Monoclonal gammopathy in Sezary syndrome. Report of three cases and review of the literature

Venencie, Pierre Y.

doi:10.1001/archderm.120.5.605

Cited by 11 publications

(6 citation statements)

References 18 publications

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“…The IgE level remained high throughout the course. The occurrence of the other monoclonal gammopathies has also been reported in 55 (26,27). We suggest that the high levels of IgE and IgG in our patient were not induced by malignant changes of B lymphocytes or plasma cells, but by secondary malignant biclonal immunoglobulinemia due to malignant proliferation of clone specific helper T lymphocytes, as suggested in the previous literature.…”

Section: Report Of a Casesupporting

confidence: 83%

Leser‐Trélat Sign Associated with Sézary Syndrome

Ikari

Ohkura

Morita

et al. 1995

The Journal of Dermatology

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A 74-year-old Japanese male had developed generalized erythroderma and rapid growth of multiple verrucous lesions over the entire surface of his face, trunk, and extremities three months before he was seen. Histologically seborrheic keratoses were revealed. Laboratory examinations showed peripheral leukocytosis with atypical lymphocytes and high levels of IgE and IgG. On the basis of these clinical and histopathologic findings, we diagnosed the patient as having Leser-Trélat sign associated with Sézary syndrome. The erythroderma subsided after administration of oral predonisone, and no new formations of seborrheic keratosis were observed. However, because of subsequent aggravation of the generalized erythroderma, we administered chemotherapy. Six months after the initial examination, lung cancer was found, and the patient subsequently died of respiratory and renal failure.

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Section: Report Of a Casesupporting

confidence: 83%

Leser‐Trélat Sign Associated with Sézary Syndrome

Ikari

Ohkura

Morita

et al. 1995

The Journal of Dermatology

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“…An association with viral infections such as HTLV-1 and Epstein-Barr virus has been identified in some T-cell leukemias and lymphomas, 23 " 25 but the higher incidence in Southeast Asia of most T-cell Iymphoproliferative disorders remains an intriguing enigma, as does their occasional association with a paraprotein. Although not a common finding, paraproteinemia has been described previously in association with T-cell lymphomas such as angioimmunoblastic lymphadenopathy (AILD), 26 mycosis fungoides, 27 and adult T-cell leukemia/lymphoma. 28 ' 29 An association with plasma cell dyscrasia and lymphoplasmacytoid proliferation, possibly due to altered immunoregulation, has also been noted.…”

Section: Discussionmentioning

confidence: 99%

Monoclonal Gammopathy of Unknown Significance and Malignant Paraproteinemia in Hong Kong

1996

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The incidence of multiple myeloma is lower in Southeast Asia than in the West. However, there are few reports on the overall incidence of paraproteinemia and its disease-associations in the Chinese. Therefore, the authors have correlated the laboratory features with the eventual clinical diagnosis in patients with paraproteinemia in a Hong Kong general/teaching hospital. Over 18 months, 1,600 patients were investigated for the presence of paraproteinemia. Paraproteinemia was detected in 157 (10%) patients. In 11 patients, investigations could not be completed. The remaining 146 patients were subjected to detailed clinical, radiologic, and laboratory investigations. Eighty-seven (59.6%) had monoclonal gammopathy of unknown significance (MGUS), 44 (30.1%) myeloma and 15 (10.3%) other lymphoproliferative disorder (LPD). There was no significant difference in the paraprotein concentration, frequency of hypogammaglobulinemia or Bence Jones proteinuria (BJP) or concentration of nonparaprotein immunoglobulin (Ig) between the myeloma and LPD groups. The overall kappa:lambda light chains ratios were 2.9,1.6 and 3.3 in the MGUS, MM and LPD groups, Paraproteinemia occurs both more frequently with increasing age and in association with a variety of diseases, and is thus a relatively common finding in hospital patients. The differential diagnosis in these patients will be between either myeloma, other lymphoproliferative disorder (LPD), or monoclonal gammopathy of uncertain significance (MGUS). The incidence of multiple myeloma is said to be lower in countries in Southeast Asia, including Hong Kong, compared to those in the West. 1 However, with the exception of a recent study from Taiwan, 2 there are no reports on the overall incidence of paraproteinemia and the frequency of associated diseases in this population. It was our clinical impression that the finding of a paraprotein in the Hong Kong hos- respectively. Polyclonal Ig elevation was rare with myeloma (4.5%) but was detected in 33% of patients with LPD (P <.02) and 40% of those with MGUS (P <.0001). Biclonal (and one triclonal) gammopathy was detected in 11.5% of patients with MGUS, 11% with LPD and 4.5% with myeloma. In the MGUS group, infection was the commonest associated clinical disorder (29.3%). Moreover, 70% of patients with biclonal gammopathy and MGUS had an infection. Five of 15 patients with LPD had a T-cell malignancy, including 3 lymphomas and 2 large granular cell leukemias. Only one patient had primary systemic amyloidosis.It is concluded that the high frequency of biclonality and its association with infection, of paraproteinemia in association with T-cell malignancy and of kappa light chains in the MGUS and LPD groups are at variance with reports from the West and probably reflect local differences.

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“…Elevated IgE levels have also been observed in other disorders of chronic antigen stimulation as well as in MF 3,4 in which antigen persistence may play an etiologic role 5 . Several cases of monoclonal gammopathy and less commonly of multiple myeloma occurring in patients with the Sézary syndrome suggests that, in some cases, neoplastic T lymphocytes may promote B lymphocyte activity and proliferation 13 …”

Section: Discussionmentioning

confidence: 99%

Tumor Stage Mycosis Fungoides in a Patient Treated with Long‐Term Corticosteroids for Asthma and Atopic‐Like Dermatitis

Abel

Nickoloff

Shelby

et al. 1986

The Journal of Dermatologic Surgery and Oncology

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An association between atopy and the Sézary syndrome has been recognized in some patients. Our case represents an association between adult-onset asthma and atopic-like dermatitis, markedly elevated IgE levels, and the development of tumor stage mycosis fungoides (MF) in a patient receiving long-term corticosteroid therapy. The chronic eczematous dermatitis may have represented evolving MF in which the specific diagnosis was delayed by treatment with topical and systemic corticosteroids until advanced tumor stage disease. This case, in conjunction with prior reports, suggests that an atopic diathesis can be associated with both leukemic and nonleukemic forms of cutaneous T-cell lymphoma.

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Monoclonal gammopathy in Sezary syndrome. Report of three cases and review of the literature

Cited by 11 publications

References 18 publications

Leser‐Trélat Sign Associated with Sézary Syndrome

Leser‐Trélat Sign Associated with Sézary Syndrome

Monoclonal Gammopathy of Unknown Significance and Malignant Paraproteinemia in Hong Kong

Tumor Stage Mycosis Fungoides in a Patient Treated with Long‐Term Corticosteroids for Asthma and Atopic‐Like Dermatitis

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