Anemia ; etiology ; Erythema ; etiology ; Fatal Outcome ; Female ; Fever ; etiology ; Humans ; Hypereosinophilic Syndrome ; complications ; diagnosis ; Infant

Adult ; Humans ; Hypereosinophilic Syndrome ; complications ; drug therapy ; Lymphatic Diseases ; diagnosis ; etiology ; pathology ; Male ; Treatment Outcome

Heart Valve Diseases ; Heart Valve Prosthesis ; Humans ; Hypereosinophilic Syndrome/complications* ; Mitral Valve ; Thrombosis/etiology*

The acute peripheral neuropathy as one of hypereosinophilic syndrome is known to be a rare disorder. The authors experienced a dramatic case with acute peripheral neuropathy, hypereosinophilia in peripheral blood, and the positive anti-GM1 antibodies. The serum protein electrophoresis showed a diffusely increased gamma-globulin region and the polyclonal gammopathy was found by the immunoelectropheresis. There was no evidence of inflammatory myopathy in vastus lateralis muscle. The sural nerve biopsy was compatible with vascular neuropathy, as there were a few myelin digestion chambers, mild perineuronal fibrosis, and perivascular lymphoplasmocytic infiltration with focal organizing thrombosis. The clinical response to prednisone therapy was excellent.
Acute Disease ; Adult ; Antibodies/*blood ; G(M1) Ganglioside/*immunology ; Humans ; Hypereosinophilic Syndrome/*complications/immunology ; Male ; Peripheral Nervous System Diseases/*etiology

Hypereosinophilic syndrome (HES) is a clinical disorder characterized by persistent eosinophilia and systemic involvement, in which a specific causative factor for the eosinophilia cannot be verified during a certain period of time. There have been only a few reported cases of this syndrome associated with malignant lymphoma. We report a case of peripheral T-cell lymphoma-unspecified with hypereosinophilic syndrome. The patient was a 42-year-old woman with an uncontrolled fever and a sore throat. Eosinophilia was observed on the peripheral blood smear. We confirmed the diagnosis by bone marrow and liver biopsies: A bone marrow aspiration demonstrated markedly increased eosinophils (24.8%), and a liver biopsy demonstrated infiltration by scattered eosinophils and atypical lymphoid cells, which were confirmed to be T-cell lymphoma cells. This case was a distinctive presentation of peripheral T-cell lymphoma with hypereosinophilic syndrome, probably due to a paraneoplastic condition.
Recurrence ; Pleural Effusion/*etiology ; Lymphoma, T-Cell, Peripheral/complications/*diagnosis ; Liver Neoplasms/complications/*diagnosis ; Hypereosinophilic Syndrome/*etiology ; Humans ; Female ; Fatal Outcome ; Adult

Adrenal Cortex Hormones ; therapeutic use ; Disseminated Intravascular Coagulation ; drug therapy ; etiology ; Heparin ; therapeutic use ; Humans ; Hypereosinophilic Syndrome ; complications ; Male ; Middle Aged

We report here a case with hypereosinophilia and peripheral artery occlusion. A 32-yr-old Korean woman presented to us with lower extremity swelling and pain. Angiography revealed that multiple lower extremity arteries were occlusive. The biopsy specimen showed perivascular and periadnexal dense eosinophilic infiltration in dermis and subcutaneous adipose tissue. Laboratory investigations revealed a persistent hypereosinophilia. She was prescribed prednisolone 60 mg daily. Her skin lesion and pain were improved and the eosinophil count was dramatically decreased. After discharge, eosinophil count gradually increased again. Cyanosis and pain of her fingers recurred. She had been treated with cyclophosphamide pulse therapy. Her eosinophilia was decreased, but the cyanosis and tingling sense were progressive. The extremity arterial stenoses were slightly progressed. Skin biopsy showed perivascular eosinophilic infiltration in the dermis and CD40 ligand (CD40L) positive eosinophilic infiltration. The serum TNF-alpha was markedly increased. These results suggest that CD40L (a member of TNF-alpha superfamily) could play a role in the inflammatory processes when eosinophil infiltration and activation are observed. We prescribed prednisolone, cyclophosphamide, clopidogrel, cilostazol, beraprost and nifedipine, and she was discharged.
Adult ; Arterial Occlusive Diseases/*diagnosis/etiology ; CD40 Ligand/analysis ; Cyanosis/etiology ; Diagnosis, Differential ; Eosinophilia/*diagnosis/etiology ; Female ; Gangrene/etiology ; Humans ; Hypereosinophilic Syndrome/blood/complications/*diagnosis ; Immunohistochemistry ; Peripheral Vascular Diseases/*diagnosis/etiology ; Skin/chemistry/pathology ; Tumor Necrosis Factor-alpha/metabolism ; Vasculitis/*diagnosis/etiology

