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CORRESPONDENCE
2284
a-Interferon and Hypereosinophilic SyndromeWith Trisomy 8: Karyotypic Remission
To the Editor:
Described in 1975 by Chusid et al,’ the hypereosinophilic syndrome (HES) is defined by a persistent eosinophilia (>1.5 X lo9/
L) of unknown cause associated with widespread organ involvement. In fact, HES is heterogenous group of diseases of variable
severity. The similarity between HES and other myeloproliferative
syndromes such as chronic myeloid leukemia (CML) has been
noted in the past.’ It is now well established that a-interferon (aI F N ) can induce hematologic and karyotypic responses in CML.3
On the other hand, therapeutic effects of a-IFNhave been recently
described in a few cases of HES.4-s We report the first case
of karyotypic remission in a HES with trisomy 8 induced by
a-IFN.
A 45-year-old man was admitted in April 1993 with a 4-month
history of pulmonary infiltrate, fever, and weight loss of 10 kg.
Physical examination was normal. Hemoglobin was 124 g&, platelets, 150 X lo9& and white blood cell count, 31.4 X 109L with
15% segmented neutrophils, 70% eosinophils, 1% basophils, 5%
lymphocytes, 7% monocytes, 1% metamyelocytes, and 1% myelocytes. The distribution of peripheral blood eosinophils on metrizamide gradients showed a very high level of hypodense eosinophils.
Level of serum interleukin-2 receptor was high, ie, 19,835 I U L
The leukocyte alkaline phosphatase score was 21 (normal range, 17
to 80); serum vitamin B12 was more than 2,000 pg/mL. Bone marrow aspirate was hypercellular, with 51% of eosinophils being precursors, with absence of an excessive number of blasts. Trephin
biopsy specimen showed eosinophilic hyperplasia without myelofibrosis. Cytogenetic studies performed on bone marrow showed 47
XY with trisomy of chromosome 8 in 12 of 18 metaphases. Stool
stamples were negative for ova and parasite. Left basal parenchymal
densities were found on chest x-ray film. Electromyogram showed
acute and chronic denervation of extremities and a two-dimentional
echocardiogram detected a diminished motion of the posterior mitral
leaflet.
The patient was initially treated with prednisone (1 mg/kg/d) and
hydroxyurea (2 g/d). During the next 3 months, the patient remained
asymptomatic with moderate eosinophilia (5.56 X 109/L).Nevertheless, the disease progressed, and in July the patient had severe mitral
regurgitation and underwent a mitral valve remplacement. Prednisone was stopped, and a-IFN was started (3 million U three times
a week) in combination with hydroxyurea (1 g/d). Eight months
later, the patient was ambulatory, and the leukocyte count was 4.7
X 109L with 752 eosinophilslpL. The bone marrow and the karyotype were normal. The level of serum interleukin-2 receptor decreased to 1,473I U L Echocardiogram was normal, and neuropathy
was improved.
The distinction among HES, eosinophilic leukemia, and CMLlike diseases is difficult in some patients. Different cytogenetic abnormalities have been described in HES9such as trisomy 8, Philadelphia translocation, abnormality in 1 2 ~ 1 3 ,andmonosomy 7 and
i(17q). None of these chromosomal aberrations are specific to the
syndrome, and some patients had abnormalities observed in leukemias, ie, t(8,21)(q22;q22), +8, -7, 14q+. In the case described
here, trisomy 8 was associated with features common to myeloproliferative disorders. In CML, a-IFW can induce karyotypic remission’
and prolong survival. Recently, a-IFN has been used to treat several
patients with HES4-’ This treatment was effective in patients resistant to conventional therapy. a-IFN was generally well tolerated,
and patients were able to discontinue or taper CorticosteroYds or
hydroxyurea? However, progression of HES despite continued therapy and rapid rebound of eosinophilia after cessation of a-IFN has
been r e p ~ r t e d . Among
~.~
the 27 patients with HES treated with aIFN, only 5 of them had reported cytogenetic studies. These chromosomal analyses were normal in all the patients except 1 who had a
trisomy 8 and died because of no response to a-IFN and other
chemotherapy.’ We report here the first case of HES with trisomy
8 in a clinical and cytogenetic remission after a-IFN treatment. It
suggests that a-IFN is a very effective agent and should be used as
a first-line treatment in patients with myeloproliferatif variant of
HES.
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
CORRESPONDENCE
2285
I. Quiquandon
Department of Haematology
J.F. Claisse
J.C. Capiod
J. Delobel
Laboratory of Haematology
L. Prin
Laboratory of Immunology
CHRV
Amiens. France
REFERENCES
1. Chusid MJ, Dale DC, West BC, Wolff S M The hypereosinophilic syndrome: Analysis of fourteen cases with review of the
literature. Medicine 5:1, 1975
2. Goh KO, Swisher SN, Rosenberg CA: Cytogenetic studies in
eosinophilic leukemia. The relationship of eosinophilic leukemia and
chronic myelogenous leukemia. Ann Intern Med 62:80, 1965
3. Talpaz M, Kartajian I", McCredie D,Keating MJ, Trujillo
J, Gutterman JU: Clinical investigation of human alpha interferon
in chronic myelogenous leukemia. Blood 69:1280, 1987
4. Murphy PT, Fennely DF, Stuart M, ODonnel JR: Alpha-interferon in a case of hypereosinophilic syndrome. Br J Haematol
75:619, 1990
5. Van den Anker-Lugtenburg PJ, van? Veer MB: Alpha-interferon ina case of hypereosinophilic syndrome. Br J Haematol
77:258, 1991
6. Coutant G , Blktry 0, Prin L, Hauteville D, De Puyfontaine 0,
Abgrall JF, Godeau P: Traitement des syndromes hyperkosinophiexpression
I
myeloprolifkrativepar I'association hydroxyheliques ?
interfkron alpha. Ann Mkd Intern 144:243, 1993
7. Kobayashi M, Katayama T, Ochiai S, Yoshida M, Kaito K,
Masuoka H, Shimada T, Nishiwaki K, Saikai 0: Interferon-alpha
therapy in the myeloproliferative variants of hypereosinophilic syndrome. Rinsho-Ketsueki 34:367, 1993
8. Butterfield JH, Gleich GJ: Interferon alpha treatment ofsix
patients with the idiopathic hypereosinophilic syndrome. Ann Intern
Med 121648, 1994
9. Da Silva MAP, Heerema N, Scwenk GR, Hoffman R: Evidence
for the clonal nature of hypereosinophilic syndrome. Cancer Genet
Cytogenet 32:109, 1988
From www.bloodjournal.org by guest on June 14, 2017. For personal use only.
1995 85: 2284-2285
alpha-Interferon and hypereosinophilic syndrome with trisomy 8:
karyotypic remission [letter]
I Quiquandon, JF Claisse, JC Capiod, J Delobel and L Prin
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