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  Table of Contents  
LETTER TO THE EDITOR
Year : 2020  |  Volume : 57  |  Issue : 4  |  Page : 489-490
 

Multiple myeloma presenting with diffuse ulcers and necrotic toe


1 Department of Hematology and Oncology, Guilan University of Medical Sciences, Rasht, Iran
2 Department of Internal Medicine, Kurdistan University of Medical Sciences, Sanandaj, Iran
3 Department of Rheumatology, Guilan University of Medical Sciences, Rasht, Iran
4 Department of Internal Medicine, Hematology and Oncology Division, Kurdistan University of Medical Sciences, Sanandaj, Iran
5 Department of Emergency Medicine, Kurdistan University of Medical Sciences, Sanandaj, Iran

Date of Submission13-Mar-2019
Date of Decision18-Jun-2019
Date of Acceptance27-Jun-2019
Date of Web Publication02-Oct-2020

Correspondence Address:
Hamed Sotoude
Department of Emergency Medicine, Kurdistan University of Medical Sciences, Sanandaj
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijc.IJC_227_19

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How to cite this article:
Nejatifar F, Mohebbi Z, Hajiabbasi A, Mozafari R, Sotoude H. Multiple myeloma presenting with diffuse ulcers and necrotic toe. Indian J Cancer 2020;57:489-90

How to cite this URL:
Nejatifar F, Mohebbi Z, Hajiabbasi A, Mozafari R, Sotoude H. Multiple myeloma presenting with diffuse ulcers and necrotic toe. Indian J Cancer [serial online] 2020 [cited 2020 Oct 23];57:489-90. Available from: https://www.indianjcancer.com/text.asp?2020/57/4/489/297018




A 64-year-old woman presented to the emergency department because of a painful and black left great toe and necrotic ulcers in her right foot and her breast, for three weeks. She also had bone pain, but no fever or significant weight loss was noted. Multiple ulcers over her hand, her foot, and her left breast and a necrotic left great toe were found on physical examination [Figure 1]. No lymphadenopathy or organomegaly was detected.
Figure 1: Cutaneous features include: (a) right foot necrotic ulcer, (b) left foot great toe necrosis, and (c) necrotic ulcer over her left breast

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Laboratory investigations revealed a high ESR (69 mm/1st hour) along with anemia (hemoglobulin = 8.8 g/dL, MCV = 86.4 fL), leukocytosis (WBC = 25000/mm3), and hypoalbuminemia (serum albumin = 2.5 mg/dL). Serum creatinine and serum calcium levels were within reference range.

The patient underwent bone marrow biopsy (BMB). A high plasma cell percentage (20%) on BMB besides lytic bone lesions on skull X-ray [Figure 2] and a high beta 2 microglobulin (serum level = 5.2 mg/L) were in favor of the diagnosis of multiple myeloma.
Figure 2: Skull X-ray showing lytic bone lesion compatible with MM

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Serum protein electrophoresis (SPEP) showed monoclonal gammopathy, with M protein of 2.8 g/dL [Figure 3]. Immunofixation electrophoresis (IFE) showed IgG lambda. Free light chain ratio was 0.19 (Lambda light chain was 52 mg/dL). Urine Bence Jones protein and cryoglobulin test were positive. P-ANCA, C-ANCA, ANA, and antiphospholipid tests were negative.
Figure 3: Serum protein electrophoresis panel, note the peaked M component

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Putting together the clinical history, BMB findings, and laboratory results, we made the diagnosis of Type 1 cryoglubulinemia in a background of multiple myeloma.

Treatment was started with bortozomib, thalidomide, low-dose dexamethason, and zolindronic acid. After four cycles of treatment, bone marrow examination showed less than 1% plasma cells, and cryoglobulins disappeared from the blood. Her vasculitic ulcers healed except that of left great toe which needed amputation. Because of patient's refusal to undergo autologous stem cell transplantation, thalidomide was continued as a maintenance therapy. After 10-month follow up, multiple myeloma is in remission.

Cryoglobulinemia (CG) is a rare disorder characterized by the presence of abnormal immunoglobulins (Ig) in the blood that precipitate in the tissues at low temperatures causing inflammation and tissue damage.[1] The prevalence of clinically significant cryoglobulinemia has been estimated to be approximately 1 in 100,000.[2]

Cryoglobulins consist of immunoglobulin and complement components which precipitate upon refrigeration of serum and plasma. It has been classified into three major types.[1] The presence of isolated monoclonal Ig (typically IgG or IgM, and less commonly IgA or free immunoglobulin light chains) is the criterion for classification as type I CG. The proportion of patients with a type I CG varies substantially among case series but accounts for about 5% to 25% of cases. Associated hematologic diagnoses with this type are typically Waldenström's macroglobulinemia or multiple myeloma. Type 2 and 3 are polyclonal and essential mixed cryoglobulinemia and are often observed in association with autoimmune disorders and infectious diseases.[2]

Cryoglobulinemia in the context of MM has variable features from diffuse livedo reticularis,[3] cutaneous ulcers, finger gangrene, and gall bladder necrosis.[4]

A nationwide retrospective study in France identified 64 patients with symptomatic type I CG over a 15-year period. All patients had either a hematologic malignancy (most often Waldenström's macroglobulinemia or multiple myeloma) or a monoclonal gammopathy of undetermined significance (MGUS) (56% and 44%, respectively).[5] Cutaneous involvement (necrosis and ulcers) in type 1 cryoglobulinemia can be severe in almost half of the patients.[5]

The management and prognosis of type 1 CG are primarily based on the underlying etiology; therefore, a thorough effort must be made to identify it. The management and prognosis of type I CG in patients with MM are poorly defined. Treatment includes glucocorticoids, plasma exchange, alkylating agents, rituximab, and chemotherapy.[5],[6] Our patient had a very good response to our selected regimen of bortezomib, thalidomide, and low-dose dexamethason. Early diagnosis and treatment are necessary for prevention of complications and amputation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M. Biologic and clinical significant of cryoglobulins: A report of 86 cases. Am J Med 1974;57:775-88.  Back to cited text no. 1
    
2.
Trejo O, Ramos CM, García CM, Yague J, Jimenez S, de la Red G, et al. Cryoglobulinemia: Study of etiologic factors and clinical and immunologic features in 443 patients from a single center. Medicine (Baltimore) 2001;80:252-62.  Back to cited text no. 2
    
3.
Requena L, Kutzner H, Angulo J, Renedo G. Generalized livedo reticularis associated with monoclonal cryoglobulinemia and multiple myeloma. J Cutan Pathol 2007;34:198-202.  Back to cited text no. 3
    
4.
Rajvanshi P, Atac BS, Seno R, Gupta S. Gallbladder vasculitis associated with type-1 cryoglobulinemia. Dig Dis Sci 2001;46:296-300.  Back to cited text no. 4
    
5.
Terrier B, Karras A, Kahn JE, Le Guenno G, Marie I, Benarous L, et al. The spectrum of type I cryoglobulinemia vasculitis: New insights based on 64 cases. Medicine (Baltimore) 2013;92:61-8.  Back to cited text no. 5
    
6.
Payet J, Livartowski J, Kavian N, Chandesris O, Dupin N, Wallet N, et al. Type I cryoglobulinemia in multiple myeloma, a rare entity: Analysis of clinical and biological characteristics of seven cases and review of the literature. Leuk Lymphoma 2013;54:767-77.  Back to cited text no. 6
    


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