What is Disease Heavy Alpha Chains?
The disease of heavy alpha chains is the most common disease among heavy chain diseases. Usually found in people younger than 50 years (usually between 10 and 30 years). A marked increase in the lymph nodes of the mesentery. Infiltration of the intestinal mucosa with lymphocytes and plasma cells and atrophy of the villi of the intestinal mucosa lead to chronic diarrhea and absorption disorder syndrome. In some patients, chemotherapy is effective according to the regimens used for lymphomas, in others – antimicrobial therapy. This disease is closely associated with Mediterranean lymphoma – a tumor that affects young people in parts of the world where intestinal parasites are common (Mediterranean, Asia, South America).
Pathogenesis during Disease Heavy Alpha Chains
The infiltration of the lamina propria of the small intestinal mucosa by lymphoplasmacytoid cells secreting abnormal alpha chains is characteristic.
The characteristic peak on an electrophoregram is usually blurred due to the propensity of paraprotein to polymerize. Despite this property of paraprotein, the syndrome of increased blood viscosity is rare (it is likely that dimerization of IgA molecules, which requires J-chains, is necessary for this).
Light chains in the blood and urine are missing.
Symptoms Diseases Heavy Alpha Chains
The disease is manifested by chronic diarrhea, anemia, weight loss, malabsorption, hepatosplenomegaly, weakness, indisposition, fever and a pronounced increase in the mesenteric and para-aortic lymph nodes. Occasionally airways are affected.
The course of the disease is very diverse. In some patients, the tumor is transformed into diffuse large B-cell lymphoma (lymphoma of a high degree of malignancy).
A typical symptom of the disease – swelling of the soft palate (due to lesions of the lymphatic pharyngeal ring), up to the development of respiratory failure.
The condition of patients usually deteriorates rapidly, death occurs from infections.
Diagnosis Diseases of Heavy Alpha Chains
The diagnosis is based on the detection of serum paraprotein, which reacts with antibodies to IgG, but not with antibodies to light chains (the level of paraprotein is often below 20 g / l). Paraprotein is usually present in both blood and urine. It most often represents heavy gamma1 chains. Thrombocytopenia and eosinophilia are possible; plasma cell bone marrow infiltration is usually absent.
Treatment Diseases of Heavy Alpha Chains
With chemotherapy, long-term remission can be achieved. Antimicrobial therapy helps individual patients, suggesting the etiological role of chronic intestinal infection and the antigen stimulation caused by it. Combining chemotherapy with antimicrobial agents seems to be more effective than single chemotherapy.
Some patients receiving chemotherapy live 5 years or longer.