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EOSINOPHILIC CYSTITIS AND PARASITIC INFECTION
Abstracts: Medically Challenging Cases / Ann Allergy Asthma Immunol 121 (2018) S63−S134
Introduction: Systemic Mastocytosis (SM) is a disorder characterized by the excessive accumulation and activation of mast cells (MC) in various organs. Many biomarkers have proven to be helpful in disease screening and diagnosis. Namely, serum tryptase, urinary N-methylhistamine, leukotriene E4 (LTE4) and prostaglandin F2 alpha (PGF2a). Our purpose is to describe a case of Indolent SM (ISM) with normal MC biomarkers. Case Description: This is a 30 y.o. female who presented with a 10-year history of headaches, clouding of consciousness, syncope, intermittent abdominal pain, nausea, diarrhea, bone pain, skin flushing and suspected episodes of anaphylaxis. Serum tryptase, 24-hour urinary N-methylhistamine, PGF2a and LTE4 were all within normal limits. Blood molecular studies confirmed KITD816V point mutation. Skin biopsy showed mastocytosis with features of telangiectasia macularis eruptiva perstans. Bone scan was consistent with bony remodeling for MC disease. Ultimately, bone marrow (BM) biopsy showed low-level (25%) exhibiting an atypical spindle shape morphology, immunohistochemistry detected aberrant CD5 and CD2 co-expression confirming SM. Discussion: Cutaneous and ISM have a good prognosis with similar survival rates compared to the general population. However, close monitoring may be warranted in SM patients, since a low-rate progression has been reported. The relationship between biomarkers and symptomatology and MC burden is not completely understood nor are the sensitivities of the tests. In the right clinical setting, clinicians should proceed with bone marrow biopsy, immunohistochemistry and molecular studies for a prompt and accurate diagnosis regardless of the status of the above mentioned serum and urine MC biomarkers.
S125
Aspergilloma
2 cm x 1.2 cm right apical spiculated pulmonary nodule with coarse calcifications laterally.
M363 M362 WHEN A COUGH ISN’T ASTHMA; WHEN A MASS IS NOT CANCER D. Sanchez-Tejera*, T. Bingemann, Rochester, NY Introduction: The differential of chronic cough is broad and changes in the pattern of cough should be evaluated. We present a case with a surprising finding during our evaluation of worsening cough. Case description: A 68-year-old female former smoker with a history of breast cancer status post mastectomy, asthma and allergic rhinitis presented with complaints of worsening cough, hoarseness, and intermittent dysphagia of 3 months duration. Chest radiograph revealed a 2.4 £ 1.6 cm right upper lung (RUL) nodule. Chest computed tomography showed a 2.4 cm x 2.1 cm RUL mass, without cavitary lesions, and no evidence of bronchiectasis. Biopsy of this lesion revealed a mycetoma with no features of invasive aspergillosis. Upon further questioning the patient revealed having night sweats and right axillary pain; however, no hemoptysis. Labs were significant for elevated Aspergillus fumigatus IgE and no eosinophilia. She completed 6 months of treatment with decrease in aspergillus IgE and resolution of her symptoms. Simultaneous ENT evaluation revealed moderate arytenoid edema, where therapy with ranitidine therapy was initiated. Discussion: We present a case of pulmonary aspergilloma discovered during an evaluation for worsening cough in an immunocompetent patient with asthma and no evidence of cavitary lung lesions or ABPA. The literature reports antifungals and/or surgery is the mainstay of therapy. Our patient’s cough improved with simultaneous treatment for GERD and aspergilloma. Changes in cough patterns should prompt further evaluation and aspergilloma should be considered in the differential diagnosis of mass lesions.
EOSINOPHILIC CYSTITIS AND PARASITIC INFECTION S. Hwang*, J. Hagerty, T. Le, Wilmington, DE Introduction: Eosinophilic cystitis is a rare condition characterized by eosinophilic inflammation of the bladder with few documented pediatric cases. The mechanism of disease remains unclear, although it has been associated with various disorders such as allergic diseases, malignancy, trauma, and infection. Treatment regimens are non-standardized and have included antihistamines, leukotriene antagonists, antimicrobials, steroids, and anti-inflammatory agents. Case Description: A 12-year-old African American male with history of food allergy and allergic rhinitis presented to urology due to persistent hematuria and dysuria. Cystouretheroscopy with biopsy demonstrated evidence of eosinophilic cystitis. He denied use of any medications or history of international travel. CBC revealed a normal AEC of 388 cells/mL; however, he was noted to have eosinophilia to 1670 cells/mL on CBC performed four years prior. He was initially treated with phenazopyridine, oxybutynin, and cetirizine. Montelukast and ibuprofen were added for refractory symptoms. Due to consideration for immunosuppressive steroid therapy, stool O&P, Strongyloides, Trichinella, Toxocara serologies, and GI PCR panel were sent to exclude parasitic infection. Strongyloides IgG and Clostridium difficile toxin were positive. He was treated with ivermectin and metronidazole for latent Strongyloides infection and C. difficile colitis, respectively, and demonstrated interval clinical improvement. Discussion: To the authors’ knowledge, this is the first case of eosinophilic cystitis associated with Strongyloides stercoralis infection. A high index of suspicion for parasitic infection in patients with eosinophilic cystitis must be maintained regardless of exposure history or peripheral eosinophil counts as chronic infection may be asymptomatic. Initiation of steroid therapy without confirming negative testing may result in life-threatening, disseminated strongyloidiasis.
