The medical records of 21 patients evaluated for mastocytosis and 2 patients seen for follow-up of known mastocytosis who underwent bilateral iliac crest aspirations and biopsies were reviewed retrospectively to determine whether mastocytosis could be confirmed in each of a patient’s biopsy specimens. In 19 cases (83%), each biopsy specimen showed evidence of mastocytosis; however, in 4 cases (...

Introduction: Anaphylaxis is defined as “a serious, life-threatening generalized or systemic hypersensitivity reaction” and “a serious allergic reaction that is rapid in onset and might cause death”. The determination of different mast cells (MC) mediators, such as serum and/or plasma histamine and tryptase, is proposed for the diagnosis of anaphylaxis. There are differences in the clinical pre...

INTRODUCTION Well-differentiated systemic mastocytosis (WDSM) is a rare, recently recognized provisional subvariant of systemic mastocytosis (SM). We report a case of WDSM that showed excellent clinical and cutaneous response to imatinib in the absence of known molecular genetic abnormalities. CLINICAL FINDINGS/DIAGNOSES We present a 24-year-old woman with childhood onset of skin manifestatio...

Cytogenetic analysis of bone marrow cells and in vitro growth for bone marrow granulocytic-macrophage stem cells have been performed in 1 3 patients with mastocytosis. six with systemic mastocytosis. and seven with urticaria pigmentosa. Clones with chromosome abnormalities were found in five patients. The number of clusters and/or colonies after seven days in culture was increased in seven pati...

We examined the association between severity of disease in mastocytosis and skeletal manifestation and bone markers in 16 patients varying in extent of mastocytosis as determined by the urine excretion of methylimidazoleacetic acid. Both osteoporosis and osteosclerosis were found. Bone density in the hip was significantly higher (p < 0.05) in both men and women with an enhanced histamine metabo...

Background: Infantile vesiculobullous lesions are caused by infections, drugs, congenital and autoimmune disorders. Mastocytosis refers to over-proliferation and accumulation of tissue mast cells, encompassing a spectrum ranging from indolent cutaneous to malignant and systemic conditions. Bullous mastocytosis is a rare cutaneous form. Case characteristics: A male infant with recurring bullous ...

Systemic mastocytoses represent neoplastic proliferations of mast cells. In about 20% of cases systemic mastocytoses are accompanied by clonal haematopoietic non-mast cell-lineage disorders, most commonly myeloid neoplasms. A case of systemic mastocytosis carrying the characteristic mutation at codon 816 (D816V) in the KIT gene of mast cells, with two concurrent accompanying clonal haematopoiet...