Blackout at Rest and Slurring in a Man Afraid of COVID-19

Ankit Raiyani, MBBS, MD, DNB (Hematology)

Disclosures

March 03, 2021

Further studies were ordered for the patient in this case because of the high clinical suspicion of amyloidosis. A serum free light chain assay showed these results:

  • Kappa free light chains: 6.3 mg/L (reference range, 3.3-19.4 mg/L)

  • Lambda free light chains: 1360 mg/L (reference range, 5.71-26.3 mg/L)

  • Ratio of kappa/lambda: 0.0046 (reference range, 0.26-1.65)

Serum immunofixation detected a lambda light chain monoclonal band. Bone marrow examination revealed no evidence of a plasma cell disorder. The C-reactive protein level was normal, and the level of rheumatoid factor was within normal limits. Renal biopsy results were suggestive of deposition of amyloid material. On the basis of these findings, AL amyloidosis, also known as systemic light chain amyloidosis, was diagnosed.

The prognosis of the disease depends on the extent of cardiac involvement. Cardiac arrhythmias and cardiac failure are the most frequently implicated causes of decreased survival of patients with AL amyloidosis. Patients with extensive cardiac involvement have uniformly poor survival (9-15 months), even with the best available treatment.[8]

At present, no therapy directed toward elimination of the amyloid deposits is available. Hence, treatment of the disease addresses the underlying clonal plasma cell dyscrasia, with the aim of rapidly reducing production of amyloidogenic light chains to limit progressive damage to amyloidotic organs. Combination therapy regimens used for multiple myeloma are also effective in AL amyloidosis. There is no established standard combination. Bortezomib (a proteasome inhibitor) is the base drug in combination therapy and may be combined with dexamethasone (a corticosteroid), daratumumab, and/or other drugs.[9,10]

Autologous stem cell transplantation is the most effective treatment but is not tolerated in patients with major cardiac involvement. Only about 20% of patients are eligible. Eligible patients should undergo high-dose therapy with melphalan followed by autologous stem cell transplant to further enhance the response to treatment.[11,12] Unlike in multiple myeloma, prolonged maintenance therapy confers no advantage in primary amyloidosis.[13]

Many new molecules that target the amyloid deposits and interfere with amyloidogenesis are in trials. Some of the promising molecules that may improve the outcomes of patients with advanced disease include:

  • 4′-Iodo-4′-deoxydoxorubicin

  • Doxycycline, the antibiotic[14]

  • Epigallocatechin gallate, a polyphenol from green tea[15]

  • CPHPC, a competitive inhibitor of amyloid P[16]

  • NEOD001, a monoclonal antibody against amyloid P[17]

The patient in this case underwent implantation of a cardioverter defibrillator to decrease the risk of sudden cardiac death from arrhythmia. He received four cycles of combination therapy, with significant improvement in quality of life. He had extensive cardiac involvement, so he was ineligible for high-dose melphalan therapy followed by autologous stem cell transplant. He is currently undergoing regular follow-up.

Comments

3090D553-9492-4563-8681-AD288FA52ACE
Comments on Medscape are moderated and should be professional in tone and on topic. You must declare any conflicts of interest related to your comments and responses. Please see our Commenting Guide for further information. We reserve the right to remove posts at our sole discretion.
Post as:

processing....