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Pulmonary function: increased residual volume, increased total lung capacity, fixed obstruction, low diffusing capacity of the lung for carbon monoxide that corrects with alveolar volume High-resolution CT scan: diffuse pulmonary nodules 4–10 mm, greater than 20 nodules, mosaic attenuation or air trapping in greater than 50% of the lung
AL amyloidosis is caused by the deposition of abnormal antibody free light chains. The abnormal light chains are produced by monoclonal plasma cells, and, although AL amyloidosis can occur without diagnosis of another disorder, it is often associated with other plasma cell disorders, such as multiple myeloma and Waldenström's macroglobulinemia. [6]
Because of the prolonged survival, which was typically about 10 years in past decades, but which can extend to a normal life expectancy, the prevalence (number of people living with the disease) is much higher than the incidence (new diagnoses). [2] CLL is the most common type of leukemia in the UK, accounting for 38% of all leukemia cases.
In a Mayo Clinic study, for example, AL amyloidosis assigned stage 1, 2, 3, or 4 based on the presence of 0, 1, 2, or 3 prognostic signs (high blood levels of [cardiac troponin T]), blood levels of a marker for congestive heart failure (viz., NT-ProBNP), or free light chain ratios) had median survivals of 94.1, 40.3, 14, and 5.8 months ...
The median survival in idiopathic pulmonary fibrosis is 3-3.5 years. In those who receive a lung transplant, the medial survival in idiopathic pulmonary fibrosis is 5.2 years, as compared to 6.7 years in those with other types of ILD. [8] ILD is associated with a 3-fold increased risk of lung cancer. [8]
Castleman's disease (CD) is a rare heterogeneous lymph node disorder characterized by elevated interleukin-6 levels. Neuropathy can occur in CD patients with or without POEMS syndrome. [ 11 ] CD and POEMS tend to overlap, with roughly 15–24% of POEMS syndrome patients also having CD, with the majority having hyaline vascular type.
Breast, lung, stomach, pancreas, and prostate cancers are the most common tumors that result in lymphangitis. Lymphangitis carcinomatosa was first described by pathologist Gabriel Andral in 1829 in a patient with uterine cancer. Lymphangitis carcinomatosa may show the presence of Kerley B lines on chest X-ray.
Common variable immunodeficiency (CVID) is an inborn immune disorder characterized by recurrent infections and low antibody levels, specifically in immunoglobulin (Ig) types IgG, IgM, and IgA. [2] Symptoms generally include high susceptibility to pathogens, chronic lung disease, as well as inflammation and infection of the gastrointestinal ...