Spectrum of hypereosinophilia syndrome based on 2 clinical case reports

BACKGROUND: Two severe cases of hypereosinophilic syndrome with cardiopulmonary symptoms, erythema nodosum, eosinophilic ileocolitis and paresthesia are reported. Definition, etiology, pathophysiological mechanisms, types, complications and therapeutic approaches of this relatively rare clinical disorder are discussed on the basis of these two cases. The distinction between eosinophilia and hypereosinophilic syndrome is clearly defined. DIFFERENTIAL DIAGNOSIS: The 2 cases of hypereosinophilic syndrome demonstrate the difficulty to decide whether they represent the idiopathic or the reactive type of hypereosinophilic syndrome. If the assumption is made in favor of the reactive type of hypereosinophilic syndrome the assumed prognosis could eventually be too promising. In case of an assumed idiopathic type of hypereosinophilic syndrome the search for causing factors like allergens could be left out. The decision concerning the type of hypereosinophilic syndrome of the 2 cases reported was made with high probability. In the case of idiopathic hypereosinophilic syndrome, eosinophilic leukemia was excluded by bone marrow biopsy. CONCLUSION: In order to be able to make a diagnosis it is necessary to search carefully for causing and influencing factors of the hypereosinophilic syndrome such as allergens or medicaments and to eliminate them. The hypereosinophilic syndrome must be put under frequent control in order to avoid complications or to start with therapeutic approaches in time.     

The hypereosinophilic variant of Ph''-positive chronic myeloleukemia

AIM: To confirm clonal nature of idiopathic hypereosinophilic syndrome (IHES), its relevance to Ph'-positive chronic myeloid leukemia. MATERIALS AND METHODS: 3 cases of idiopathic hypereosinophilic syndrome are reported with morphologic analysis of bone marrow cells and cytogenetic examinations. In one patient the presence of Ph'-chromosome was confirmed at fluorescent in situ hybridization (FISH) and molecular-genetic analysis (bcr/abl). Samples of bone marrow, spleen and liver were examined pathohistologically. RESULTS: The presence of chromosome anomaly t(9;22), i.e. Ph'-chromosome, associated with chronic myeloid leukemia (CML) was identified in all the 3 cases. There was also myeloid hyperplasia in the bone marrow (with primarily mature, eosinophilic granulocytes), spleen and liver, depression of megakaryocyto- and erythropoiesis. 2 patients had similar clinical symptoms which was not typical for CML in chronic phase: fever, elevated ESR, clear-cut anemia and thrombocytopenia. In the absence of hyperleukocytosis, blood and bone marrow eosinophils remained high (42.5, 21.5, 42.5% and 21.4, 7.1, 6.5%, respectively) due to "mature" forms. The number of blasts in the bone marrow was maximum 2.4%. CONCLUSION: The literature and the obtained data suggest closeness of idiopathic hypereosinophilic syndrome and Ph'-positive CML within myeloproliferative diseases.     

The interleukin-5 messenger RNA expression in a patient with idiopathic hypereosinophilic syndrome

Interleukin-5 has a specific role in various eosinophilic activities. It is the predominant cytokine produces by activated T-lymphocytes isolated from patients with idiopathic hypereosinophilic syndrome. We studied a young patient suffering from idiopathic hypereosinophilic syndrome who presented with Horner's syndrome, peripheral neuropathy and skin ulcers. The IL-5 gene expression by CD4+ T-lymphocytes and the peripheral eosinophil count were raised. The skin ulcers continued to deteriorate despite a swift reduction of the IL-5 gene expression and peripheral eosinophil count following systemic corticosteroid treatment. We suggest that peripheral eosinophilia may not be responsible for the damage in skin lesions and more aggressive treatment may be required.     

