Literature DB >> 24298498

Systemic mastocytosis: predominantly involving the bone, a case report.

Ketan P Mallya1, Sushma Belurkar, Annamma Kurian, Laxmi Rao, Bikash Singhania.   

Abstract

Systemic mastocytosis (SM) is a rare clonal disorder of mast cells that can range from chronic smouldering type to aggressive mast cell leukaemia. It presents with non-specific symptoms like urticaria pigmentosa, unexplained flushing, hypotension and abdominal cramps, which may lead to a misdiagnosis, if there is no high index of clinical suspicion. This is a case report of a 52-year-old lady, with systemic mastocytosis, who presented with severe chronic back ache and no other clinical features. X - ray revealed lytic lesions in the lumbar vertebrae and bone marrow aspiration and a trephine biopsy examination showed infiltration by mast cells, with a positivity for Toluidine blue stain and CD 117. She was also noted to have peripheral eosinophilia, which is frequently encountered with this condition. She was diagnosed to have chronic indolent systemic mastocytosis which involved the bone predominantly.

Entities:  

Keywords:  CD117; Mast cells

Year:  2013        PMID: 24298498      PMCID: PMC3843398          DOI: 10.7860/JCDR/2013/5669.3493

Source DB:  PubMed          Journal:  J Clin Diagn Res        ISSN: 0973-709X


  6 in total

1.  Advanced systemic mastocytosis as a mimicker of metastatic clear cell renal cell carcinoma.

Authors:  Christoph Walz; Marion Subklewe; Hans-Peter Horny; Michael Flaig; Andreas Reiter; Thomas Kirchner; Karl Sotlar
Journal:  Leuk Res       Date:  2012-03-28       Impact factor: 3.156

2.  Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms.

Authors:  Iván Alvarez-Twose; David González de Olano; Laura Sánchez-Muñoz; Almudena Matito; Maria I Esteban-López; Arantza Vega; Maria Belén Mateo; Maria D Alonso Díaz de Durana; Belén de la Hoz; Maria D Del Pozo Gil; Teresa Caballero; Ana Rosado; Isabel Sánchez Matas; Cristina Teodósio; María Jara-Acevedo; Manuela Mollejo; Andrés García-Montero; Alberto Orfao; Luis Escribano
Journal:  J Allergy Clin Immunol       Date:  2010-06       Impact factor: 10.793

Review 3.  Pathogenesis, clinical features, and treatment advances in mastocytosis.

Authors:  A Pardanani; C Akin; P Valent
Journal:  Best Pract Res Clin Haematol       Date:  2006       Impact factor: 3.020

Review 4.  Diagnosis of mastocytosis: general histopathological aspects, morphological criteria, and immunohistochemical findings.

Authors:  H P Horny; P Valent
Journal:  Leuk Res       Date:  2001-07       Impact factor: 3.156

5.  Slowly progressive systemic mastocytosis with high mast-cell burden and no evidence of a non-mast-cell hematologic disorder: an example of a smoldering case?

Authors:  C Akin; L M Scott; D D Metcalfe
Journal:  Leuk Res       Date:  2001-07       Impact factor: 3.156

6.  Assessment of the extent of cutaneous involvement in children and adults with mastocytosis: relationship to symptomatology, tryptase levels, and bone marrow pathology.

Authors:  Knut Brockow; Cem Akin; Mary Huber; Dean D Metcalfe
Journal:  J Am Acad Dermatol       Date:  2003-04       Impact factor: 11.527

  6 in total

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