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ALPHA‐INTERFERON IN A CASE OF HYPEREOSINOPHILIC SYNDROME
Author(s) -
Van Den P. J.,
Van 'T Veer M. B.
Publication year - 1991
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1991.tb07993.x
Subject(s) - hypereosinophilic syndrome , medicine , alpha interferon , immunology , eosinophilia , interferon
We have read with interest the report of Murphy et a1 (1990) on the use of alpha-interferon (a-IFN) in a case of hypereosinophilic syndrome (HES). Their patient with severe HES showed a dramatic clinical and haematological improvement with the introduction of recombinant a-IFN. However, follow-up was only 3 months. We also treated a patient with HES using a-IFN (Essex). This led to a good response. However, in a longer follow-up we observed that the response lasted only 6 months. In a 43-year-old man HES was diagnosed in October 198 7 according to the diagnostic criteria of HES as outlined by Chusid et a1 (1975). His initial evaluation revealed a white blood count (WBC) of 11 3 x 10y/l with 90% eosinophils. 1% polymorphonuclear leucocytes, 8% lymphocytes and 1 % monocytes. The bone marrow examination showed hypercelM a r marrow with a marked predominance of eosinophilic precursors (77%). There was no excess of blast cells. The neutrophil alkaline phosphatase score and karyotype were normal. His spleen was enlarged and in the lungs bilateral transient infiltrations were found. Renal function was decreased. An echocardiogram revealed a restrictive cardiomyopathy with pulmonal hypertension and tricuspid-regurgitation. The patient had no HLA-identical sibling donor. He was initially treated with hydroxyurea and prednisone. without further clinical or haematological improvement. As his condition worsened, IFN was commenced in October 1988. He received 5 x 10" units subcutaneously daily. In the first 4 months of IFN therapy the WBC decreased from 120 to 10.6 x 109/1 with 90% eosinophils and his condition improved dramatically. The congestive heart failure was controlled by diuretics and afterload reduction. There was stable disease until March 1989. Then IFN was stopped because of progression of the HES. Hydroxyurea was started again, but the WBC rose to 126 x 10y/l. He developed progressive congestive heart failure due to thickening of the mural endocardium of the right ventricle and tricuspid valve destruction. Endomyocardial biopsy showed marked fibrosis in the subendocardium. Despite continued activity of the underlying disease, he had cardiac surgery in February 1990. The mitral and tricuspid valves were replaced and thrombotic masses were removed from the right ventricle. His clinical condition improved dramatically after the surgery. Unexpectedly he died of cardiorespiratory failure in April 1990. HES is an extremely rare disease. Our experience shows that IFN has a place in the treatment of HES and we think it has to be given early in the treatment. However, the haematological response may only be short, as in our patient, although every response may postpone the tissue damage which makes this disease fatal.