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Journal of Clinical Oncology, Vol 26, No 26 (September 10), 2008: pp. 4355-4356 © 2008 American Society of Clinical Oncology. DOI: 10.1200/JCO.2008.16.2933
Sweet Syndrome Associated With Granulocyte Colony-Stimulating FactorDepartment of Internal Medicine, University of Texas Medical Branch, Galveston, TX
Department of Oncology, University of Texas Medical Branch, Galveston, TX A 72-year-old white female with newly diagnosed myelodysplastic syndrome (MDS) with secondary pancytopenia and past medical history of hypertension, coronary artery disease, and peripheral vascular disease was started on granulocyte colony-stimulating factor (G-CSF) (filgrastim, 480 µg subcutaneously) daily for 7 days for management of her neutropenia. Subsequently, she received first cycle of azacitidine for MDS followed by daily and then weekly dose of G-CSF along with epoetin alfa for marrow recovery. At a time when the peripheral blood count was recovering and neutropenia had resolved, there was sudden development of bilateral conjunctivitis with surrounding periorbital erythematous lesions, erythema on her ears, and bilateral erythematous, pruritic, painful, papular lesions on the dorsal hands (Fig 1). There was no documented fever. The neutrophil count was 2.9 x 103 with absolute neutrophil count of 1800/µL. Erythrocyte sedimentation rate and C-reactive protein were not measured. A skin biopsy from the dorsum of the hand showed dense neutrohilic infiltrate in the dermis with karyorhexis and prominent dermal edema without vasculitis consistent with Sweet syndrome (SS; Fig 2). The eye symptoms were thought to be consistent with conjunctivitis. G-CSF was discontinued and she was started on a low dose of prednisone, 10 mg/d. There was a prompt resolution (5 to 7 days) of the skin lesions along with a significant improvement in the eye symptoms and periorbital lesions.
SS is the prototype of neutrophilic dermatosis characterized by acute onset of fever, leukocytosis, erythematous plaques and nodules, which may be recurrent, and a dense infiltrate of neutrophils, primarily in the dermis without vasculitis.1,2 SS is associated with a variety of disorders, including hematological malignancies and solid tumors, autoimmune diseases, inflammatory bowel disease, infections, medications, and pregnancy.2,3 SS is a recognized, although uncommon, complication of treatment with G-CSF. Drug-induced SS is a more recently defined clinical classification of SS in addition to classic or idiopathic SS and malignancy-associated or paraneoplastic SS. The diagnostic criteria proposed by Walker and Cohen4 include (1) acute onset of painful erythematous plaques or nodules, (2) histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis, (3) pyrexia greater than 38°C, (4) temporal relation between the drug ingestion and clinical presentation (5 to 7 days after ingestion5) or temporally related recurrence after oral rechallenge, and (5) temporally related resolution of lesions after drug withdrawal or treatment with systemic steroids.2,4 The pathogenesis of SS is not clearly understood. The current hypothesis suggests that it is related to altered immunologic reactivity, potentially involving cytokines like interleukins (IL-1, IL-3, IL-6, and IL-8), G-CSF, granulocyte macrophage colony-stimulating factor, and interferon gamma.6,7 G-CSF is the most commonly associated agent with drug-induced SS. G-CSF is known to induce stem cell proliferation; differentiation of neutrophils, and neutrophil survival8,9 G-CSF is thus responsible for neutrophil skin accumulation and seems to show a dose-dependent effect in provoking SS.10 Other medications associated with SS are all-trans-retinoic acid, carbamazepine, celecoxib, diazepam, diclofenac, hydralazine, levonorgestrel/ethinyl estradiol, minocycline, nitrofurantoin, propylthiouracil, trimethoprim–sulfamethoxazole, and furosemide.11,12 In our case report there was no documented fever, but the lesions were located in the expected areas (upper extremities). What seems to be unique to our case is that the dose of prednisone required to treat her SS was much lower than the ones described in the literature (30 to 60 mg orally per day for 6 to 8 weeks13) despite her