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Hereditary Angioedema and Pancreatitis
Abstracts S273
J ALLERGY CLIN IMMUNOL VOLUME 119, NUMBER 1
Allergy to Marijuana: Case report S. Perez-Bustamante, M. Vazquez de la torre, A. Villanueva, R. Pelta, M. Rubio, M. Baeza; Hospital General Universitario Gregorio Maran˜on, Madrid, SPAIN. RATIONALE: Allergic reactions to marijuana (Cannabis sativa) is rare. Contact urticaria is the most common presentation. No cutaneous allergy has ever been reported following marijuana inhalation. METHODS: We described a 21-year-old man who referred, in the last two years, several episodes of papular lessions, itching and generalized erythema associated in some cases with palebral angioedema after smoking a considerable amount of marijuana. These episodes dissapeared in hours with antihistamines. He tolerated to smoke 2 cigarettes of marijuana without any symptoms. An extract of marijuana leaves was prepared in phosphate buffer. Prickprick was performed with marijuana leaves and extract. Serum specific marijuana IgE levels were measured by ELISA. The molecular mass of the IgE binding proteins was determinated by SDS-PAGE IgEInmunoblotting. RESULTS: Prick-prick test resulted positive to leaves and extract of marijuana. Specific IgE to marijuana was positive: absorbance OD490: 0,710 kU/L (control: 0.100). The apparent molecular weight of the allergens present in marijuana extract measured 69, 38, 26 and 10 kD. The patient reduced the number of marijuana cigarettes to two and since then he is asymptomatic. CONCLUSIONS: We present a dose-dependent urticarial reaction to marijuana smoke exposure, in a nonatopic patient. Specific IgE mechanism is suggested by a positive prick test and IgE determination.
1068
Hereditary Angioedema and Pancreatitis D. Chung, P. Busse, M. Oh; Mount Sinai Medical Center, New York, NY. RATIONALE: Hereditary angioedema (HAE) is a rare disease in which patients either lack, or have an abnormally functioning C1-esterase inhibitor enzyme. Symptoms typically include relapsing and unpredictable episodes of angioedema in the extremities, gastrointestinal tract, genitourinary, face or upper airways. While abdominal pain secondary to edema is common, pancreatitis is rarely a complication of an acute HAE attack. METHODS: Case report; literature review. RESULTS: A 51 year-old female with HAE (diagnosed >30 years ago, on Danazol for prophylaxis) presented with severe epigastric pain and mild facial swelling. Laboratory evaluation revealed elevated amylase (369 U/L) and lipase (1735 U/L), with normal liver enzymes and function (ALT563, AST530, alkaline phosphatase535, total bilirubin 1.2, albumin53.9). Her white blood cell count was 19.3 without a left shift and she was afebrile (she had been transferred from another hospital where she had been given corticosteroids for her HAE). She denied a history of, or recent alcohol consumption, trauma or use of other medications known to induce pancreatitis. She was treated for acute pancreatitis by withholding food intake, administering intravenous hydration and giving analgesics for pain control. The dose of Danazol was unchanged. After a few days, her symptoms improved and her pancreatic enzymes returned to normal. CONCLUSIONS: Intestinal swelling associated with acute HAE attacks may induce pancreatitis. There are five previously reported cases of pancreatitis associated with HAE exacerbations. Therefore it may be prudent to check serum amylase and lipase in patients with HAE presenting with severe abdominal attacks, as it may affect management.
