LIGHT CHAIN DISEASE B. DHANALAKSHMI 1 & V. HEMAVATHY 2

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1 TJPRC: International Journal of Nursing and Patient Safety & Care (TJPRC: IJNPSC) Vol. 1, Issue 1, Dec 2016, TJPRC Pvt. Ltd. LIGHT CHAIN DISEASE B. DHANALAKSHMI 1 & V. HEMAVATHY 2 1 Associate Professor, Sree Balaji College of Nursing, Chromepet, Chennai, Tamil Nadu, India 2 Principal, Sree Balaji College of Nursing, Chromepet, Chennai, Tamil Nadu, India ABSTRACT Light chain deposition disease (LCDD) is a rare blood cell disease which is characterized by deposition of fragments of infection-fighting immunoglobulins, called light chains (LCs), in the body. LCs are normally cleared by the kidneys, but in LCDD, these light chain deposits damage organs and cause disease. The kidneys are almost always affected and this often leads to kidney failure. About half of people with light chain deposition disease also have multiple myeloma. Unlike in AL amyloidosis, in which light chains are laid down in characteristic amyloid deposits, in LCDD, light chains are deposited in non-amyloid granules KEYWORDS: Light Chain Deposition Disease, Multiple Myeloma, Bortezomib, Thalidomide and Lenalidomide INTRODUCTION Immune system produces antibodies, or immunoglobulins, to identify and eliminate foreign invaders such as bacteria and viruses, also called antigens. The immunoglobulins have the shape of a Y and are typically made of two heavy and two light chains. The body of the Y is made by the two heavy chains and the two light chains attach left and right to the Y s arms. Together, the two pairs of chains produce a specific shape at each tip of the Y that is designed to capture a specific antigen. The antigen s shape fits the shape of the antibody, similar to how a key fits into a lock. Patho Physiology Deposition of extracellular matrix proteins Mixed with Kappa light chains Mesangial nodularity within the glomerulus Nodular Sclerosing Glomerualr Nephiritis Present Predominantly along the loops of the Henle, Distal tubules and proximal tubules. Definition Is a rare blood cell disease which is characterized by deposition of fragments of infection fighting immunoglobulins called light chains (LCs), in the body. LCs are normally cleared by the kidneys, but in LCDD, these light chain deposits damage organs and cause disease. The kidneys are almost always affected and this often leads to kidney failure. About half of people with light chain deposition disease also have multiple myeloma in amyloid deposits, in light

2 22 B. Dhanalakshmi & V. Hemavathy chains are deposite in non-amyloid granules. Signs and Symptoms Heart: Enlargement, congestive heart failure and irregular heartbeat. Lungs: Damage the lung parenchyma. Involvement of the large airways has recently been reported. Nodular and diffuse pulmonary interstitial diseases of the lungs. Liver: Deposits in the liver usually cause no symptoms. Liver failure in rare cases has been reported. Kidney: Nephrotic syndrome with proteinuria, low levels of blood serum protein, high levels of blood lipids and swelling or edema. Peripheral Neurophathy: Pain, Loss of Sensation or an inability to control muscles can develop if the nervous system is involved, and if light chains are deposited in the skin. Skin: Lesions can appear. Mortality / Morbidity The most common cause of mortality and morbidity in light-chain deposit in disease (LCDD) is related to renal complications, including hypertension, nephrotic syndrome, and progression to end-stage renal disease (ESRD). Liver dysfunction can also occur, with progression to hepatic failure. Other symptoms of LCDD relate to congestive heart failure, peripheral neuropathy, and skin lesions secondary to the deposition of light chains. Diagnostic Approach Immunohistologic analysis of tissue from an affected organ, light or heavy chain is monoclonal. Echocardiogram. Abdominal ultrasound to assess liver, spleen, and lymph nodes. Bone marrow aspirate and biopsy should be performed to rule out the presence of MM and/or Light amyloidosis. Troponin-I or T and brain natriuretic peptide (BNP) Nerve studies and CT, MRI, or PET Cardiac biopsy to rule out other possibilities, or assessment (pretransplant). Treatment Autologous stem cell transplantation (ASCT) Bortezomib Immunomodulatory drugs Renal transplantation Summary Light chain deposition disease (LCDD) is a monoclonal gammopathy characterized by nonamyloid deposition of

3 Light Chain Disease 23 immunoglobulin light chains in various organs. Most cases present with renal dysfunction, a ubiquitous feature of this disease, and in some instances, it may progress to end-stage renal disease. Unfortunately, until now, no standard treatment has been established. The use of alkylating agents and steroids has been extensively reported. However, conventional chemotherapy response is generally limited with minor effects on kidney function. The use of novel agents such as bortezomib has shown a more rapid response with a dramatically important reduction of light chains in serum and/or urine in small series of cases. Furthermore, autologous stem cell transplantation has been reported as a feasible strategy in LCDD, able to prolong the dialysis-free survival. Nonetheless, toxicity from these therapies should be considered carefully because most of patients might present with kidney dysfunction REFERENCES 1. "Treatment of light chain deposition disease with bortezomiban dexamethasone". Haematologica 94 (2): Randall R.E., Williamson W.C. Jr, Mullinax F., Tung M.Y. & Still W.J. (1976) Manifestations of systemic light chain deposition. The American Journal of Medicine 60, Rostagno A., Frizzera G., Ylagan L., Kumar A., Ghiso J. & Gallo G. (2002) Tumoral non-amyloidotic monoclonal immunoglobulin light chain deposits British Journal of Haematology 119, Croitoru A.G., Hytiroglou P., Schwartz M.E. & Saxena R. (2006) Liver transplantation for liver rupture due to light chain deposition disease: a case report. 26, abbian F., Stabellini N., Sartori S., TombesiP., Aleotti A., Bergami M., Uggeri S., GaldA., Molino C. & Catizone L. (2007) Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report. Journal of Medical Case Reports Gallo G., Goni F., Boctor F., Vidal R., KumarA., Stevens F.J., Frangione B. & GhisoJ. (1996) Light chain cardiomyopathy. Structural analysis of the lightchain tissue deposits. The American Journal of Pathology 148,

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