 / 2.03
SPE Albumin: 3.23 g/dL (latest) / 3.26 / 3.14 / 3.29

C4 Complement Ser QN: 4 mg/dL (latest) / 4 / 6 / 6
C1Q Pl QN: < 50 mCg/mL
Total Complement Ser QN: < 390 CH 100 Units/mL

IgG Ser QN: 1820 mg/dL

IMAGING

Bone Scan: Negative
PET: Negative

Anyone else dealing with a monoclonal gammopathy associated with a complement deficiency and/or angioedema?

[jhorner]

Re: Monoclonal gammopathy, complement deficiency, angioedema

by jhorner on Mon Apr 11, 2016 4:02 pm

Hello,

I'm not a doctor, but I believe complement deficiency is often associated with an autoimmune disease and MGUS, smoldering multiple myeloma, multiple myeloma are often associated with an initial immune problem, so I don't think it is that surprising for your wife to have both. Angioedema is also often caused by an overactive immune response or other immune problem. I have a son that gets this and onset could be triggered by seasonal allergies, medication, food etc.

I think what would be important for her having both is a quantitative immunoglobulin test which measures the level of all antibodies. I suggest this because angioedema is often associated with a low IgA and often MGUS causes suppression of this antibody. You mentioned her IgG level, so this test may have already been completed already. If so, see if this was low and that might help you connect these ailments, keeping in mind they are all essentially connected, being immune system problems.

Best
J.