extensive skin involvement. In our patient, we discontinued the G-SCF and started on low-dose of prednisone (10 mg/d); there was a dramatic improvement in her symptoms, and skin lesions almost cleared in 1 week, when the dose was changed to 5 mg/d prednisone and then 5 mg on alternate days. She received prednisone for a total of two weeks. She also received her second cycle of azacytidine, and her peripheral blood counts have been improving, so there has been no indication to use G-CSF. In case there is further need of G-CSF use, we plan to give it concurrently with oral prednisone to prevent recurrence of drug-induced SS, although we know that patients may not necessarily experience SS relapse with further exposure to G-CSF.14 AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST Although all authors completed the disclosure declaration, the following author(s) indicated a financial or other interest that is relevant to the subject matter under consideration in this article. Certain relationships marked with a "U" are those for which no compensation was received; those relationships marked with a "C" were compensated. For a detailed description of the disclosure categories, or for more information about ASCO's conflict of interest policy, please refer to the Author Disclosure Declaration and the Disclosures of Potential Conflicts of Interest section in Information for Contributors. Employment or Leadership Position: None Consultant or Advisory Role: Avi B. Markowitz, Amgen (C), Genentech (C) Stock Ownership: None Honoraria: Avi B. Markowitz, Amgen Research Funding: Avi B. Markowitz, Amgen, OrthoBiotech, Bristol-Myers Squibb Co, Johnson and Johnson Pharmaceutical Research and Development LLC Expert Testimony: None Other Remuneration: None Editor's Addendum—See Vance E, Granter S, Skarin A: Sweet Syndrome. J Clin Oncol 15:860-861, 1997
REFERENCES 1. Sweet RD: An acute febrile neutrophilic dermatosis. Br J Dermatol 76:349-356, 1964[Medline] 2. Cohen PR, Kurzrock R: Sweet's syndrome: A neutrophilic dermatosis classically associated with acute onset and fever. Clin Dermatol 18:265-282, 2000[CrossRef][Medline] 3. Park JW, Mehrotra B, Barnett BO, et al: The Sweet syndrome during therapy with granulocyte-colony stimulating factor. Ann Intern Med 116:996-998, 1992[Medline] 4. Walker DC, Cohen PR: Trimethoprim-sulfamethoxazole–associated acute febrile neutrophilic dermatosis: Case report and review of drug-induced Sweet's syndrome. J Am Acad Dermatol 34:918-923, 1996[CrossRef][Medline] 5. Paydaçs S, Berksoy S, Seyrek E, et al: Sweet's syndrome associated with GCSF. Br J Haematol 85:191-192, 1993[Medline] 6. Reuss-Borst MA, Muller CA, Waller HD: The possible role of G-CSF in the pathogenesis of Sweet's syndrome. Leuk Lymphoma 15:261-264, 1994[Medline] 7. Reuss-Borst MA, Pawelec G, Saal JG, et al: Sweet's syndrome associated with myelodysplasia: Possible role of cytokines in the pathogenesis of the disease. Br J Haematol 84:356-358, 1993[Medline] 8. Prevost-Blank PL, Shwayder TA: Sweet's syndrome secondary to granulocyte colony-stimulating factor. J Am Acad Dermatol 35:995-997, 1996[CrossRef][Medline] 9. Kawakami T, Ohashi S, Kawa Y, et al: Elevated serum granulocyte colony-stimulating factor levels in patients with active phase of Sweet syndrome and patients with active Behcet disease: Implication in neutrophil apoptosis dysfunction. Arch Dermatol 140:570-574, 2004 10. Richard MA, Grob JJ, Laurans R, et al: Sweet's syndrome induced by granulocyte colony-stimulating factor in a woman with congenital neutropenia. J Am Acad Dermatol 35:629-631, 1996[CrossRef][Medline] 11. Govindarajan G, Bashir Q, Kuppuswamy S, et al: Sweet syndrome associated with furosemide. South Med J, 98:570-572, 2005[CrossRef][Medline] 12. Cohen PR, Kurzrock R. Sweet's syndrome revisited: A review of disease concepts. Int J Dermatol. 42:761-778, 2003 13. Cohen PR, Kurzrock R: Sweet's syndrome: A review of current treatment options. Am J Clin Dermatol 3:117-131, 2002[CrossRef][Medline] 14. Hasegawa M, Sato S, Nakada M, et al: Sweet's syndrome associated with granulocyte colony-stimulating factor. Eur J Dermatol 8:503-505, 1998[Medline]
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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