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Immunoglobulin (Ig) light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of Ig light chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, peripheral/ autonomic neuropathy. The diagnosis can be challenging, requiring a biopsy and often specialized testing to confirm the subtype of systemic disease. The goal of treatment is eradication of the monoclonal plasma cell population and suppression of the pathologic light chains which can result in organ improvement and extend patient survival. Standard treatment approaches include high dose melphalan (HDM) followed by autologous hematopoietic stem cell transplantation (SCT) or oral melphalan with dexamethasone (MelDex). The use of novel agents (thalidomide, lenalidomide and bortezomib) alone and in combination with steroids and alkylating agents has shown efficacy and continues to be explored.

The heart is an organ often occupied by various forms of amyloidosis; cardiomyopathies are the leading cause of mortality in patients with amyloidosis. Cardiac amyloidosis is often diagnosed late because of nonspecific symptoms and missed early signs in the imaging routine. A method for treating cardiac amyloidosis depends on the type of amyloid protein. In the treatment of amyloidosis associated with immunoglobulins systemic chemotherapy is used without transplant or stem cell transplantation and in the treatment of familial transthyretin amyloidosis liver transplantation is used. There are still clinical studies on the use of siRNA for the treatment of cardiomyopathy associated with transthy retin amyloidosis, and on the use of amyloid protein stabilizers. The prognosis depends on the type of amyloid protein; worse results observed in the case of light chain amyloidosis. Care support is the cornerstone of treatment; it is expected that advances in cardiac imaging and proteomics positive impact on our ability to diagnosis, prognosis and treatment outcomes of amyloidosis of the heart.

Amyloidoses form a group of diseases in which extracellular insoluble fibrils cause organ injury and failure. These aggregates are built of misfolded proteins. Current amyloidosis classification is based on the type of the precursor amyloid protein. Distribution of the amyloid fibrils can be localized or systemic. The two most common systemic diseases are: amyloid light chain (AL) amyloidosis, being a consequence of clonal expansion of plasma cells in the bone marrow, and amyloid A (AA) amyloidosis, secondary to chronic inflammatory disorders. Renal involvement remains a frequent clinical manifestation of both of them with clinical pattern of massive proteinuria and progressive renal failure. Early diagnosis and treatment is essential because of the progressive character of the disease. The goal of current treatment approaches is to decrease the amount of amyloidogenic proteins and depends on its type. Systemic amyloidosis remains a disease with poor prognosis, especially in patients with cardiac involvement.

AL amyloidosis is a systemic disease characterized by extracellular amyloid deposition in tissues, causing progressive dysfunction of affected organs. The main clinical syndroms include nephrotic-range proteinuria with or without renal dysfunction, congestive heart failure, hepatomegaly and peripheral or autonomic neuropathy. The aim of therapy is the reduction of monoclonal light chains, by suppressing the underlying plasma cell dyscrasia. Recent therapeutic strategies involve intermediate-dose chemotherapy or high-dose melphalan supported by peripheral blood stem cell transplantation. Alternative therapeutic approaches include thalidomide, lenalidomide, iododoxorubicin, etanercept and rituximab. This paper reviews the pathogenesis, diagnosis and therapy of the AL amyloidosis, focusing on clinico-morphological symptoms of renal involvement, monitoring of treatment response and supportive therapy.

Introduction: Amyloid is a pathological protein which can accumulate in almost every tissue, consequently resulting in illness (amyloidosis). Amyloid has a red color when the slice preparation is stained with Congo red staining and under polarized light exhibits a characteristic apple-green color. Over 26 different proteins, that can form amyloid, have been described. AL, AA and ATTR amyloidosis are the most frequent type of amyloidosis. Head and neck is a rare region for amyloidosis, which can be localized and/or systemic. Early diagnosis and precise classification of the amyloidosis is essential for treatment planning. Various treatment strategies: antinflammatory management, chemotherapy, immunotherapy, surgery, gene therapy, to name just a few, are already readily available or are currently being explored or researched., Material and Methods: Three cases are presented here: an elderly women with tongue and neck tumor and two patients with laryngeal tumors. The biopsy of the lingual and laryngeal tumor and ultrasonography in the first case have been performed in the ENT-Department and Ambulance., Results: In the Internal Medicine Department the patient with lingual and neck tumor, due to symptoms such as: swallowing disorders, dysartria, painful joints swelling and carpal syndrome in anamnesis, previously underwent an examination in order to rule out scleroderma. The investigation appeared to be negative. The histopathology investigation of the lingual tumor identified amyloidosis. USG has not revealed any significant findings. The two patients with laryngeal tumor, except for hoarseness, has not manifested other symptoms suggesting general illness. The biopsy established that there was localized amyloidosis., Conclusion: Diagnosis of amyloidosis and its correct classification continue to pose a great challenge. Two factors play a pivotal role in the identification of local or systemic amyloidosis: the local and/or systemic manifestation and Congo red staining as a gold diagnostic standard.

The definitive diagnosis of dialysis-related amyloidosis based on histologic findings suffers from invasive surgical procedure and punctual information. Imaging techniques: ultrasonography, X-ray, computed tomography or magnetic resonance are relatively nonspecific and not sensitive enough. Scintigraphy using radio-labelled beta-2-microglobulin currently represents the most specific method to noninvasively diagnosis dialysis-related amyloidosis. Available therapeutic methods include the removal of BMG and palliation of symptoms. Hemodialysis high-flux and hemodiafiltration based on convection are more effective in elimination of BMG than standard hemodialysis with diffusive transport as a dominant mechanism. Use of the adsorption columns Lixelle reduces plasma BMG concentration and leads to clinical improvement of DRA. Medical therapy consists of the use of low dose corticosteroids and non-steroidal anti-inflammatory drugs. Surgical therapy is often necessary in the late stages of DRA. Renal transplantation is still the optimal method of DRA treatment.

Current views on the pathogenesis of reactive amyloidosis are presented. Diagnostic methods used, clinical picture, prognostic factors and methods of treatments are discussed.

Contemporary view on diagnosis and treatment of paraproteinemia is presented dealing particularly with nephrological implications. Treatment of multiple myeloma including new drugs is presented in details. Treatment of cryofibrinogenemia and amyloidosis are also reviewed.

Three cases of amyloidosis were described. In all diagnosis was confirmed by histological examination. There was amyloidosis limited to the lungs in 2 cases and in 1 generalised. In 1 patient lobectomia was performed. Next 2 pts were treated with prednisone and cytostatic drugs (melphalane and cyclophosphamide).

The aim of the study was to summarize the experience in the treatment of chronic renal failure due to secondary amyloidosis in the course of juvenile rheumatoid arthritis. Fourteen children aged 7.5-17.7 years were treated with dialysis; 12 with CAPD, 2 with HD. Our results indicate that CAPD is a proper dialysis technique for children with amyloidosis, despite a high rate of complications in early period of CAPD, such as: bleeding, leaks, hernias, and impaired wound healing.

A case of hepatic and intestinal secondary amyloidosis in 43 years old patient with Hodgkin's disease is presented. Diagnostic difficulties due to atypical localization of Hodgkin's disease were discussed. Therapeutic problems were caused by severe hepatic insufficiency.

A case of amyloidosis is presented in a man aged 21 years, discussing the diagnostic difficulties in immunologically-determined syndromes, and considerable serious consequences of their inappropriate treatment. On the margin of this case important problems are discussed connected with juvenile rheumatoid arthritis, rheumatic fever and amyloidosis.