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A Case of Idiopathic Hypereosinophilic Syndrome Causing Mitral Valve Papillary Muscle Rupture.

Tamse T, Rampersad A, Jordan-Villegas A, Ireland J - Case Rep Pediatr (2015)

Bottom Line: This disease can cause significant morbidity and mortality if left untreated.She was treated with steroids with improvement of her symptoms and scheduled for close follow-up.In general patients with IHES that have cardiac involvement have poorer prognoses.

View Article: PubMed Central - PubMed

Affiliation: Florida Hospital for Children, Orlando, FL 32803, USA ; University of Central Florida, Orlando, FL 32827, USA.

ABSTRACT
Idiopathic Hypereosinophilic Syndrome (IHES) is a rare disease that can be difficult to diagnose as the differential is broad. This disease can cause significant morbidity and mortality if left untreated. Our patient is a 17-year-old adolescent female who presented with nonspecific symptoms of abdominal pain and malaise. She was incidentally found to have hypereosinophilia of 16,000 on complete blood count and nonspecific colitis and pulmonary edema on computed tomography. She went into cardiogenic shock due to papillary rupture of her mitral valve requiring extreme life support measures including intubation and extracorporal membrane oxygenation (ECMO) as well as mitral valve replacement. Pathology of the valve showed eosinophilic infiltration as the underlying etiology. The patient was diagnosed with IHES after the exclusion of infectious, rheumatologic, and oncologic causes. She was treated with steroids with improvement of her symptoms and scheduled for close follow-up. In general patients with IHES that have cardiac involvement have poorer prognoses.

No MeSH data available.


Related in: MedlinePlus

(a) Peripheral smear: marked eosinophilia (most eosinophils show intact cytoplasmic granules). (b) Bone marrow biopsy: normocellular marrow with mild megakaryocytic hyperplasia, eosinophilia, and no increase in blasts. No monoclonal B-cells or immunophenotypically abnormal T-cells are detected.
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fig2: (a) Peripheral smear: marked eosinophilia (most eosinophils show intact cytoplasmic granules). (b) Bone marrow biopsy: normocellular marrow with mild megakaryocytic hyperplasia, eosinophilia, and no increase in blasts. No monoclonal B-cells or immunophenotypically abnormal T-cells are detected.

Mentions: Pathology specimens, peripheral smear, bone marrow, bowel, and heart valve all demonstrated marked eosinophilic infiltration (Figures 2 and 3). Workup was negative for vasculitis, collagen vascular, infectious, and myeloproliferative diseases (Table 1).


A Case of Idiopathic Hypereosinophilic Syndrome Causing Mitral Valve Papillary Muscle Rupture.

Tamse T, Rampersad A, Jordan-Villegas A, Ireland J - Case Rep Pediatr (2015)

(a) Peripheral smear: marked eosinophilia (most eosinophils show intact cytoplasmic granules). (b) Bone marrow biopsy: normocellular marrow with mild megakaryocytic hyperplasia, eosinophilia, and no increase in blasts. No monoclonal B-cells or immunophenotypically abnormal T-cells are detected.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4660015&req=5

fig2: (a) Peripheral smear: marked eosinophilia (most eosinophils show intact cytoplasmic granules). (b) Bone marrow biopsy: normocellular marrow with mild megakaryocytic hyperplasia, eosinophilia, and no increase in blasts. No monoclonal B-cells or immunophenotypically abnormal T-cells are detected.
Mentions: Pathology specimens, peripheral smear, bone marrow, bowel, and heart valve all demonstrated marked eosinophilic infiltration (Figures 2 and 3). Workup was negative for vasculitis, collagen vascular, infectious, and myeloproliferative diseases (Table 1).

Bottom Line: This disease can cause significant morbidity and mortality if left untreated.She was treated with steroids with improvement of her symptoms and scheduled for close follow-up.In general patients with IHES that have cardiac involvement have poorer prognoses.

View Article: PubMed Central - PubMed

Affiliation: Florida Hospital for Children, Orlando, FL 32803, USA ; University of Central Florida, Orlando, FL 32827, USA.

ABSTRACT
Idiopathic Hypereosinophilic Syndrome (IHES) is a rare disease that can be difficult to diagnose as the differential is broad. This disease can cause significant morbidity and mortality if left untreated. Our patient is a 17-year-old adolescent female who presented with nonspecific symptoms of abdominal pain and malaise. She was incidentally found to have hypereosinophilia of 16,000 on complete blood count and nonspecific colitis and pulmonary edema on computed tomography. She went into cardiogenic shock due to papillary rupture of her mitral valve requiring extreme life support measures including intubation and extracorporal membrane oxygenation (ECMO) as well as mitral valve replacement. Pathology of the valve showed eosinophilic infiltration as the underlying etiology. The patient was diagnosed with IHES after the exclusion of infectious, rheumatologic, and oncologic causes. She was treated with steroids with improvement of her symptoms and scheduled for close follow-up. In general patients with IHES that have cardiac involvement have poorer prognoses.

No MeSH data available.


Related in: MedlinePlus