Macroamylasemia as a Rare Cause of Hyperamylasemia: A Case Report.

Macroamylasemia is a rare condition characterized by the formation of an amylase-globulin complex that is too large to be readily excreted by the kidneys and leads to elevated serum amylase levels. It is a benign condition lacking severe signs and symptoms that does not require treatment. This paper presents a case of a middle-aged man with unexplained elevated serum amylase levels. Despite an initially elevated triglyceride level, clinical findings, laboratory test results, and radiological findings were not suggestive of pancreatitis. The ratio of renal amylase clearance to creatinine clearance was calculated at <1%, consistent with macroamylasemia. No specific treatment was given, and he was monitored periodically. Nevertheless, macroamylasemia is a diagnostic challenge because of the need to differentiate it from other causes of hyperamylasemia to avoid unnecessary tests and treatments.

Significant elevations of serum amylase caused by macroamylase: Case reports and detection possibilities.

BACKGROUND: The presence of isolated elevated serum amylase levels can be caused by high molecular mass complexes. We describe 13 cases of hyperamylasemia detected in adult patients without clinical symptoms of a pancreatic disorder. Five of them were thoroughly examined using different tools for the detection of macrocomplexes. METHODS: We performed both screening and more advanced methods of macroamylase detection, including polyethylene glycol precipitation, sample storage at 4°C and separation by gel filtration. RESULTS: The presence of macroamylase in the suspected samples was confirmed by the methods described, except for the sample storage at 4°C. In this method, the enzyme activity did not decrease. The polyethylene glycol precipitation activity (% PPA) averaged 89.1% for amylase, whereas the control samples averaged 30.7%. Gel filtration chromatography confirmed an IgA macroamylase peak in three samples and an IgG macroamylase peak in two samples. CONCLUSION: The presence of macroamylase should be suspected whenever the clinical history and condition of the patient do not match the measured enzyme value to avoid diagnostic errors and unnecessary invasive examinations. The presence of macrocomplexes is considered a benign process that may occur in apparently healthy individuals. Cooperation between clinicians and laboratory staff is necessary.

Macroamilasemia en el Hospital Víctor Lazarte Echegaray de Trujillo, Perú: reporte de un caso / Macroamylasemia at the Hospital Víctor Lazarte Echegaray in Trujillo (Peru): Case report

Resumen La macroamilasemia debe sospecharse en todo paciente con aumento catalítico de amilasa α plasmática persistente y sin clínica de dolor abdominal, descartando otras etiologías de patología pancreáticas y extrapancreática. La macroamilasemia se caracteriza por la unión de complejos de amilasa α con inmunoglobulina, más frecuente inmunoglobulina A; pueden presentarse 3 tipos de macroamilasemia. Es importante realizar el diagnóstico diferencial de otras patologías que puedan causar el aumento de la amilasa y así evitar los procedimientos innecesarios. Se presenta el caso de un paciente de 53 años, de sexo femenino, que ingresó a emergencia por clínica de dolor abdominal y hiperamilasemia, que fue diagnosticada inicialmente de pancreatitis aguda.

Abstract Macroamylasemia should be suspected in any patient with a persistent catalytic increase of plasma α-amylase but no other clinical signs of abdominal pain after ruling out other causes of pancreatic and extra-pancreatic disease. The binding of α-amylase complexes with immunoglobulin, most commonly immunoglobulin A, characterizes this condition. Macroamylasemia is classified into three kinds. To prevent unnecessary procedures, it is critical to make a differential diagnosis of other conditions that can cause amylase increase. The present article reports the case of a 53-year-old female patient who was admitted to the emergency room with abdominal pain and hyperamylasemia, who was initially diagnosed with acute pancreatitis.

Simultaneous macroamylasemia and macrolipasemia in a patient with mucosa-associated lymphoid tissue lymphoma.