1069
Hereditary Angioedema: Clinical Aspects in a Brazilian Family M. Ferraro, M. Thiesen, L. Arruda, W. Sarti; School of Medicine of Ribeira˜o Preto-University of Sa˜o Paulo, Ribeira˜o Preto, BRAZIL. RATIONALE: To describe clinical features and treatment outcomes within a six-generation family with hereditary angioedema. METHODS: Members of a family from Minas Gerais, Brazil, with a history of recurrent episodes of facial swelling and attacks of abdominal pain were investigated. Family pedigree was constructed with 44 individuals, and showed 15 affected members (10 women), distributed in six generations. Seven patients were interviewed and examined, and information on eight members (two deceased) was provided by relatives. Twentynine individuals did not present history suggestive of hereditary angioedema. Levels of complement C3 and C4 were obtained by nephelometry, and quantitation of C1 inhibitor was carried out by radial immunodiffusion. Four patients are receiving Danazol. RESULTS: Seven female patients, 6 to 42 years-old, were evaluated. Four patients showed levels of C1 inhibitor of 4.0 to 7.0mg/dl (normal values 1430mg/dl), of whom 3 had low levels of C4. Two girls (6 and 12 years-old) presented normal C1 inhibitor levels and activity, but low C4 levels. One patient had normal levels of C4 and C1 inhibitor on two occasions. A prominent feature among these patients was presence of recurrent attacks of abdominal pain. For those patients with low C1 inhibitor levels, treatment was initiated with Danazol 50mg daily, or 50mg alternate days, followed by excellent clinical response, with no recurrence of symptoms or side effects. CONCLUSIONS: Hereditary angioedema is a genetic entity that can lead to severe complications. Long term prophylaxis with Danazol was shown to be effective at lower doses than those recommended in the literature. Funding: FAPESP
1070
Fresh Frozen Plasma for the Treatment of Hereditary Angioedema M. Prematta, J. Gibbs, T. J. Craig, E. Pratt, T. Stoughton; Penn State University, Hershey, PA. RATIONALE: Fresh frozen plasma (FFP) has been used as a treatment option for patients with Hereditary Angioedema (HAE) because it contains C1-esterase inhibitor and alternate therapies are not yet available in the USA. However, because FFP also contains other substrates, it has been hypothesized to have the potential to worsen or precipitate an acute attack of HAE. It is our aim to research patient records and the medical literature to determine whether FFP can exacerbate symptoms or precipitate an attack of HAE. METHODS: The following search terms were used in PubMed and OVID: ‘‘Hereditary Angioedema’’ and ‘‘Angioedema and Fresh Frozen Plasma.’’ Articles were searched from 1966 to the present. Charts of HAE patients at our institution who have received FFP since 1990 were reviewed to determine if there was any evidence that FFP exacerbated the symptoms of HAE. RESULTS: The literature and our patient chart review failed to identify instances when FFP exacerbated symptoms of HAE or precipitated an attack. Several reports and our experience demonstrate that FFP is an effective prophylactic agent immediately before surgery, during pregnancy, and for treatment of acute attacks of HAE, without evidence of exacerbation or initiation of symptoms. CONCLUSIONS: FFP seems to be safe and effective in preventing exacerbations of HAE before surgery, during pregnancy, and for acute exacerbations of HAE without evidence of initiating an attack or worsening a preexisting attack.
TUESDAY
1067
J ALLERGY CLIN IMMUNOL VOLUME 119, NUMBER 1
Allergy to Marijuana: Case report S. Perez-Bustamante, M. Vazquez de la torre, A. Villanueva, R. Pelta, M. Rubio, M. Baeza; Hospital General Universitario Gregorio Maran˜on, Madrid, SPAIN. RATIONALE: Allergic reactions to marijuana (Cannabis sativa) is rare. Contact urticaria is the most common presentation. No cutaneous allergy has ever been reported following marijuana inhalation. METHODS: We described a 21-year-old man who referred, in the last two years, several episodes of papular lessions, itching and generalized erythema associated in some cases with palebral angioedema after smoking a considerable amount of marijuana. These episodes dissapeared in hours with antihistamines. He tolerated to smoke 2 cigarettes of marijuana without any symptoms. An extract of marijuana leaves was prepared in phosphate buffer. Prickprick was performed with marijuana leaves and extract. Serum specific marijuana IgE levels were measured by ELISA. The molecular mass of the IgE binding proteins was determinated by SDS-PAGE IgEInmunoblotting. RESULTS: Prick-prick test resulted positive to leaves and extract of marijuana. Specific IgE to marijuana was positive: absorbance OD490: 0,710 kU/L (control: 0.100). The apparent molecular weight of the allergens present in marijuana extract measured 69, 38, 26 and 10 kD. The patient reduced the number of marijuana cigarettes to two and since then he is asymptomatic. CONCLUSIONS: We present a dose-dependent urticarial reaction to marijuana smoke exposure, in a nonatopic patient. Specific IgE mechanism is suggested by a positive prick test and IgE determination.
