ABSTRACT
Objective:To investigate the clinical characteristics of idiopathic hypereosinophilic syndrome (IHES) with gastrointestinal manifestations,and to improve the level of diagnosis and treatment of IHES. Methods:The clinical materials, process of diagnosis and treatment and prognosis of 9 patients diagnosed as IHES with gastrointestinal manifestations were retrospectively analyzed. Results:The average age of 9 patients was (22.66± 12.86)years old,and the ratio of male and female was about 1.25∶ 1. The main clinical manifestations included abdominal pain,diarrhea and abdominal distension.The eosinophil percentages in peripheral blood and bone marrow of the patients were (42.66 ± 19.88 )% and (39.33 + 15.99 )%, respectively.The ascites exudate cytology examination showed eosinophil infiltrated.The results of gastroscope or colonoscope showed mucosal hyperemia and edema,scattered bleeding spots, and dark red granular hyperplasia; the colon was affected frecuently.The histological biopsy confirmed that the mucosal was infiltrated by eosinophils.The abdominal CT of 6 patients showed that the walls of stomach or bowel were thickened.The abdominal symptoms disappeared,and the ascites was absorpted in 9 patients after the treatment of glucocorticoid.After 2 years of follow up,2 patients had relapse, others had no recurrence.Conclusion:Performing the routine diagnosis and treatment of gastrointestinal diseases, the clinicians should consider the possibility of IHES in order to avoid the misdiagnosis and delayed treatment. When IHES is diagnosed,steroid treatment should be performed in preference.
ABSTRACT
Although idiopathic hypereosinophilic syndrome(IHES) commonly involves the lung, it is rarely associated with acute respiratory distress syndrome (ARDS). Here we describe a case of IHES presented in conjunction with ARDS. A 37-year-old male visited the emergency department at Samsung Medical Center, Seoul, Korea, with a chief complaint of dyspnea. Blood tests showed profound peripheral eosinophilia and thrombocytopenia. Patchy areas of consolidation with ground-glass opacity were noticed in both lower lung zones on chest radiography. Rapid progression of dyspnea and hypoxia despite supplement of oxygen necessitated the use of mechanical ventilation. Eosinophilic airway inflammation was subsequently confirmed by bronchoalveolar lavage, leading to a diagnosis of IHES. High-dose corticosteroids were administered, resulting in a dramatic clinical response.
Subject(s)
Adult , Humans , Male , Adrenal Cortex Hormones , Hypoxia , Bronchoalveolar Lavage , Diagnosis , Dyspnea , Emergency Service, Hospital , Eosinophilia , Eosinophils , Hematologic Tests , Hypereosinophilic Syndrome , Inflammation , Korea , Lung , Oxygen , Pulmonary Embolism , Radiography , Respiration, Artificial , Respiratory Distress Syndrome , Seoul , Thorax , ThrombocytopeniaABSTRACT
Idiopathic hypereosinophilic syndrome is characterized by prolonged markedly elevated peripheral blood eosinophil count and eosinophil-related tissue damage to variable organs without an identifiable underlying cause. Eosinophilopoiesis is related with T-lymphocyte activation and cytokine cascade controlling eosinophilic production. It shows tissue infiltration in many organs including endomyocardium, lung, liver, gastrointestinal tract. Here we report a case of idiopathic hypereosinophilic syndrome presenting ruptured rectus sheath hematoma due to coagulopathy involving the liver. Severe postoperative complications were developed after general anesthesia. The patient suffered from life-threatening acute respiratory distress syndrome (ARDS). This case suggest that, in patients with marked eosinophilia requiring general anesthesia, perioperative steroid cover is advisable. This may reduce or prevent serious lung damage and other complications.
Subject(s)
Humans , Anesthesia, General , Eosinophilia , Eosinophils , Gastrointestinal Tract , Hematoma , Hypereosinophilic Syndrome , Liver , Lung , Postoperative Complications , Respiratory Distress Syndrome , T-LymphocytesABSTRACT
Idiopathic hypereosinophilic syndrome (IHES) is a rare disease characterized by peripheral blood eosinophilia and eosinophilic infiltration of multiple organs, including the nervous system. The neurologic manifestations in hypereosinophilic syndrome are variable, but eosinophilic meningitis as a manifestation has not been well documented in IHES. We report a case of eosinophilic meningitis associated with IHES, which showed a good response to steroid treatment.
