A disorder of plasma cells (special white blood cells that produce antibodies), this is one of a group of diseases (called amyloidosis) in which protein deposits (amyloid) accumulate in one or more organ systems in the body. Primary amyloidosis is the most common type of amyloidosis in many countries including the U.S.
The protein deposits in primary amyloidosis are made up of immunoglobulin light chain proteins. These light chain proteins are created in the bone marrow by malfunctioning plasma cells. Primary amyloidosis occurs by itself (in isolated form) and is not associated with any other disease.
The organs most often involved in primary amyloidosis include the heart, kidneys, nervous system, and gastrointestinal tract. Amyloid deposits in these organs can cause shortness of breath, fatigue, edema (swelling of ankles and legs), dizziness upon standing, a feeling of fullness in the stomach (especially after eating), diarrhea, weight loss, enlarged tongue, and numbness of the legs and arms. In the laboratory, a great excess of protein is found in the urine.
Primary amyloidosis is usually treated with chemotherapy. The drug melphalan in pill form has for years been the key chemotherapeutic agent for primary amyloidosis. More aggressive treatment is under study with intravenous melphalan plus return of the patient’s own bone marrow stem cells. Stem cells are the “parent” cells formed in the bone marrow from which all blood cells develop. This approach aims to eliminate the malfunctioning plasma cells which make the amyloid light chains and to re-create a wholly healthy bone marrow.
- Primary atelectasis
Failure of the lung to expand fully at birth. In contrast to secondary atelectasis in which there is partial or complete collapse of a lung that once had expanded, as may happen after chest surgery.
- Primary biliary cirrhosis (PBC)
A liver disease caused by an abnormality of the immune system. Small bile ducts within the liver become inflamed and obliterated. Backup of bile causes intense skin itching and yellowing of the skin (jaundice). Lack of bile decreases absorption of calcium and vitamin D, leading to osteoporosis. Cirrhosis (scarring of the liver) develops over time.
- Primary care
A patient’s main source for regular medical care, ideally providing continuity and integration of health care services. All family physicians, and many pediatricians and internists, practice primary care. The aims of primary care are to provide the patient with a broad spectrum of preventive and curative care over a period of time and to coordinate […]
- Primary care provider
In insurance parlance, a physician who is chosen by or assigned to a patient and both provides primary care and acts as a gatekeeper to control access to other medical services.
After mealtime. A postprandial rise in the blood glucose level is one that occurs after eating.