2022 Clinical problem solving pre conference asynchronous learning around Multiple Myeloma
Link to conference here
Medicine PG(Narketpally) introduced herself to Hematoncology expert (Wisconsin) and shared a case report of a patient in our hospital where the diagnosis of multiple myeloma was made thanks to the patient presenting with Pulmonary Tuberculosis.
January 4th 2022 evening
Professor and HOD of medicine(Narketpally) shared conference details and informed Hematoncology expert that the Medicine PG would be making all futher communications regarding the conference and diagnostic and treatment uncertainties surrounding our patient.
January 8th 2022 afternoon
Medicine PG shared a case report click here with Hematoncology expert and asked the following questions pertaining to it:
1. What was the etiology of Multiple Myeloma in this patient?
2. Why did the patient, despite being on immunomodulators and hemodialysis for cast nephropathy only live uptil 4 months?
3. What can be done for early diagnosis of Multiple Myeloma so as to improve prognosis?
The Medicine PG further shared 2 case reports of previously admitted patients where Multiple Myeloma was seen along with pericardial effusion and joint pains
January 8th and 9th 2022
Professor and HOD of medicine and Hematoncology expert raise concern over the lack of a clear mention about the treatment modalities used for the patients of Multiple Myeloma.
January 15th 2022
Medicine PG shared a case report outlining the treatment given to a patient of Multiple Myeloma simultaneously undergoing chemotherapy since 10 to 12 months
January 16th 2022 morning
Professor and HOD of Medicine highlights to the Hematoncology expert a curious finding in this report which is as follows:
1. Patient developed cutaneous Myeloma
2. This Myeloma responded well to chemotherapy cycles but kept recurring between these cycles.
Further he posed the following questions to the expert:
1. What would the treatment be in a more advanced setup as is present with the Hematoncology expert
2. How does he overcome the disappointments over poor prognosis of patients so as to emerge from therapeutic nihilism.
January 16th 2022 evening
Hematoncology expert reviewed the case and reverted back saying that the lesions aren't cutaneous Myeloma but are actually Amyloid deposits subtype AL. He further suggested that based on this finding patient could be a case of advanced (stage IV) cardiac amyloidosis.
Furthermore, the expert suggested that along with chemotherapy an aggressive management of heart failure is needed.
He also acknowledged that prognosis for the case is very poor.
January 16th 2022 evening
Professor and HOD of Medicine reverted back saying that they would review the patient's cardiac status and update him with the markers as well as an ECHO video
Further he asked the expert's opinion on the chemotherapy already received by the patient and what chemotherapeutic option would be best suited for further treatment.
January 16th 2022 later in the evening
Hematoncology expert reverted back saying that the chemotherapy currently being offered is a standard practice even in advanced setups like in the USA.
He said that daratumumab can be added to cyclophosphamide and bortezomib. Thus if daratumumab could be added to current regimen being followed it would be great.
Further, he said that autologous transplantation along with Melphalan is done for eligible patients but our patient won't be eligible due to her poor cardiac status.
January 16th 2022 later in the evening
Professor and HOD of Medicine asked if patient outcome would be as dismal as it is currently even in advanced setups, he further asked how budding doctors are expected to deal with such disappointing outcomes in diseases with poor prognosis like our present case.
To this the Hematoncology expert reverted back saying that adding daratumumab to the regimen was our best bet for now. He further acknowledged how disappointing it can be while working on such cases and mentioned that some patients die while undergoing work up for the diagnosis of such conditions.
He ended the conversation on a positive note talking about recent advances in therapy targeting Amyloid fibrils which seems promising.
To this Professor and HOD of Medicine affirmed that they would try their best to acquire daratumumab for treatment.
January 18th 2022
Bringing the discussion back to our patient with Myeloma undergoing chemotherapy, The Professor and HOD of Medicine made the following observations about the case:
1. Constrictive pericarditis is seen in analysis of ECHO. A case report was shared similar to this case. link to report
2. Skin lesions appeared to be related to her thrombocytopenic purpura and have currently subsided.
3. Patient developed severe acute renal failure (non oliguric) for the first time and her admission creatinine that was 8 is currently declining gradually at 6.
Further he questioned the reason behind her current presentation of renal failure and thrombocytopenic purpura.
January 24th 2022
Medicine PG reverted back to the above questions with the following answer:
The renal failure in any patient of MM could be Immunoglobulin (Ig) mediated Renal injury ,Non Immunoglobulin (Ig) mediated Renal injury like sepsis, volume depletion, or GLomerulo nephritis
The etiology of Renal failure in our patient is due to Immunoglobulin (Ig) mediated Renal injury which could be either Cast Nephropathy, Monoclonal Immunoglobulin deposition disease (MIDD), or Amyloid Ligh chain deposition(AL)
It is more likely AL or MIDD because of clinically she had purpura , melena(suggesting GI bleed) and Cardiac amyloidosis (Sympotms of heartfailure and echo wise demonstrated heart failure)
And death of her was also attributed secondary to AV blocks causing sudden cardiac arrest.
Evaluation of them is done by proteinuria and albuminuria measurement
Where there is only proteinuria in cast nephropathy as Glomerular basemnent membrane (GBM)is intact
And proteinuria and albuminuria will be there in MIDD and AL as the GBM is destroyed
Ultimately it is the kidney biopsy which gives us the final diagnosis of cause of renal failure.
Though kidney biopsy is the diagnostic test to confirm the association between a monoclonal protein and kidney disease, it can be deferred in the following situations:
A presumptive diagnosis of the light chain cast nephropathy can be made when MM presents with AKI or subacute kidney injury with an SFLC concentration greater than or equal to 1500 mg/L, the predominance of monoclonal light chains in the urine by urine protein electrophoresis and immunofixation.
A presumptive diagnosis of renal amyloidosis can be made in patients with albuminuria or nephrotic syndrome with an established diagnosis of immunoglobulin light chain AL based on biopsies of non-kidney tissues.
Furthermore she said that as they are considering Cardiac amyloidosis her kindey innurh was also secondary to AL.
Comments
Post a Comment