Incidence of ligament lesions and associated degenerative changes in the elderly wrist

As the carbohydrate lacto-N-fucopentaose III (CD15 antigen or X-determinant) and its sialylated derivative sialyl-Lewis X are involved in the adhesion of cells rolling along the surface of endothelial cells, experiments were done to study the presence of these molecules on human eosinophils from patients with the idiopathic hypereosinophilic syndrome. Normal-density eosinophils from some patients showed higher levels of expression for lacto-N-fucopentaose III than light-density eosinophils. In contrast, sialyl-Lewis X was highly expressed by light-density eosinophils. Activation of normal-density eosinophils with calcium ionophore A23187 resulted in increased expression of these molecules for a short time. Monoclonal antibodies to these carbohydrates stimulated eosinophils to secrete eosinophil cationic protein, but not eosinophil peroxidase, and acted as costimulatory signals for C3b-induced degranulation of eosinophil cationic protein. It was suggested that CD15 and sialyl-Lewis X might contribute to eosinophil-mediated tissue injury in patients with the idiopathic hypereosinophilic syndrome.     

Eosinophilic pulmonary arteritis in a full-term fetus with hypereosinophilia

The case refers to a term-fetus, small-for-date, with polydramnios, idiopathic hypereosinophilic syndrome and eosinophilic vasculitis of large, medium and small-size pulmonary arteries. The pulmonary arteritis was the only lesion observed in the fetus.     

Prostate cancer masquerading as a zygomatic posttraumatic mass

INTRODUCTION: Idiopathic hypereosinophilic syndrome is an uncommon disease often associated with diverse non-specific skin manifestations. Mucosal ulcerations suggest a myeloproliferative from with poor prognosis due to possible progression to malignant hemopathy or visceral complications. CASE REPORT: A 28-year-old man presented idiopathic hypereosinophilia with isolated mucosal ulcerations involving the buccal and genital areas. Laboratory results (hematology, CD25) suggested a myeloproliferative form. Treatment with alpha interferon (18 months) led to regression of the mucosal lesions and a decrease in the markers of eosinophil toxicity. There was no visceral involvement. DISCUSSION: Immunosuppression with/without high-dose alpha interferon is usually used for the treatment of hypereosinophilic syndrome. In our case favorable outcome was obtained with lower doses of alpha interferon than those reported in the literature. There was objective decrease in eosinophil toxicity (regular counts of hypodense eosinophils, CD25 or interleukin 2 soluble receptor) and no progression (malignant hemopathy, mortal visceral involvement).     

Cine-MRI-aided endomyocardectomy in idiopathic hypereosinophilic syndrome

The idiopathic hypereosinophilic syndrome is a leukoproliferative disorder marked by a predilection to damage specific organs, including the heart. This report describes a patient with extensive endocardial fibrosis accompanying this syndrome. Right ventricular endomyocardectomy with preservation of the tricuspid valve was performed. The procedure was aided by cine-magnetic resonance imaging for preoperative assessment and follow-up of surgical results.     

Advised follow-up after emergency treatment of adolescents with violence-related injuries

INTRODUCTION: The finding of eosinophilic ascites is unusual. It requires the search for the main etiology, i.e., parasitic or malignant disease, vasculitis or hypereosinophilic syndrome. The diagnosis of exclusion is either mucosal, muscular or serous eosinophilic gastroenteritis. This last type, the most unusual--as less than 50 cases have been documented until now--is associated with eosinophilic ascites. EXEGESIS: We report a new case of serous eosinophilic gastroenteritis that occurred in a 23-year-old woman. This case was unusual because of its clinical history, as abdominal pain fits (along with the occurrence of ascites) were associated with urticaria fits. The lack of eosinophils in both the blood counts and the various digestive biopsies were unusual too. The disease evolution was favorable with corticosteroid therapy; however, a minimal dose of 8 mg/day was necessary to control the disease symptoms. CONCLUSION: Because of its association with urticaria fits, this case emphasizes the need for differential diagnosis in patients with hypereosinophilic syndrome and food allergy.     

