Alpha-heavy-chain disease

Alpha-heavy chain disease Description, Causes and Risk Factors: Alpha-HCD. Alpha-heavy chain disease  is a type of heavy-chain disease characterized by the production of incomplete monoclonal alpha-heavy chains without associated light chains. Alpha-HCD is considered to be a subtype of immunoproliferative small intestinal disease. The clinical presentation includes chronic diarrhea with evidence of malabsorption. The prevalence of the disease is unknown but most cases have been from North African and Middle Eastern/Mediterranean countries and have been associated with poor sanitation. There have been more than 400 cases reported in the world literature. Alpha-heavy chain disease has a predilection for young age groups (20-30 years). Patients present with symptoms of malabsorption. Diarrhea, weight loss and abdominal pain are common. Parasitic infections are often present. Infiltration of the jejunal mucosa with plasmacytoid cells is the most frequent pathologic feature. Immunoblastic lymphoma occurs as alpha-heavy chain disease progresses. The exact cause of alpha-heavy chain disease is unknown. The lymphoplasmacytic infiltration of the intestinal mucosa is felt to be a response of the alimentary tract immune system to prolonged luminal antigenic stimulation by intestinal organisms. In most cases, no risk factors are defined. Symptoms: • Chronic diarrhea. • Weight loss. • Malabsorption. • Extensive mesenteric and paraaortic adenopathy. Diagnosis alpha-heavy chain disease: Demonstrating alpha heavy chains is difficult because the alpha chains tend to polymerize and appear as a smear instead of a sharp peak on electrophoretic profiles. The diagnosis of alpha-HCD is based on identification of free alpha-heavy chains without associated light chains. Truncated alpha-heavy chains can be detected in biological fluids (serum, urine, jejunal secretions) by immunoelectrophoresis, immunoselection, or immunofixation. Alpha-HCD disease must be differentiated from non-Hodgkin lymphoma (NHL; see this term), although this is an uncommon diagnosis in the age range typical of alpha-HCD. Other causes of small bowel malabsorption need to be considered, especially celiac disease (see this term). Initial treatment consists of eradication of any concurrent infection (eg, parasites, viruses, Helicobacter pylori, Campylobacter jejuni) with appropriate antibiotics. For patients with symptomatic disease not responding adequately to antibiotics, chemotherapy similar to that used to treat NHL is recommended. Surgical resection is sometimes needed when bulky masses are present. The disease course of alpha-HCD is variable and long-term prognosis of the disease is imprecise. Without antibiotics and chemotherapy the disease progresses rapidly and prognosis is poor.Alpha-heavy chain disease Diagnosis is confirmed by: • Immunofluorescence staining of biopsy tissue with anti-heavy-chain antibodies. • Immunoelectrophoresis or immunofixation of free monoclonal heavy-chain fragments. Treatment: Initial treatment consists of eradication of any concurrent infection eg, parasites, viruses, Helicobacter pylori, Campylobacter jejuni (a thermophilic bacterial species that causes in humans an acute gastroenteritis of sudden onset with constitutional symptoms (malaise, myalgia, arthralgia, and headache) and cramping abdominal pain; it has been associated with a demyelinating sequela, which can present with ascending paralysis. Potential sources of human infection include poultry, cattle, sheep, pigs, and dogs. This species also causes abortion in sheep) with appropriate antibiotics. For patients with symptomatic disease not responding adequately to antibiotics, chemotherapy similar to that used to treat NHL is recommended. Surgical resection is sometimes needed when bulky masses are present. The disease course of alpha-HCD is variable and long-term prognosis of the disease is imprecise. Without antibiotics and chemotherapy the disease progresses rapidly and prognosis is poor. NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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