Systemic amyloidosis

Hereditary transthyretin amyloidosis is an uncommon multisystem disorder caused by mutation of the transthyretin protein, leading to peripheral neuropathy often with autonomic features, cardiomyopathy, or a mixed phenotype. Multiple other organ systems can be involved with ophthalmologic, renal, hematologic, gastrointestinal, and/or genitourinary symptoms and signs.

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Systemic amyloidosis is group of disorders characterized by the accumulation of insoluble proteins in tissues. The most common form of systemic amyloidosis is light chain amyloidosis, which results from the accumulation of misfolded immunoglobulins.

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Transthyretin and immunoglobulin light-chain amyloidoses cause amyloid deposition throughout various organ systems. Recent evidence suggests that soft tissue amyloid deposits may lead to orthopedic conditions before cardiac manifestations occur.

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Pulmonary is an uncommon site of extramedullary involvement in multiple myeloma (MM). Diffuse parenchymal amyloidosis as pulmonary manifestation of MM is even rarer. We report a rare case of diffuse parenchymal pulmonary amyloidosis associated with MM diagnosed by video-assisted thoracoscopic lung biopsy (VATLB).

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part 2 book “handbook of hematologic malignancies” has contents: multiple myeloma, hodgkin lymphoma, immunoglobulin light chain amyloidosis, burkitt lymphoma, double hit lymphoma, mantle cell lymphoma, primary central nervous system lymphoma, follicular lymphoma, marginal zone lymphoma,… and other contents.

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(bq) part 2 book "lasers in dermatological practice" presentation of content: sexually transmitted diseases, keratinising and papulosquamous disorders, connective tissue disorders, bullous disorders, sarcoidosis, amyloidosis, diseases of blood vessels and lymphatic system, diseases of pigmentation,...

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It is important that cord blood transplantation is one of the risk factors of symptomatic adenoviremia (Robin et al., 2007), which develops to acute renal failure in the terminal stage (Abe T et al., 2009). At present, there is no established consensus about the treatment for acute renal failure induced by adenovirus after stem cell transplantation. This chapter focuses on the recent advances in diagnosis, mostly due to the development of molecular methods, and therapeutic interventions.

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Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Successful management of refractory pleural effusion due to systemic immunoglobulin light chain amyloidosis by vincristine adriamycin dexamethasone chemotherapy: a case report

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Underlying Systemic Diseases that Cause or Exacerbate a Bleeding Tendency Acquired bleeding disorders are commonly secondary to, or associated with, systemic disease. The clinical evaluation of a patient with a bleeding tendency must therefore include a thorough assessment for evidence of underlying disease. Bruising or mucosal bleeding may be the presenting complaint in liver disease, severe renal impairment, hypothyroidism, paraproteinemias or amyloidosis, and conditions causing bone marrow failure.

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Systemic causes of nonpalpable purpura fall into several categories, and those secondary to clotting disturbances and vascular fragility will be discussed first. The former group includes thrombocytopenia (Chap. 109), abnormal platelet function as is seen in uremia, and clotting factor defects. The initial site of presentation for thrombocytopenia-induced petechiae is the distal lower extremity.

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Pink Lesions The cutaneous lesions associated with primary systemic amyloidosis are often pink in color and translucent. Common locations are the face, especially the periorbital and perioral regions, and flexural areas. On biopsy, homogeneous deposits of amyloid are seen in the dermis and in the walls of blood vessels; the latter lead to an increase in vessel wall fragility. As a result, petechiae and purpura develop in clinically normal skin as well as in lesional skin following minor trauma, hence the term pinch purpura.

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Disordered gut sensorimotor function also commonly causes nausea and vomiting. Gastroparesis is defined as a delay in emptying of food from the stomach and occurs after vagotomy, with pancreatic adenocarcinoma, with mesenteric vascular insufficiency, or in systemic diseases such as diabetes, scleroderma, and amyloidosis. Idiopathic gastroparesis occurring in the absence of systemic illness may follow a viral prodrome, suggesting an infectious etiology.

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