Pulmonary involvement in idiopathic hypereosinophilic syndrome.
LV Mirchandani, JV Mandke, JM Joshi
Department of Respiratory Medicine, B.Y.L. Nair Hospital, Mumbai.
L V Mirchandani
Department of Respiratory Medicine, B.Y.L. Nair Hospital, Mumbai.
A case of idiopathic hypereosinophilic syndrome (HES) is presented. The patient had been symptomatic and had documented peripheral blood eosinophilia for 9 years. The patients having only pulmonary involvement, seem to have a good prognosis and hence must be considered as a separate subgroup of HES.
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Mirchandani L V, Mandke J V, Joshi J M. Pulmonary involvement in idiopathic hypereosinophilic syndrome. J Postgrad Med 1997;43:23-4
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Mirchandani L V, Mandke J V, Joshi J M. Pulmonary involvement in idiopathic hypereosinophilic syndrome. J Postgrad Med [serial online] 1997 [cited 2019 Nov 22 ];43:23-4
Available from: http://www.jpgmonline.com/text.asp?1997/43/1/23/417
The idiopathic HES represents a heterogeneous group of disorders with the common features of prolonged eosinophilia of an undetectable cause and organ system dysfunction. The idiopathic HES is generally considered to have a poor prognosis, with an average reported survival of nine months and a three year survival of 12%. We are reporting a case of HES with persistent peripheral blood eosinophilia with only pulmonary involvement. The patient has survived for nine years with minimal morbidity.
A 39-year-old man presented with symptoms of dry cough, episodes of paroxysmal dyspnoea and history suggestive of atopy for last nine years. His past records revealed a persistent eosinophilia for which he had received multiple courses of diethylcarbazine and intermittent steroid therapy [Table:1]. Three years ago, he had polydipsia which was diagnosed to be secondary to ‘neurosis’. In January 1994, when the patient first presented to us, his total leucocyte count was 14,600/cumm. His chest radiograph was normal. High resolution computerised tomography (CT) scan of the thorax revealed a diffuse ground glass appearance suggestive of alveolitis [Figure:1]. Pulmonary function tests showed a mild restrictive and obstructive defect with reversibility of 14% in the forced expiratory volume at the end of 1 second (FEV1) following bronchodilators. Bone marrow examination revealed a hypercellular marrow, with erythroid-myeloid ratio of 1:2, presence of megakaryocytes, increase in eosinophilic cell precursors, which was 17% of all nucleated cells; but no blast cells were seen. There were no parasites in the smear. Repeated examinations of stool were negative for any ova or cyst. Blood smears were negative for haemoparasites. Mantoux test and sputum for acid fast bacilli were negative. The electrocardiogram and two dimensional echocardiography were normal. Liver and renal function tests were within normal limits. Ultrasonography of the abdomen was normal. Oesophagogastroduodenal scopy did not reveal any abnormalities. Oesophageal, antral mucosal and colonic biopsies were normal. An open lung biopsy revealed sheets of eosinophils within the alveoli and interstitium with no evidence of vasculitis and granulomas [Figure:2]. This eosinophilic pneumonitis was suggestive of the HES. The patient was treated with prednisolone in the dose of 1 mg/kg/day for eight weeks, which was slowly tapered over six months to a maintenance dose of 10 mg alternate day. The patient has responded to treatment, has normal peripheral blood eosinophil count and continues to followup.
Diagnostic criteria for HES are - Persistent eosinophilia of more than 1500 eosinophils/mm3 for at least six months or death before six months with signs and symptoms of HES2 Lack of evidence of other recognized causes of eosinophilia despite careful evaluation. Signs and symptoms of organ dysfunction either directly related to eosinophilia or unexplained in the given clinical setting,. In addition a distinct syndrome of eosinophilia without any identifiable underlying cause; termed the idiopathic HES is characterized by blood and bone marrow eosinophilia with tissue infiltration by relatively mature eosinophils and multisystem organ dysfunction. The hypereosinophilic syndrome is known by a variety of names including eosinophilic leukemia because of an apparently idiopathic leucocytosis and eosinophilia of a large magnitude associated with hepatosplenomegaly, other organ involvement, and a very poor prognosis, generally leading to a fatal outcome.
In 1969, Benvenisti and Ultmann reported that 39 of 48 patients (81%) had died at one year after diagnosis of HES. Roberts et al reported that 38 of 40 patients had died at two years. A 1975 review by Chusid et al reported an average survival of nine months and a three year survival of only 12%. However, Parrillo et al reported a three year mortality of 4% which they have attributed to the therapeutic regimen employed. They used corticosteroids in most patients and hydroxy urea in steroid resistant patients.
In a recent review, Spry et al have suggested that patients with idiopathic HES should be divided into three subgroups namely. With predominant pulmonary involvement which has the best prognosis and responds well to steroids. With predominant cardiac and/or central nervous system involvement, which has a bad prognosis and Involvement suggestive of eosinophilic leukaemia along with cytogenic abnormalities of the eosinophil. This third group is rare and has a bad prognosis. The case reported here had bone marrow eosinophilia with predominant pulmonary involvement as proved by high resolution CT scan and open lung biopsy. There was no clinical or laboratory evidence of involvement of any other organ system.
Our case indicates a good prognosis in patients of HES with pulmonary and bone marrow involvement only. However, these patients require a careful follow up as they can develop dysfunction of other organs. The inflammatory pulmonary disease can progress to substantial pulmonary fibrosis, most commonly with the development of HES related heart disease. Our experience and the review of literature shows that the prognosis in idiopathic HES depends upon the degree of involvement of vital organs and pulmonary involvement alone have the best outcome.
Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: Analysis of fourteen cases with review of the literature. Medicine (Baltimore), 1975; 54:1-27.|
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