1068
Hereditary Angioedema and Pancreatitis D. Chung, P. Busse, M. Oh; Mount Sinai Medical Center, New York, NY. RATIONALE: Hereditary angioedema (HAE) is a rare disease in which patients either lack, or have an abnormally functioning C1-esterase inhibitor enzyme. Symptoms typically include relapsing and unpredictable episodes of angioedema in the extremities, gastrointestinal tract, genitourinary, face or upper airways. While abdominal pain secondary to edema is common, pancreatitis is rarely a complication of an acute HAE attack. METHODS: Case report; literature review. RESULTS: A 51 year-old female with HAE (diagnosed >30 years ago, on Danazol for prophylaxis) presented with severe epigastric pain and mild facial swelling. Laboratory evaluation revealed elevated amylase (369 U/L) and lipase (1735 U/L), with normal liver enzymes and function (ALT563, AST530, alkaline phosphatase535, total bilirubin 1.2, albumin53.9). Her white blood cell count was 19.3 without a left shift and she was afebrile (she had been transferred from another hospital where she had been given corticosteroids for her HAE). She denied a history of, or recent alcohol consumption, trauma or use of other medications known to induce pancreatitis. She was treated for acute pancreatitis by withholding food intake, administering intravenous hydration and giving analgesics for pain control. The dose of Danazol was unchanged. After a few days, her symptoms improved and her pancreatic enzymes returned to normal. CONCLUSIONS: Intestinal swelling associated with acute HAE attacks may induce pancreatitis. There are five previously reported cases of pancreatitis associated with HAE exacerbations. Therefore it may be prudent to check serum amylase and lipase in patients with HAE presenting with severe abdominal attacks, as it may affect management.
1069
Hereditary Angioedema: Clinical Aspects in a Brazilian Family M. Ferraro, M. Thiesen, L. Arruda, W. Sarti; School of Medicine of Ribeira˜o Preto-University of Sa˜o Paulo, Ribeira˜o Preto, BRAZIL. RATIONALE: To describe clinical features and treatment outcomes within a six-generation family with hereditary angioedema. METHODS: Members of a family from Minas Gerais, Brazil, with a history of recurrent episodes of facial swelling and attacks of abdominal pain were investigated. Family pedigree was constructed with 44 individuals, and showed 15 affected members (10 women), distributed in six generations. Seven patients were interviewed and examined, and information on eight members (two deceased) was provided by relatives. Twentynine individuals did not present history suggestive of hereditary angioedema. Levels of complement C3 and C4 were obtained by nephelometry, and quantitation of C1 inhibitor was carried out by radial immunodiffusion. Four patients are receiving Danazol. RESULTS: Seven female patients, 6 to 42 years-old, were evaluated. Four patients showed levels of C1 inhibitor of 4.0 to 7.0mg/dl (normal values 1430mg/dl), of whom 3 had low levels of C4. Two girls (6 and 12 years-old) presented normal C1 inhibitor levels and activity, but low C4 levels. One patient had normal levels of C4 and C1 inhibitor on two occasions. A prominent feature among these patients was presence of recurrent attacks of abdominal pain. For those patients with low C1 inhibitor levels, treatment was initiated with Danazol 50mg daily, or 50mg alternate days, followed by excellent clinical response, with no recurrence of symptoms or side effects. CONCLUSIONS: Hereditary angioedema is a genetic entity that can lead to severe complications. Long term prophylaxis with Danazol was shown to be effective at lower doses than those recommended in the literature. Funding: FAPESP
1070
Fresh Frozen Plasma for the Treatment of Hereditary Angioedema M. Prematta, J. Gibbs, T. J. Craig, E. Pratt, T. Stoughton; Penn State University, Hershey, PA. RATIONALE: Fresh frozen plasma (FFP) has been used as a treatment option for patients with Hereditary Angioedema (HAE) because it contains C1-esterase inhibitor and alternate therapies are not yet available in the USA. However, because FFP also contains other substrates, it has been hypothesized to have the potential to worsen or precipitate an acute attack of HAE. It is our aim to research patient records and the medical literature to determine whether FFP can exacerbate symptoms or precipitate an attack of HAE. METHODS: The following search terms were used in PubMed and OVID: ‘‘Hereditary Angioedema’’ and ‘‘Angioedema and Fresh Frozen Plasma.’’ Articles were searched from 1966 to the present. Charts of HAE patients at our institution who have received FFP since 1990 were reviewed to determine if there was any evidence that FFP exacerbated the symptoms of HAE. RESULTS: The literature and our patient chart review failed to identify instances when FFP exacerbated symptoms of HAE or precipitated an attack. Several reports and our experience demonstrate that FFP is an effective prophylactic agent immediately before surgery, during pregnancy, and for treatment of acute attacks of HAE, without evidence of exacerbation or initiation of symptoms. CONCLUSIONS: FFP seems to be safe and effective in preventing exacerbations of HAE before surgery, during pregnancy, and for acute exacerbations of HAE without evidence of initiating an attack or worsening a preexisting attack.
TUESDAY
1067