Subject(s)
Eosinophilia , Eosinophils , Hypereosinophilic Syndrome , Meningitis , Nervous System , Neurologic Manifestations , Rare DiseasesABSTRACT
The idiopathic hypereosinophilic syndrome (HES) comprises a heterogeneous group of disorders with unknown pathogenesis characterized by persistent peripheral blood and bone marrow eosinophilia. And the eosinophil infiltrates of multiple organs in HES lead to severe organ dysfunction. The disseminated intravascular coagulation (DIC) is a rare complication of HES. We have experienced a case of HES complicated with DIC and pulmonary thromboembolism. After intravenous injection of methylprednisone, blood eosinophil count was normalized but DIC was persisted. With cortico steroid and cyclosporine therapy, the disease activity was favorably remitted.
Subject(s)
Bone Marrow , Cyclosporine , Dacarbazine , Disseminated Intravascular Coagulation , Eosinophilia , Eosinophils , Hypereosinophilic Syndrome , Injections, Intravenous , Pulmonary Embolism , ThromboembolismABSTRACT
The idiopathic hypereosinophilic syndrome (HES) is a disorder marked by the sustained overproduction of eosinophils. The disease is characterized by damage of multiple organ including heart, nerve system, skin and lung due to eosinophilic infiltration and the diagnosis is one of exclusion. Rheumatologic manifestations of HES are infrequent. In about 10~40% of rheumatoid arthritis (RA) patients, persistent eosinophilia is observed. That can be due to the RA itself and is often associated with active disease and extra-articular features. Sometimes, it is attributed to the drug therapy, especially gold and penicillamine. We would like to report a 37-year-old female patient with HES who developed seronegative RA 2 years later.
Subject(s)
Adult , Female , Humans , Arthritis, Rheumatoid , Diagnosis , Drug Therapy , Eosinophilia , Eosinophils , Heart , Hypereosinophilic Syndrome , Lung , Penicillamine , SkinABSTRACT
The idiopathic hypereosinophilic syndromes are rare hematologic disorders showing a wide spectrum of diseases with eosinophilia and associated organ damage. Three criteria for diagnosis include persistent eosinophilia of at least 1, 500 eosinophils/mm3 for longer than 6 months, or death before 6 months with signs and symptoms of hypereosinophilic disease; lack of evidence for parasitic, allergic or other known causes of eosinophilia; and signs and symptoms of organ involvement. We report a case of a 8 month-old male patient who had persistent marked eosinophilia reaching peak eosinophil count more than 100, 000/mm3, and organ dysfunction involving the bone marrow, heart, brain, lung and spleen. This patient, who did not respond to administration of prednisolone or hydroxyurea alone, has been treated successfully with hydroxyurea and interferon alpha-2b combination therapy.
Subject(s)
Humans , Infant , Male , Bone Marrow , Brain , Diagnosis , Eosinophilia , Eosinophils , Heart , Hydroxyurea , Hypereosinophilic Syndrome , Interferons , Lung , Prednisolone , SpleenABSTRACT
Idiopathic hypereosinophilic syndrome is defined as the presence of prolonged eosinophilia without an identifiable underlying cause and with evidence of end-organ dysfunction. The organs involved are the heart, bone marrow, nervous system, lungs, liver, skin, and gastrointestinal tract. Hepatic involvement is found in about 30% of patients of idiopathic hypereosinophilic syndrome. It occurs rarely in infants and children. In this report, we experienced one case of idiopathic hypereosinophilic syndrome with hepatic involvement in a 5-year-old boy who complained of intermittent fever and right upper quadrant abdominal pain. An abdominal ultrasound examination revealed an ill-defined low-echoic lesion in the liver. Pathologic findings of a biopsy specimen clearly showed the infiltration of eosinophils in the liver. Laboratory data disclosed absolute eosinophilia. There was no evidence of allergic disease or parasitic infestation.
Subject(s)
Child, Preschool , Humans , Male , English Abstract , Eosinophils/pathology , Hypereosinophilic Syndrome/complications , Liver/pathology , Liver Diseases/complicationsABSTRACT
Transient peripheral eosinophilia occurs in several disorders, such as allergic diseases, cancer, and parasitic in fections. However, in most cases, their persence is not accompanied by tissue destruction or organ dysfunc tion. In certain disease states, eosinophils can accumulate in any organ in the body and cause tissue destruction as a result of the eosinophil infiltration or the toxic effects of the degranulated proinflammatory products. Idiopathic hypereosinopilic syndrome is a rare disorder characterized by persistent eosinophilia of an unknown origin, usually associated with a dysfunction of organs such as the heart, lung, skin, and nervous system. Idiopathic hypereosinophilic syndrome usually has an indolent course over a period of several months. However, in some cases, they have grave symptoms if vital organs such as heart and lung are infiltrated. Here we report a case of idiopathic hypereosinophilic syndrome presenting acute pulmonary edema involving the heart, bone marrow, and lung with a review of the relevant literatures.
