Hypereosinophilic syndrome: Long-term remission following allogeneic stem cell transplant in spite of transient eosinophilia post-transplant

Abstract

A 38-year-old male with progressive myeloproliferative variant of hypereosinophilic syndrome (HES) underwent allogeneic bone marrow transplantation from a matched unrelated donor. The preparative regimen consisted of TBI, cytarabine, and cyclophosphamide. The graft was T-cell-depleted. The patient had slow, but complete, hematologic recovery, and all cells were shown by VNTR analysis to be of donor origin. Five months after transplant, the patient developed prominent eosinophilia (peak 4.1 · 10 9 /L) with dermatographism and very high IL-5 levels. Eosinophils isolated to purity by cell sorting were all of donor origin. Mild increase in immunosuppression led to a normalization of eosinophil count after about 6 months. The patient is now 6 years after transplant, off all medications, and without evidence of disease. Allogeneic stem-cell transplantation is a potentially curative therapy for HES. Am. J. Hematol. 78:33-36, 2005. ª 2004 Wiley-Liss, Inc.Key words: hypereosinophilic syndrome; HES; allogeneic stem cell transplant; long-term remission; transient eosinophilia Hypereosinophilic syndrome (HES) is a poorly defined disorder, or more likely group of disorders, characterized by a sustained overproduction of eosinophils [1,2]. The prominent eosinophilia can be reactive or neoplastic in origin and may lead to organ damage [1,2]. Allogeneic stem-cell transplantation has been reported to be potentially curative in this disorder, and a number of case reports have been published [3][4][5][6][7][8][9][10][11][12][13]. Many of these have a rather limited follow-up, and a late relapse at 40 months has been reported [7]. Herein we report on a patient with HES of probably myeloproliferative origin who has been in complete remission of his disease for more than 6 years. CASE REPORTA 38-year-old Caucasian male presented with a 6-year history of eosinophilia (20,000/mm 3 ), itching, and persistent dry cough. Initially he had been treated for 1 year with hydroxyurea, prednisone, cyclosporine, and interferon without obvious effect. He moved out of the country for 5 years and was without medical care or medication. He then moved back home and presented with prominent splenomegaly (extending beyond the midline), hemoglobin 8.8 g/dL, platelets 116 Â 10 9 /L, and WBC 27 Â 10 9 /L with 75% mature eosinophils. The bone marrow biopsy showed 100% cellularity, mild reticulin fibrosis, and marked increase in mature eosinophils and eosinophilic precursors, all with prominent dyspoiesis. Blast cells were not increased, nor were CD34 + cells on immunohistochemistry. Cytogenetic analysis of unstimulated bone marrow cells showed a normal karyotype. The patient was treated with hydroxyurea in combination with interferon. He developed skin lesions, bone pains, progressive anemia (transfusions became necessary 8 months after the start of therapy in spite of

Eosinophilia: secondary, clonal and idiopathic

“…Otherwise, additional treatment experience is needed to enable choosing one of the remaining agents over the other. Finally in drug‐refractory HES, myeloablative and non‐myeloablative allogenic peripheral blood stem cell transplants have been used and were found to reverse organ dysfunction (Juvonen et al , 2002; Ueno et al , 2002; Cooper et al , 2005).…”

Modern Diagnosis and Treatment of Primary Eosinophilia

“…Recent additions to this list of therapeutic approaches include imatinib [12, 31, 41, 54, 55, 57,115,116,117,118,119,120,121,122,123,124], mepolizumab (monoclonal antibody to IL-5) [39, 54,125,126,127], alemtuzumab (monoclonal antibody to CD52) [128,][129], and allogeneic hematopoietic stem cell transplantation (AHSCT) [130,131,132,133,134,135,136,137,138,139]. With regards to imatinib therapy, it is currently not clear if ‘HES’ patients previously reported to have achieved complete remission represented true HES cases [57, 73,115,116,117,118,119,120, 122, 123, 140].…”

Hematopoietic cell transplantation for chronic myeloproliferative disorders

“…Anecdotal cases of successful HCT for other chronic disorders such as mastocytosis, chronic neutrophilic leukemia, or hypereosinophilic syndrome have also been reported [3,4,15,21,33]. These reports show proof of principle, although it would be premature to draw firm conclusions from few reported cases.…”