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Year : 2012  |  Volume : 8  |  Issue : 4  |  Page : 652-653

Immunoglobulin a multiple myeloma associated with sweet syndrome

Department of Hematology, CHU Mohamed VI, Cadi Ayyad University, Marrakech, Morocco

Date of Web Publication29-Jan-2013

Correspondence Address:
I Tazi
Department of Hematology, CHU Mohamed VI, Cadi Ayyad University, Marrakech
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.106591

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How to cite this article:
Tazi I, Nafil H, Mahmal L. Immunoglobulin a multiple myeloma associated with sweet syndrome. J Can Res Ther 2012;8:652-3

How to cite this URL:
Tazi I, Nafil H, Mahmal L. Immunoglobulin a multiple myeloma associated with sweet syndrome. J Can Res Ther [serial online] 2012 [cited 2022 Sep 30];8:652-3. Available from: https://www.cancerjournal.net/text.asp?2012/8/4/652/106591

Dear Sir,

In May 2011, a 57-year-old Moroccan female was referred to the department of hematology in Marrakech because of symptomatic anemia. She complained of bony pain in her back, hips, and shoulders. She received treatment with nonsteroid antirheumatics, but without effect. Her past medical history was insignificant. Physical examination disclosed pallor, painfulness of spine pressure. Laboratory investigations showed a hemoglobin level of 8 g/dL (normal range: 12 to 16), mean cell corpuscular volume (MCV) of 88 fL (80 to 98), white cell count (WBC) of 3,500/L (4,000 to 10,000/L), and platelets of 124,000/L (150,000 to 300,000/L). Erythrocyte sedimentation rate was 94 mm/h (0 to 16). Monoclonal spike was found in the betaglobulin region of the serum protein electrophoresis. Immunofixation identified it as immunoglobulin A (IgA) k and the level was 3.9 (0.085-0.45) g/dL. The bone marrow contained 40% plasma cells, with atypical features. Radiographs showed diffuse lytic lesions. A 24-hour specimen of urine revealed a creatinine clearance of 110 (120±20) mL/min/1.5 m 2 . Calcium level was normal and β2 microglobuline was 4 mg/L (0.9-2.5). She was diagnosed with IgA k multiple myeloma (MM) stage IIIA. The patient was treated according to CDT protocol (oral cyclophosphamide 500 mg/week, oral thamidomide 100 mg/day, and oral dexamethasone 40 mg/day on days 1-4 and 12-15).Ten days following the first course, the patient developed fever (38°) and a cutaneous rash consisting of erythematoviolaceous plaques on the palms, forearms, trunk, and legs [Figure 1]. There were no pustules or peripheral vesiculation. The patient had an elevated WBC of 17,000/L with 70% neutrophils and an elevated erythrocyte sedimentation rate of 120 mm/h. A diagnosis of Sweet's syndrome (SS) was made. Skin biopsies confirmed the clinical diagnosis of SS, with neutrophilic infiltration of the dermis, leukocytoclasia, and subepidermal edema in the absence of signs of vasculitis. [1] Therapy was instituted with prednisone 0.5 mg/Kg/day. The skin lesions progressed to desquamation and cleared within 10 days.
Figure 1: Erythematoviolaceous plaques on the forearms

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The first description of MM associated with SS appeared in 1978. [2] It is a rare, recurrent, fairly dramatic skin disease characterized by painful plaque-forming inflammatory papules and associated with fever, arthralgia, and peripheral leukocytosis. [3] The majority of SS is idiopathic but it may also occur as a paraneoplastic syndrome predominantly in hematological malignancies [4] such as acute myelogenous leukemia, lymphoma, and myelodysplastic syndromes. Association with MM is uncommon; only a few cases have been reported in the literature. [5]

It would seem likely that SS developed in this patient as a paraneoplastic phenomenon.

 > References Top

1.Nischal KC, Khopkar U. An approach to the diagnosis of neutrophilic dermatoses: A histopathological perspective. Indian J Dermatol Venereol Leprol 2007;73:222-30.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.Saxe M, Gordon W. Acute febrile neutrophilic dermatosis (Sweet's syndrome). Four case reports. S Afr Med J 1978;53:253-6.  Back to cited text no. 2
3.Van Regenmortel N, Van de Voorde K, De Raeve H, Rombouts S, Van de Velde A, Lambert J, et al. Bortezomib induced Sweet's syndrome. Haematologica 2005;90 Suppl 12:e116-7.  Back to cited text no. 3
4.Bayer-Garner IB, Cottler-Fox M, Smoller BR. Sweet syndrome in multiple myeloma: A series of six cases. J Cutan Pathol 2003;30:261-4.  Back to cited text no. 4
5.Belhadji H, Chaabane S, Njim L, Youssef M, Zakhama A, Zili J. Sweet's syndrome associated with multiple myeloma. Acta Dermatovenerol Alp Panonica Adriat 2008;17:31-3.  Back to cited text no. 5


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