Sd hipereosinofilica

Question 1

O que são Sd Hipereosinofilicas

Answer 1

The hypereosinophilic syndromes (HES) are a group of disorders marked by the sustained overproduction of eosinophils, in which eosinophilic infiltration and mediator release cause damage to multiple organs

Question 2

Definicao de Hipereosinofilia

Answer 2

Hypereosinophilia — Hypereosinophilia (HE) in the peripheral blood is defined as an absolute eosinophil count (AEC) >1.5 x 109/L (or >1500 cells/microL) on two examinations separated in time by at least one month and/or pathologic confirmation of tissue HE.

● On bone marrow section, a percentage of eosinophils that exceeds 20 percent of all nucleated cells, and/or

● Tissue infiltration that is extensive in the opinion of a pathologist (hematoxylin-eosin, May-Grunwald-Giemsa and Wright-Giemsa are the recommended stains for visualizing eosinophils in tissue section) and/or

● Marked deposition of eosinophil granule proteins in tissue (in the absence or presence of major tissue infiltration by eosinophils). This is demonstrated by immunofluorescence and usually performed in expert centers. There is also a novel monoclonal antibody that recognizes eosinophil peroxidase, which can detect both tissue eosinophils and eosinophil degranulation [

Question 3

Categorias de SHE

Answer 3

Categories of HES — HES are further subclassified according to the pathogenic mechanisms resulting in eosinophil expansion: primary, secondary, or idiopathic. (See ‘Pathophysiology’ below.)
● Primary (or neoplastic) HES – In primary HES, eosinophilic expansion occurs in the setting of an underlying stem cell, myeloid, or eosinophilic neoplasm, and is considered (or proven) clonal.

● Secondary (or reactive) HES – In secondary HES, eosinophilic expansion is driven by overproduction of eosinophilopoietic cytokines by other cell types and is polyclonal. This is typically the situation encountered in parasitic infections, certain solid tumors, and T cell lymphoma, wherein marked secondary HE may be responsible for organ damage and dysfunction. Lymphocytic variant HES (L-HES) is a subvariant in this category

Question 4

Clinica SHE

Answer 4

• SCV: miocardite
• Neuro: HES may be complicated by cerebral thromboemboli, encephalopathy, peripheral neuropathy, or longitudinal and/or transverse sinus thrombosis
  Systemic symptoms such as fever, chills, night sweats, and weight loss
• Pele: Common skin manifestations of HES include eczema (involving hands, flexural areas, or dispersed plaques), erythroderma, generalized thickening of the skin (lichenification), dermographism, recurrent urticaria, and angioedema
• Pulmonary disease — Pulmonary involvement is common in HES and may result from eosinophilic infiltration of the lung with subsequent fibrosis, heart failure, or pulmonary emboli
• TGI: Eosinophilic gastritis, enteritis, and/or colitis may occur secondary to HES and cause weight loss, abdominal pain, vomiting, and/or severe diarrhea [5]. Hepatic involvement may take the form of chronic active hepatitis, focal hepatic lesions, eosinophilic cholangitis, or the Budd-Chiari syndrome.

Question 5

Mneumonico CHINA

Answer 5

CHINA
C olagenoses
H Elmintos
I diopatica hipereosinofilica
Neoplasia - Linfoma mais comum
A alergia, asma

Question 6

Tto

Answer 6

GC

Imatinib