We report a case of simultaneous macroamylasemia and macrolipasemia complicated with mucosa-associated lymphoid tissue (MALT) lymphoma. A 78-year-old man presented with hyperamylasemia and hyperlipasemia for 2 years and was misdiagnosed with chronic pancreatitis at another hospital. However, his other pancreatic enzymes were normal, his amylase-creatinine clearance ratio was low, and no definite findings of pancreatic disease were evident. Immunological analyses revealed that both amylase and lipase were bound to immunoglobulin (Ig) A-κ, and that serum IgA was high (827.1 mg/dL). He was diagnosed with simultaneous macroamylasemia and macrolipasemia. Since these diseases are associated with malignancy, an additional investigation was performed which revealed the complication of MALT lymphoma, and polymerase chain reaction analysis showed monoclonal immunoglobulin light chain gene rearrangement (κ >> λ). In this case, macroamylasemia and macrolipasemia may have developed due to the formation of macroenzymes resulting from excess IgA-κ secreted by the MALT lymphoma. Simultaneous macroamylasemia and macrolipasemia are very rare and difficult to diagnose and can lead to diagnostic and therapeutic errors. When encountering atypical clinical features associated with hyperamylasemia and hyperlipasemia, the possibility of macroenzymes and underlying diseases such as lymphoproliferative disorders should be considered.

Macroamylasemia in a 4-year-old girl with abdominal pain / 소아과

Macroamylasemia is a benign condition characterized by abnormally large-sized serum amylase; it has been reported to occur in 1-2% of the population. In macroamylasemia, a macromolecular complex consisting of amylase linked to immunoglobulins circulates in the plasma and usually causes hyperamylasemia with low or normal amylasuria. Macroamylasemia is extremely rare in children. We report a case of a 4-year-old girl with abdominal pain and macroamylasemia, who was initially misdiagnosed as having acute pancreatitis. Failure to immediately identify macroamylase as the cause of the unexplained but benign hyperamylasemia can lead to the misdiagnosis of the condition, necessitating costly analyses for ruling out pancreatic disease and unnecessary prescriptions such as fasting and intravenous replacement therapies, as was observed in our patient.

Macroamylasemia in a 4-year-old girl with abdominal pain / 소아과

Macroamylasemia is a benign condition characterized by abnormally large-sized serum amylase; it has been reported to occur in 1-2% of the population. In macroamylasemia, a macromolecular complex consisting of amylase linked to immunoglobulins circulates in the plasma and usually causes hyperamylasemia with low or normal amylasuria. Macroamylasemia is extremely rare in children. We report a case of a 4-year-old girl with abdominal pain and macroamylasemia, who was initially misdiagnosed as having acute pancreatitis. Failure to immediately identify macroamylase as the cause of the unexplained but benign hyperamylasemia can lead to the misdiagnosis of the condition, necessitating costly analyses for ruling out pancreatic disease and unnecessary prescriptions such as fasting and intravenous replacement therapies, as was observed in our patient.

Macroamylasemia as the first manifestation of celiac disease.

Macroamylasemia is a biochemical disorder characterized by an elevated serum amylase activity resulting from the circulation of a macromolecular complex of amylase with a serum component, often an immunoglobulin. The increased molecular weight of this complex prevents the normal renal excretion of the enzyme. A few cases of celiac patients with macroamylasemia have been published in whom the biochemical disorder disappeared after treatment with a gluten-free diet.

Macroamylasemia and macrolipasemia in patient with alcoholic liver cirrhosis / 대한내과학회지

Macroenzymes are high molecular weight complexes formed in the serum by self-polymerization or by association with other proteins. Macroenzymes are filtered with difficulty by normal renal glomeruli. Clinically, it is important to detect macroenzymes, because they frequently interfere with the interpretation of serum enzyme results, and as a result they can cause diagnostic and therapeutic errors. Macroamylasemia and macrolipasemia have been found to occur in apparently healthy humans, as well as in a variety of disease states, including liver disease, diabetes, cancer, malabsorption, and autoimmune disorders. We report a patient with alcoholic liver cirrhosis and macroamylasemia and macrolipasemia, the latter two of which were discovered using a screening test.