Idiopathic hypereosinophilic syndrome with eosinophilic myositis, peripheral neuropathy and central nervous system involvement

Idiopathic hypereosinophilic syndrome (HES) is a rare disorder marked by a sustained overproduction of eosinophils and a predilection for damage to multiple organ systems. Its neurologic involvement ranges from the central to the peripheral nervous system, and can be associated with eosinophilic myositis. We report a 68-year-old woman who had eosinophilia, eosinophilic dermatitis and eosinophilic pneumonia. She also suffered from numbness and weakness of the lower limbs. Because of long-lasting (> 6 mo) eosinophilia (> 1.5 x 10(9)/L) in the peripheral blood and the fact that no other underlying causes of eosinophilia and neurologic involvement could be identified, a diagnosis of idiopathic hypereosinophilic syndrome was made. The muscle biopsy showed infiltration of inflammatory cells, including a few eosinophils (Liu's stain). Magnetic resonance images, motor evoked potentials, somatosensory evoked potentials and nerve conduction velocities also showed abnormalities in the central and peripheral nervous systems. The pathogenesis and treatments of HES are discussed in this report.     

Development of a scoring system to assess disability in an Italian elderly population. Evaluation of a disability questionnaire

Patients with idiopathic and symptomatic restless legs syndrome (RLS) suffer from "dyskinesia while awake" or "daytime myoclonus" when at rest preceded by sensory symptoms. In order to characterise the RLS either as reflex movement or as voluntary movement we measured movement-related cortical potentials in 5 idiopathic and 8 uraemic RLS patients. Movements from both legs were polygraphically recorded concomitantly with cortical activity 2000 msec before to 500 msec after onset of EMG activity. These data were compared with a voluntary simulation of each patient's movement pattern and with 5 age-matched controls performing dorsiflexion of the right, left and both feet. Cortical activity preceding daytime myoclonus was absent in RLS patients whereas self-initiated leg movements in patients elicited onset times (1180-1380 msec) and amplitudes of Bereitschaftspotential (readiness potential) not significantly different from readiness potentials in control subjects (P > 0.05). Lack of movement-related potentials in myoclonus and/or dyskinesias during daytime in RLS patients is compatible with an involuntary mechanism of induction and points towards a subcortical or spinal origin of RLS.     

Reduction of pleural fluid complement activity in patients with systemic lupus erythematosus and rheumatoid arthritis

In this paper the author gives a survey and a classification of 642 cases of narcolepsy and hypersomnia which he himself studied in the course of 26 years. 368 cases were classified as narcolepsy, 274 as hypersomnia. The author further classifies narcolepsies according to their etiology, clinical form and pathophysiological mechanisms of origin. Hypersomnias are divided by the author into the symptomatic and the functional groups. According to the author it is useful to distinguish "short cycle hypersomnia", i.e. those with short duration of sleep attacks (hours) and intervals, from "long cycle hypersomnia", i.e. those with long attacks (days or weeks) and intervals. The author goes on to describe different forms of symptomatic and functional hypersomnias, such as idiopathic hypersomnia, neurotic hypersomnia, the Pickwickian syndrome" and its variants as well as different varieties of periodic long cycle hypersomnias. Finally the author makes a brief mention of the syndrome of insufficiency of wakefulness.     

Raynaud syndrome complicated by digital gangrene during treatment with interferon-alpha

In a 43-year old male suffering from idiopathic hypereosinophilic syndrome (HES) since 1984, successfully treated with alpha interferon (alpha IFN) for 32 months, a severe Raynaud's phenomenon of the four extremities occurred and eventually evolved into digital necrosis within a few weeks. The arterial echography/doppler and plethysmography patterns were suggestive of isolated small-to medium-size digital artery occlusions. An extensive search for an aetiology of digital necrosis, including complete tests of autoimmunity, remained negative. Two months later, despite alpha IFN withdrawal and intravenous infusions of ilomedin, the digital ischemia evolved to extensive necrosis that necessitated several amputations and a definitive spinal chord stimulation. Pathologic examination of arteries showed no vasculitis but diffuse arterial occlusions by thrombi.     