Introduction: Systemic Mastocytosis (SM) is a disorder characterized by the excessive accumulation and activation of mast cells (MC) in various organs. Many biomarkers have proven to be helpful in disease screening and diagnosis. Namely, serum tryptase, urinary N-methylhistamine, leukotriene E4 (LTE4) and prostaglandin F2 alpha (PGF2a). Our purpose is to describe a case of Indolent SM (ISM) with normal MC biomarkers. Case Description: This is a 30 y.o. female who presented with a 10-year history of headaches, clouding of consciousness, syncope, intermittent abdominal pain, nausea, diarrhea, bone pain, skin flushing and suspected episodes of anaphylaxis. Serum tryptase, 24-hour urinary N-methylhistamine, PGF2a and LTE4 were all within normal limits. Blood molecular studies confirmed KITD816V point mutation. Skin biopsy showed mastocytosis with features of telangiectasia macularis eruptiva perstans. Bone scan was consistent with bony remodeling for MC disease. Ultimately, bone marrow (BM) biopsy showed low-level (25%) exhibiting an atypical spindle shape morphology, immunohistochemistry detected aberrant CD5 and CD2 co-expression confirming SM. Discussion: Cutaneous and ISM have a good prognosis with similar survival rates compared to the general population. However, close monitoring may be warranted in SM patients, since a low-rate progression has been reported. The relationship between biomarkers and symptomatology and MC burden is not completely understood nor are the sensitivities of the tests. In the right clinical setting, clinicians should proceed with bone marrow biopsy, immunohistochemistry and molecular studies for a prompt and accurate diagnosis regardless of the status of the above mentioned serum and urine MC biomarkers.
S125
Aspergilloma
2 cm x 1.2 cm right apical spiculated pulmonary nodule with coarse calcifications laterally.
M363 M362 WHEN A COUGH ISN’T ASTHMA; WHEN A MASS IS NOT CANCER D. Sanchez-Tejera*, T. Bingemann, Rochester, NY Introduction: The differential of chronic cough is broad and changes in the pattern of cough should be evaluated. We present a case with a surprising finding during our evaluation of worsening cough. Case description: A 68-year-old female former smoker with a history of breast cancer status post mastectomy, asthma and allergic rhinitis presented with complaints of worsening cough, hoarseness, and intermittent dysphagia of 3 months duration. Chest radiograph revealed a 2.4 £ 1.6 cm right upper lung (RUL) nodule. Chest computed tomography showed a 2.4 cm x 2.1 cm RUL mass, without cavitary lesions, and no evidence of bronchiectasis. Biopsy of this lesion revealed a mycetoma with no features of invasive aspergillosis. Upon further questioning the patient revealed having night sweats and right axillary pain; however, no hemoptysis. Labs were significant for elevated Aspergillus fumigatus IgE and no eosinophilia. She completed 6 months of treatment with decrease in aspergillus IgE and resolution of her symptoms. Simultaneous ENT evaluation revealed moderate arytenoid edema, where therapy with ranitidine therapy was initiated. Discussion: We present a case of pulmonary aspergilloma discovered during an evaluation for worsening cough in an immunocompetent patient with asthma and no evidence of cavitary lung lesions or ABPA. The literature reports antifungals and/or surgery is the mainstay of therapy. Our patient’s cough improved with simultaneous treatment for GERD and aspergilloma. Changes in cough patterns should prompt further evaluation and aspergilloma should be considered in the differential diagnosis of mass lesions.
EOSINOPHILIC CYSTITIS AND PARASITIC INFECTION S. Hwang*, J. Hagerty, T. Le, Wilmington, DE Introduction: Eosinophilic cystitis is a rare condition characterized by eosinophilic inflammation of the bladder with few documented pediatric cases. The mechanism of disease remains unclear, although it has been associated with various disorders such as allergic diseases, malignancy, trauma, and infection. Treatment regimens are non-standardized and have included antihistamines, leukotriene antagonists, antimicrobials, steroids, and anti-inflammatory agents. Case Description: A 12-year-old African American male with history of food allergy and allergic rhinitis presented to urology due to persistent hematuria and dysuria. Cystouretheroscopy with biopsy demonstrated evidence of eosinophilic cystitis. He denied use of any medications or history of international travel. CBC revealed a normal AEC of 388 cells/mL; however, he was noted to have eosinophilia to 1670 cells/mL on CBC performed four years prior. He was initially treated with phenazopyridine, oxybutynin, and cetirizine. Montelukast and ibuprofen were added for refractory symptoms. Due to consideration for immunosuppressive steroid therapy, stool O&P, Strongyloides, Trichinella, Toxocara serologies, and GI PCR panel were sent to exclude parasitic infection. Strongyloides IgG and Clostridium difficile toxin were positive. He was treated with ivermectin and metronidazole for latent Strongyloides infection and C. difficile colitis, respectively, and demonstrated interval clinical improvement. Discussion: To the authors’ knowledge, this is the first case of eosinophilic cystitis associated with Strongyloides stercoralis infection. A high index of suspicion for parasitic infection in patients with eosinophilic cystitis must be maintained regardless of exposure history or peripheral eosinophil counts as chronic infection may be asymptomatic. Initiation of steroid therapy without confirming negative testing may result in life-threatening, disseminated strongyloidiasis.