Mastocytosis

Published on 27/02/2015 by admin

Filed under Anesthesiology

Last modified 27/02/2015

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58. Mastocytosis

Definition

Mastocytosis is a clonal disorder in which mast cells and the precursor cells accumulate in different tissues, such as bone marrow, skin, gastrointestinal tract, liver, and spleen.
Classifications of Mastocytosis

Category IA Indolent disease with skin involvement
Category IB Systemic involvement with or without skin involvement
Category II A hematopoietic disorder is a part of the disease
Category IIA Dysmyelopoietic disorder associated with mastocytosis
Category IIB Accompanied by myeloproliferative disease
Category IIC Associated with nonlymphocytic leukemia
Category IID Associated with malignant lymphoma
Category IIE Associated with neutropenia
Category III Mast cell leukemia becomes a part of the disease
Category IV Associated with lymphadenopathic mastocytosis with eosinophilia (also known as aggressive mastocytosis)

Incidence

The incidence of mastocytosis is extremely rare. The specific frequency of occurrence has not been documented.

Etiology

Mutations of the c- kit proto-oncogene have been implicated as the cause of some forms of mastocytosis. The systemic form, a codon-816 c- kit mutation, is commonly found.

Signs and Symptoms

• Abdominal pain
• Anemia
• Ascites
• Basophilia
• Budd-Chiari syndrome (see p. 59)
• Dilated small bowel
• Diverticulitis
• Eosinophilia
• Esophageal stricture
• Esophageal varices
• Esophagitis
• Hepatomegaly
• Leukocytosis
• Leukopenia
• Lymphadenopathy
• Lymphopenia
• Malabsorption
• Monocytosis
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