Prenatal etiologies of West syndrome

We investigated the etiology of West syndrome (WS) with special reference to prenatal factors in 180 cases. Prenatal cause was the most frequent diagnosis (77 cases, 42.8%), followed by perinatal (25 cases, 13.9%) and postnatal factors (12 cases, 6.7%); 48 cases (26.7%) were of uncertain etiology; eighteen cases (10.0%) were idiopathic. Of the three forms of age-dependent epileptic encephalopathy, prenatal cause was present in 12 of 15 cases (80.0%) of early-infantile epileptic encephalopathy with suppression-burst, 77 of 180 cases (42.8%) of WS, and 31 of 123 cases (25.2%) of Lennox-Gastaut syndrome (LGS). Prenatal factors of WS included tuberous sclerosis (23), chromosome abnormalities (10), cerebral dysgenesis (10), porencephaly (7), hydrocephalus (5), Aicardi syndrome (3), Aicardi syndrome associated with chromosome abnormality (1), and other causes (18). Chromosome abnormalities with WS consisted of 6 cases with 21 trisomy and one case each with 18q duplication, t(1;y) translocation, 7q duplication, and partial 2p trisomy. One patient with Aicardi syndrome also had a t(12;21) translocation. No significant difference was observed in the age of onset of WS among the five etiologic groups. The evolution from WS to LGS was not influenced by etiology, except for the idiopathic group. In patients followed for over 3 years, seizure remission occurred in 46.8% (22 of 47 cases) of the prenatal group. This was lower than the other four groups. Intellectual prognosis was also relatively poor in those with prenatal onset. Pyridoxal phosphate (PAL-P) treatment was effective in 9 of 70 (12.9%) prenatal cases and 5 of 18 (27.8%) idiopathic cases.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pharmacokinetics of pamidronate disodium in patients with cancer with normal or impaired renal function

Among the variable manifesting conditions of neuronal migration disorders, mental retardation, motor disturbance and epilepsy are the main features of developmental disabilities. We analyzed the relationship between clinical symptoms and magnetic resonance (MR) images, including surface anatomy scan (SAS). Thirty nine patients (23 males, 16 females; mean age 6.1 years) with neuronal migration disorders were studied. The diagnoses were cerebral palsy in 23 cases, mental retardation in 4. West syndrome in 4, Fukuyama type congenital muscular dystrophy (FCMD) in 6. Walker-Warburg syndrome in 1 and Dubowitz syndrome in 1. Cortical dysplasias were classified into the following 7 groups, mainly based on the SAS findings: complete agyria (AG 1), mixture of agyria and pachygyria (AG 2), bilateral complete pachygyria (BP 1), diffuse pachygyria with marked widening of the bilateral superior frontal gyrus (BP 2), unilateral pachygyria with hemispheric atrophy or hemimegalencephaly UP), focal cortical dysplasia (FP) and other findings such as solitary schizencephaly (Others). Most cases of AG 1 and AG 2 showed spastic quadriplegia (6/7) and symptomatic generalized epilepsy (5/7), whereas cases of BP1 showed spasticity only in 1/8 and epilepsy in 7/8. Hemiplegia was observed in 6/7 of UP, 2/8 of FP and 2/4 of Others. Partial epilepsy was observed in 2/7 of UP and 1/8 of FP. Intellectual level was variable in BP 1, UP, FP and Others, but all cases showed severe mental retardation in AG 1, AG 2 and BP 2. BP 2 was observed in all cases of typical FCMD (5/5). The birth weight was less than 2,500 g in 6/7 of UP. The structural findings well correlated with clinical symptoms and epileptic seizure types. The surface anatomy scan was a very useful technique for detecting cortical dysplasias.     

