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The lower legs and feet of a 23-year-old woman with Waldmann’s since infancy. Waldmann disease, also known as Primary Intestinal Lymphangiectasia (PIL), is a rare disease [1] characterized by enlargement of the lymph vessels supplying the lamina propria of the small intestine. [2]
When it occurs in the intestines it is known as intestinal lymphangiectasia, colloquially recognized as Waldmann's disease in cases where there is no secondary cause. [3] The primary defect lies in the inability of the lymphatic system to adequately drain lymph, resulting in its subsequent accumulation and leakage into the intestinal lumen. [ 3 ]
The disease was renamed 'pantothenate kinase-associated neurodegeneration' or PKAN by Zhou et al. (2001) [3] who suggested the name to avoid misinterpretation and to better reflect the true nature of the disorder. Most recently Pellecchia et al. (2005) published a report of 16 patients affected by PKAN, confirmed by genetic analysis.
A Mayo Clinic study of 124 patients initially diagnosed as having non-secretory multiple myeloma were later found to be composed of 65% free light chain secretors and 35% true non-secretors. As a group, these patients response to therapy, time to disease recurrence, and overall survival were similar to typical myeloma patients.
Five-year survival rates can be used to compare the effectiveness of treatments. Use of five-year survival statistics is more useful in aggressive diseases that have a shorter life expectancy following diagnosis, such as lung cancer, and less useful in cases with a long life expectancy, such as prostate cancer.
An example of a Kaplan–Meier plot for two conditions associated with patient survival. The Kaplan–Meier estimator, [1] [2] also known as the product limit estimator, is a non-parametric statistic used to estimate the survival function from lifetime data.
As reported by Dispenzieri et al. [4] Mayo Clinic treatment regimens are tailored to treat the clinical manifestations and prognosis for the rate of progression of the POEMS syndrome in each patient. In rare cases, patients may have minimal or no symptoms at presentation or after successful treatment of their disorder.
The revised five-factor score is associated with five-year mortality from GPA and is based on these criteria: Age greater than 65 years, cardiac symptoms, gastrointestinal involvement, chronic kidney disease, and the absence of ear, nose, and throat symptoms. [7] With corticosteroids and cyclophosphamide, five-year survival is over 80%. [13]