OBJECTIVETo evaluate the cardiovascular involvements in Chinese patients with hypereosinophilic syndrome.

METHODWe respectively reviewed 149 inpatients with hypereosinophilic syndrome admitted to Peking Union Medical College Hospital and analyzed the cardiovascular involvements in these patients.

RESULTSCardiac abnormalities was evidenced in 32.9% patients (49/149). The ratio of male vs female was 34:15. The average age of the patients was (41.3 ± 16.9) years and course of disease was (26.4 ± 72.3) months. Cardiovascular involvements included ST segment and/or T wave (ST-T) ischemic changes, arrhythmia, myocardial injury, cardiac thrombosis, pericardial effusion, pulmonary hypertension, valve disorder, vein or artery thrombosis. After glucocorticoid and/or chemotherapeutic agents and treatment for symptoms, 11 (22.4%) patients achieved remission but have recurrent attacks and 3 (6.1%) patients died from failure in treatment. The prognosis in patients with echocardiogram abnormalities were poorer than those only with electrocardiogram abnormalities (P < 0.05).

CONCLUSIONSCardiovascular involvements are common in patients with hypereosinophilic syndrome and the manifestation of these involvement is various. Cardiovascular complications of HES are a major source of morbidity and mortality in these disorders.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Heart Diseases ; diagnosis ; diagnostic imaging ; etiology ; Humans ; Hypereosinophilic Syndrome ; complications ; diagnostic imaging ; physiopathology ; Infant ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Ultrasonography ; Young Adult

BACKGROUND: Eosinophilia may be associated with various primary and reactive conditions. The incidence and the causes of eosinophilia might have been changed according to the changes in the incidence of diseases such as cancer, chronic degenerative diseases, etc. We have conducted a retrospective study to investigate the incidence and causes of eosinophilia. METHODS: Eosinophilia and hypereosinophilia were defined when absolute eosinophil count was greater than 500/microL and 1,500/microL, respectively. Patient's clinical records were reviewed to find out the underlying clinical conditions responsible for causes of hypereosinophilia. Conventional chromosomal analysis, reverse transcriptase PCR and FISH for gene rearrangement were performed to check the presence of clonal eosinophilia. RESULTS: Out of 41,137 patients who had a hematology profile performed, 5,019 (12.2%) and 373 patients (0.9%) were found to have eosinophilia and hypereosinophilia, respectively. Among patients with hypereosinophilia, 227 patients (60.9%) had identifiable and/or possible causes. The major causes of hypereosinophilia were malignancy (35.2%), allergy and skin diseases (18.1%), infectious diseases (15.4%), hepatobiliary diseases (7.5%), bone marrow clonal diseases (6.6%) and parasite infections (6.6%). We also found a rare case of FIP1L1-PDGFRalpha positive chronic eosinophilic leukemia combined with light chain multiple myeloma. CONCLUSIONS: We found a difference in the distribution of causes of hypereosinophilia in comparison with previous Korean studies, and the most common cause of hypereosinophilia in the current study was malignancy. A rare case of clonal eosinophilia (chronic eosinophilic leukemia) associated with multiple myeloma was confirmed using molecular studies.
Adolescent ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Bone Marrow/pathology ; Child ; Child, Preschool ; Eosinophilia/epidemiology/*etiology/genetics ; Female ; Hospitals, University ; Humans ; Hypereosinophilic Syndrome/epidemiology/*etiology/genetics ; Infant ; Infant, Newborn ; Male ; Middle Aged ; Receptor, Platelet-Derived Growth Factor alpha/genetics/metabolism ; Retrospective Studies ; Sex Factors ; Young Adult ; mRNA Cleavage and Polyadenylation Factors/genetics/metabolism