Association of precipitating anti-adrenal anti-adrenal antibodies with moniliasis-polyendocrinopathy syndrome

The association of precipitating anti-adrenal antibodies with different subgroups of idiopathic Addison's disease were studied. We had previously found these antibodies in patients with the moniliasis-polyendocrinopathy syndrome. Sera of 36 adult patients suffering from different froms of Addison's disease were examined for the presence of adrenal antibodies demonstrable either by immunofluorescence (IFL) or by gel diffusion. 3 of the 17 patients with tuberculous and 17 of 19 patients with idiopathic Addison's disease had IFL antibodies but only one had precipitating antibodies. There was one typical case of Schmidt's syndrome, and four additional cases with Addison's disease combined with diabetes or thyroiditis, who may later develop the syndrome. None of htese patients had precipitating anti-adrenal antibodies. The only patients with precipitating adrenal antibodies had the moniliasis-polyendocrinopathy syndrome. He was not typical as Addison' disease appeared unusually late and he did not have hypoparathyroidism. The presence of precipitating anti-adrenal antibodies in this patient, and the absence of these in other groups of Addison's disease, is further evidence for the association of precipitating antibodies with the moniliasis-polyendocrinopathy syndrome.     

The problems in the use of superficial cervical artery skin flap and it''s relevant solution]

In six patients aged 59-71 years carpal tunnel syndrome, seemingly idiopathic, was followed by connective tissue disorders, in most cases autoimmune in nature. Patients with idiopathic carpal tunnel syndrome may therefore require long-term follow-up, if inflammatory rheumatic conditions are not to be missed.     

Endomyocardiopathy with eosinophilia

Five patients were seen at the Mayo Clinic over an 8-year period with the following complex of clinical and morphologic features; striking eosinophilia, cardiomyopathy, hepatosplenomegaly, and either a rapidly fatal or a prolonged, debilitating illness. In recent years, controversy has raged over the precise designation of this syndrome, with proposals ranging from eosinophilic leukemia to hypereosinophilic syndromes. To focus on the major target organ of the disease, we have favored the term endomyocardiopathy with eosinophilia. Experience with these five patients showed that (1) eosinophilia can persist for many years before symptoms appear; (2) progressive restrictive cardiac disease was the major cause of death and debility; (3) osmiophilic cytoplasmic inclusions are present in eosinophils of these patients and also in cells from other patients with marked eosinophilia; and (4) echocardiography may prove to be a useful noninvasive tool to diagnose and follow the progress of cardiac involvement. Although none of these patients was thought to have leukemia, intensive therapy with steroids or cytotoxic agents, or both, is considered necessary to control the progression of the disease.     

Eosinophilic cholecystitis, appendiceal inflammation, pericarditis, and cephalosporin-associated eosinophilia

A patient with eosinophilic cholecystitis and accompanying eosinophilic appendiceal inflammation, eosinophilic pericarditis, and peripheral eosinophilia is described. Review of the nine previously reported cases of eosinophilic cholecystitis suggests that this is the first case with closely associated eosinophilic appendiceal inflammation and pericarditis as manifestations of a systemic hypereosinophilic syndrome. The possible etiologic role of cephalosporin hypersensitivity is discussed.     

Hyperimmunoglobulinemia D or periodic fever syndrome. Cutaneous manifestations in 3 cases

INTRODUCTION: We describe 3 cases of hyperimmunoglobulinemia D syndrome (hyper-IgD syndrome). CASES REPORT: As the 2 first cases are conventional with an onset in childhood, the third one appeared later suggesting possibility of acquired syndrome. The 3 cases presented with one or several skin eruptions during febrile attacks. COMMENTS: Up to now, 64 cases of this syndrome have been described. Skin lesions are observed in 80 p. 100 of patients. In this particular clinical context, they must lead to the diagnosis. This 3 cases and the review of literature show that skin manifestations are polymorphic, transient, not very symptomatic and not correlated to IgD rates. Histological examination may reveal urticarial reaction with leucocytoclasic vasculitis, where IgD could directly or not interact.