Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Amyloidosis is defined by tissue deposits of amyloid, a proteic substance with a characteristic spatial structure of beta-sheet fibrils assembled into bundles. This structure results in specific staining with Congo red dye, with green birefringence under polarized light microscope. AL amyloid forms from amyloidogenic immunoglobulins produced by clonal plasma cells. Pulmonary amyloidosis may be either part of systemic amyloidosis (primary amyloidosis or associated with myeloma) or organ-limited. Pulmonary interstitial amyloidosis in systemic amyloidosis is rarely symptomatic unless amyloid deposits severely affect gas exchange alveolar structures, thus resulting in serious respiratory impairment. Localized parenchymal involvement may present as nodular amyloidosis or as amyloid deposits in the vicinity of pulmonary lymphomas. Tracheobronchial amyloidosis, which is not associated with evident clonal proliferation, results in airway stenoses necessitating iterative laser treatment. Treatment of systemic amyloidosis aims at reducing the clonal cell populations producing amyloidogenic immunoglobulins, using high-dose chemotherapy followed by autologous stem cell transplantation in carefully selected patients. Its efficiency in treating diffuse pulmonary amyloidosis has not been established.
 ...
PMID:Pulmonary amyloidosis in hematological disorders. 1626 1

Pulmonary amyloidosis is a rather rare complication of multiple myeloma particularily the rare Ig D myeloma. It is often generalized and is seen in a late stage of the disease. We report a case of an Ig G myeloma complicated of a pulmonary amyloidosis in a 66-year-old man hospitalised for infectious pulmonary disease with a radiologic interstitial syndrome. Discovery of the multiple myeloma and of the amyloidosis was fortuitous.
 ...
PMID:[Association of IGg multiple myeloma with pulmonary amyloidosis: about one case]. 1683 1

Multiple Myeloma is a hematologic malignancy of plasma cell origin. Pleural effusion may develop in the setting of myeloma due to various reasons but is extremely uncommon as a presenting symptom. A 69-year-old Caucasian man presented with pleural effusions of undetermined etiology after extensive work up, and multiple failed pleurodesis. Lung biopsy revealed pulmonary amyloidosis and led to the diagnosis of multiple myeloma. Patient was started on chemotherapy but died within 6 weeks of his diagnosis due to multiorgan failure. Pulmonary amyloidosis should be suspected as a cause of intractable pleural effusions, even in patient who do not have evidence of lung involvement on imaging studies or typical features of multiple myeloma. Pleural effusions due to amyloidosis are often refractory to treatment, and a high index of suspicion is required for early diagnosis and treatment.
 ...
PMID:Bilateral Pleural Effusions due to Pulmonary Amyloidosis as the Presenting Manifestation of Multiple Myeloma. 2234 92

The article presents the case of a 60-year-old asymptomatic woman whose chest X-ray screening showed bilateral pulmonary nodules of uncertain etiology. Initially, the main suspicion concerned multiple pulmonary metastases, but the anatomical pathology examination of two of the surgically removed lung nodules revealed a benign pattern--foreign body granulomatous reaction to cholesterol crystals. Patient follow-up with a repeat computed tomography one year later showed that some pulmonary nodules had slightly increased in number and size, so the diagnosis required re-evaluation. Congo red staining revealed a positive reaction in the amorphous material, pointing to a nodular form of pulmonary amyloidosis. This case attests to the wide range of investigations needed to examine multiple pulmonary nodules and to the great variety of possible diagnoses. Surgical biopsy, alongside histopathological examination and immunohistochemical tests of the lung are critical in establishing a positive diagnosis. Pulmonary amyloidosis requires additional investigations and long-term follow-up of the patient, as this condition is frequently associated with MALT (mucosa-associated lymphoid tissue) lymphoma or multiple myeloma.
 ...
PMID:Nodular pulmonary amyloidosis--rare cause of calcified pulmonary nodules. 2601 53

Amyloidosis is a rare condition in which tissue deposits of inert fibrillar protein result in organ damage and dysfunction. There are several types of amyloid fibrils. Some of the most common forms are AL (amyloid light chain) protein and AA (amyloid-associated) type of amyloid fibril protein. Pulmonary amyloidosis is relatively common but is usually asymptomatic. Thus, the diagnosis may be easily overlooked. A 78-year-old male with a history of multiple myeloma followed by systemic amyloidosis presented with abnormal chest CT showing diffuse interlobular thickening in the whole lung field with bilateral pleural effusion. Bronchoalveolar lavage and transbronchial biopsy were performed. Due to the patient's poor condition and hemorrhage, only one fragment was available from forceps biopsy. Histologically, there was no amyloid deposition in the lung parenchyma; however, some histiocytes showed eosinophilic granular contents which prompted us to perform additional staining. The cytoplasmic material turned to be positive with direct fast scarlet (DFS) staining and AA amyloid immunostaining. Similar macrophages with AA amyloid were also found in the bronchoalveolar fluid. We experienced a case with AA amyloidosis affecting the lung diagnosed by the presence of intracytoplasmic amyloid in alveolar macrophages. The microscopic changes were so subtle that they may be overlooked. Recognition of amyloid deposition in alveolar macrophages may be an important clue to diagnose pulmonary amyloidosis. Such finding is of particular significance in the small-sized specimens, such as biopsies and cytologic smears.
 ...
PMID:A case report of pulmonary amyloidosis recognized by detection of AA amyloid exclusively in alveolar macrophages. 3230 31