Concluded Case

Respected teachers, please teach me through this case.

70 year Male, (comorbidities are controlled hypertension and severe depression) with history of productive cough, no fever, sweating profusely in winter. His first CBC was hb 12 and esr 150 in first hour, 120 In second hour, 3 months back. Subsequently his creatinine started rising, first report being 1.9 , lft showed slightly raised globulin, normal ALP, normal calcium. Subsequent cbc reported hb 11.2, WBC 15000 with predominant neutrophilia , and no sign of fever, profuse sweating was present, sputum AFB and gram stain was negative and sputum c/s was negative, no other focus of neutrophilia could be found. LFt again showed mild raised globulin, esr was still raised till 90, creat was 1.6. So anemia, raised creat, slightly raised globulin, with raised esr led me to send serum protein electrophoresis which brought back M band of 3g/L. So immunofixation studies and free light chain assay and 24 hr urinary protein was sent, reports are attached. Kappa and lambda are raised, serum microglobulin is raised. Its not multiple myeloma as immunofixation almost rules out....but I am not sure why is light chain high, why is microglobulin high, Can it be light chain disease? Because his creat is raised and egfr is 41..... Or is it amyloidosis? I can't seem to understand more than what I have done. I leave interpretation to teachers here to tell me what to think and how to proceed...where all I went wrong....what all should I think? Was my approach right?

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Concluded answer

To diagnosed plasma cell dyscrasia (PCD) you need to have a monoclonal protein, either in blood or urine. Normal plasma cells usually make either kappa or lambda light containing immunoglobulin and are usually equal in number. However, if there is a clonal process one light chain is produced more than the other therefore leading to an abnormal free light chain ratio. Now coming to your patient M-spike 1 report says not detected, the other one is 0.3mg/dl. The free light chain ratio is normal, although the individual number is high. Urine M spike is not done. B2M is high. Cr is high and calcium is normal. 1) I do not think there is a clonal process ongoing as FLCR is normal and one SPEP report said that there is no monoclonal component is present. Now the other report says that there is an M spike present. Let us presume that report is correct. Then this patient would be classified as having low-risk MGUS (M spike is less than 1.5 g/dl and normal FLCR) which is very common in patients >70 years. Of course, there are variant MM conditions like low secreting MM and non-secreting MM where you see a small or no M spike at all. Those are usually diagnosed if the patient has a bony lesion, anemia (hb <10) which cannot be attributed to another condition, or high plasma cell count on bone marrow biopsy. That does not seem to be the case with your patient. 2) Light chain disease may not have an M spike in the serum as most of the light chains are excreted out in the urine. However, they will have an abnormal serum FLCR. A UPEP can be done, however, I doubt it will show a monoclonal component. 3) For diagnosing AL amyloid you need to have an M-spike. Usually in AL amyloid patients with kidney involvement have nephrotic range proteinuria, not the case with this patient. 4) In your patient the absolute number of FLC is high which can happen due to any infection/inflammation. The ratio is important, not the absolute number. 5) B2M is a non-specific marker. It can be raised in CKD, inflammation. Not used to diagnosed PCD, only used in staging. What I would do- Find another cause of patient's complaints. Just for surety repeat SPEP, FLCR, and UPEP in 2 months.

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To diagnosed plasma cell dyscrasia (PCD) you need to have a monoclonal protein, either in blood or urine. Normal plasma cells usually make either kappa or lambda light containing immunoglobulin and are usually equal in number. However, if there is a clonal process one light chain is produced more than the other therefore leading to an abnormal free light chain ratio. Now coming to your patient M-spike 1 report says not detected, the other one is 0.3mg/dl. The free light chain ratio is normal, although the individual number is high. Urine M spike is not done. B2M is high. Cr is high and calcium is normal. 1) I do not think there is a clonal process ongoing as FLCR is normal and one SPEP report said that there is no monoclonal component is present. Now the other report says that there is an M spike present. Let us presume that report is correct. Then this patient would be classified as having low-risk MGUS (M spike is less than 1.5 g/dl and normal FLCR) which is very common in patients >70 years. Of course, there are variant MM conditions like low secreting MM and non-secreting MM where you see a small or no M spike at all. Those are usually diagnosed if the patient has a bony lesion, anemia (hb <10) which cannot be attributed to another condition, or high plasma cell count on bone marrow biopsy. That does not seem to be the case with your patient. 2) Light chain disease may not have an M spike in the serum as most of the light chains are excreted out in the urine. However, they will have an abnormal serum FLCR. A UPEP can be done, however, I doubt it will show a monoclonal component. 3) For diagnosing AL amyloid you need to have an M-spike. Usually in AL amyloid patients with kidney involvement have nephrotic range proteinuria, not the case with this patient. 4) In your patient the absolute number of FLC is high which can happen due to any infection/inflammation. The ratio is important, not the absolute number. 5) B2M is a non-specific marker. It can be raised in CKD, inflammation. Not used to diagnosed PCD, only used in staging. What I would do- Find another cause of patient's complaints. Just for surety repeat SPEP, FLCR, and UPEP in 2 months.

@Dr. Rajendra Pandey Respected sir, I am extremely honoured and thankful to you for your reply , you taught me and presented your own views at the same time. Its absolutely wonderful. Thank you very very much sir. I would do as you suggested. I read on beta 2 microglobulin in a little more detail last night and I came to similar conclusion of cod, inflammation , specially glomerular disease as beta 2.microglobulin is filtered by glomerulus but reabsorbed by tubules, so in glomerular diseases it's serum level should be high and in tubular it should be low in serum and more in protein. There is lots and lots for me to learn understand and comprehend. the question of rising creat and thus falling gfr comes. How should one approach this? Sir, I would also like to know if in your clinical judgment if my approach to this presentation was right or should I have gone any other wah around?
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I sincerely appreciate.. Masterly disussion of dr. Pandey. Thanks sir

Sir. Is there any role of skeletal survey And Bone marrow study?
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