9 rezultātiem
Light chain deposition disease is a severe complication of certain immunoproliferative disorders, due to the secretion of a monoclonal light chain which precipitates close to basement membranes of several tissues. A kappa isotype restriction and an unusual frequency of a variable region subgroup
We have compared the distribution of lambda-light-chain variable-region (V lambda) subgroups among Ig lambda molecules found in the serum of normal individuals with that of monoclonal Ig lambda components obtained from patients with plasma cell and related immunoproliferative disorders. A panel of
Lymphocyte transformation with phytohaemagglutinin (PHA) was studied in 30 patients with immunoproliferative disorders (lymphoproliferative and plasma cell disorders).Lymphocyte transformation at 3 days was reduced in the lymphoproliferative disorders (chronic lymphocytic leukaemia (CLL),
BACKGROUND
Monoclonal free light chain (LC) proteins are present in the circulation of patients with immunoproliferative disorders such as light chain (AL) amyloidosis and multiple myeloma (MM). Light chain-associated amyloid is a complex pathology composed of proteinaceous fibrils and extracellular
The clinical significance of Bence Jones (BJ) proteinuria at low concentration (less than 0.2 g/24 hours) was investigated in 33 unselected patients who had no intact monoclonal immunoglobulin in their serum. The great majority (79%) of the patients were recognized as having malignant
The natural history of orphan lung diseases is often unclear. We report the long-term follow-up of a case of bronchiectasis due to pulmonary non amyloid light chain deposition disease (LCDD).A 50-year-old woman who was a smoker, was diagnosed with diffuse Light chain deposition disease (LCDD) is a rare disorder that very uncommonly affects the lung. We report three cases of severe cystic pulmonary LCDD leading to lung transplantation. Such a presentation has never been previously reported. The three patients present with a progressive obstructive
Sixty-six consecutive patients exhibiting isolated urinary excretion of monoclonal free light chains, i.e. Bence Jones protein (BJP), on screening investigation for serum and urine monoclonal immunoglobulins were studied in order to better define the spectrum of immunoproliferative disorders
One hundred and fifty-four patients with a monoclonal gammopathy, diagnosed between 1965-1974 in the Hadassah University Hospital are reviewed with special reference to the relative incidence of associated disorders. Most patients (63 per cent) had immunoproliferative disorders (multiple myeoloma,