Lymphadenopathic mastocytosis with eosinophilia and biclonal gammopathy

Frieri, Marianne; Linn, N.; Schweitzer, Mark E.; Angadi, Channabapassa; Pardanani, Bhagwati

doi:10.1016/s0091-6749(05)80132-0

Cited by 17 publications

(5 citation statements)

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“…A CAT scan revealed abdominal lymphadenopathy, heptosplenomegaly, and liver and lymph nodes with increased mast cells. 17 He was diagnosed with acute nonlymphocytic leukemia. 18 Pediatric mastocytosis is often mistaken for more common rashes such as in one study with a 9-month-old boy who presented with a rash that was originally mistaken for a milk allergy and then for atopic dermatitis, 19 or a 10-month-old boy with recurrent urticarial episodes.…”

Section: Acute Myeloid Leukemia and Selected Clinical Casesmentioning

confidence: 99%

Pediatric Mastocytosis: A Review of the Literature

Frieri

Quershi

2013

Pediatric Allergy, Immunology, and Pulmonology

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Mastocytosis has a bimodal distribution often presenting in children from birth to 2 years of age and in those over the age of 15. Pediatric mastocytosis is due to the effects of mast-cell degranulation enzymes such as histamine and tryptase causing the presentation of pruritis, flushing, vesicles, abdominal and bone pain, or headache. Three different forms of mastocytosis can occur: urticaria pigmentosa, diffuse cutaneous, and solitary mastocytoma. Systemic symptoms are typically a result of mast-cell mediator release but do not prove systemic mast-cell hyperplasia. In this review, we present several research studies related to pediatric mast-cell disorders, and discuss several cases of pediatric mastocytosis, acute myeloid leukemia, pathophysiology, genetic studies, and treatment. Introduction and Research Studies

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Section: Acute Myeloid Leukemia and Selected Clinical Casesmentioning

confidence: 99%

Pediatric Mastocytosis: A Review of the Literature

Frieri

Quershi

2013

Pediatric Allergy, Immunology, and Pulmonology

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“…We earlier reported a case of lymphadenopathic mastocytosis in a patient who presented with SM with eosinophilia and biclonal gammopathy who eventually died of mast cell leukemia [34]. Gotlib noted that eosinophils and mast cells serve critical roles as part of the host immune response and in maintenance of normal homeostasis.…”

Section: Relationship With Mastocytosismentioning

confidence: 99%

Mast Cell Activation Syndrome

Frieri

2015

Clinic Rev Allerg Immunol

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Mast cell activation syndrome (MCAS) involves the skin, gastrointestinal, cardiovascular, respiratory, and neurologic systems, classified as primary, secondary, and idiopathic. Earlier criteria for MCAS diagnosis included episodic symptoms with mast cell mediators affecting two or more organ systems with urticaria, angioedema, flushing, nausea, vomiting, diarrhea, abdominal cramping, hypotensive syncope, tachycardia, wheezing, conjunctival injection, pruritus, nasal stuffiness, decrease in frequency, severity, or resolution of symptoms with anti-mediator therapy including H/H receptor antagonists, anti-leukotrienes, or mast cell stabilizers. Laboratory data includes an increased validated urinary or serum markers of MCAS, documentation of an increase of the marker above the patient's baseline value during symptomatic periods on more than two occasions, or baseline serum tryptase levels that are persistently above 15 ng/mL. Laboratory data also includes an increase of the tryptase level above baseline value on one occasion. Other assays are 24-h urine histamine metabolites, PGD or its metabolite, and 11-β-prostaglandin F alpha. A recent global classification is a response of clinical symptoms, a substantial transient increase in serum total tryptase or increase in other mast cell-derived mediators, histamine or PGD2 or urinary metabolites, and agents that attenuate production or mast cell mediator activities. "Spectrum of MCAS disorders" has been proposed, highlighting symptoms' diagnostic tests and treatments.

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“…5 Lymphadenopathic mastocytosis with eosinophilia is a distinct subtype, characterized by blood and/or tissue eosinophilia. 30 MCL is defined by a bone marrow aspirate with >20% MC and criteria for SM fulfilled. and evidence of organ dysfunction (i.e.…”

Section: Diagnosis and Classificationmentioning

confidence: 99%