Subject(s)
Bone Marrow , Eosinophilia , Eosinophils , Heart , Hypereosinophilic Syndrome , Lung , Nervous System , Pulmonary Edema , SkinABSTRACT
A 45-year-old man presented with muscle pain, skin nodules and persistent hypereosinophilia over a period of 4 months. Laboratory data excluded the diagnosis of trichinosis or any other parasite infection. The patient's course of the disease over the 9 months was compatible with idiopathic hypereosinophilic syndrome. In a muscle biopsy, eosinophilic and lymphoplasma cells are predominantly infiltrated. Authors report a case of rhabdomyolysis due to eosinophilic polymyositis in idiopathic hypereosinophilic syndrome.
Subject(s)
Male , Humans , BiopsyABSTRACT
Chronic hepatitis associated with the idiopathic hypereosinophilic syndrome has been very rarely reported worldwide. Recently, we experienced a case of chronic hepatitis with piecemeal necrosis as the clinical feature of the idiopathic hypereosinophilic syndrome. The patient was a 49-year-old woman who complained of a mild fever, nausea, vomiting, and pain in the right upper quadrant. The eosinophil count of peripheral blood increased up to 14,020/microliter (64% of WBC). Liver biopsy specimen showed severe porto-periportal inflammation with marked eosinophilic infiltration and ballooning degeneration of hepatocytes. Corticosteroid therapy significantly normalized the eosinophil count of peripheral blood.
Subject(s)
Female , Humans , Middle Aged , Biopsy , Eosinophils , Fever , Hepatitis, Chronic , Hepatocytes , Hypereosinophilic Syndrome , Inflammation , Liver , Nausea , Necrosis , VomitingABSTRACT
Eosinophilia accompanied by eosinophilic invasion and organ dysfunction may develope idiopathic hypereosinophilic syndrome. Any organ can be involved including bone marrow, lung, skin, heart, gastrointestinal tract and nervous system. Cough, dyspnea, pleural effusion or chest pain are common pulmonary manifestation, and they may be attributed to parenchymal infiltration, pulmonary embolism or heart failure. We report a 43-year-old woman with idiopathic hypereosinophilic syndrome involving bone marrow, skin, and lung. The patient developed acute dyspnea and chest pain. High resolution CT demonstrated multiple wedge-shaped segmental involvement with pleural effusion thought to be a pulmonary infarction or heart failure. Echocardiography could not find any abnormality. Lung biopsy showed interstitial eosinophilic infiltration with increased eosinophils in BAL fluid. She was treated with high dose corticosteroid and hydroxyurea. Within few days, most of her symptoms disappeared and chest radiography nearly cleared up.
Subject(s)
Adult , Female , Humans , Biopsy , Bone Marrow , Chest Pain , Cough , Dyspnea , Echocardiography , Eosinophilia , Eosinophils , Gastrointestinal Tract , Heart , Heart Failure , Hydroxyurea , Hypereosinophilic Syndrome , Lung , Nervous System , Pleural Effusion , Pulmonary Embolism , Pulmonary Infarction , Radiography , Skin , ThoraxABSTRACT
Eosinophilia is quite common in Thailand due to the high prevalence of parasitic diseases. A study in 1993-1994 showed that 41% of patients with different parasitic infections had eosinophilia. We report herein a patient with idiopathic hypereosinophilic syndrome (HES). A 23-year-old man was admitted to the hospital with a 1.5 month history of chest pain and dyspnea on exertion. He had symptoms and signs of congestive heart failure. His peripheral blood revealed eosinophils 42% (8.4 x 109/1) with many hypogranulated and vacuolated eosinophils. Bone marrow aspiration showed a hypercellular marrow with eosinophils 10% and plasma cells 5-10%. Chest x-ray showed cardiomegaly and an echocardiography revealed pericardial effusion. As this patient had eosinophilia, signs and symptoms of cardiac involvement and lack of evidence for parasitic, allergic or other causes of eosinophilia, a diagnosis of idiopathic HES was made. Treatment with prednisolone resulted in the improvement of congestive heart failure and the eosinophil count returned to normal. A prompt diagnosis and treatment are needed in patents with this syndrome.