A case of Sezary's syndrome associated with granulomatous lesions, myelodysplastic syndrome and transformation into CD30-positive large-cell pleomorphic lymphoma

Carrozza, P.M.; Kempf, Werner; Kazakov, Dmitry V.; Dummer, Reinhard; Burg, Günter

doi:10.1046/j.1365-2133.2002.04839.x

Cited by 26 publications

(22 citation statements)

References 43 publications

(72 reference statements)

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“…2 In addition, the incidence of large cell transformation in MF and less often in SS is a well known phenomenon, having been reported in 8% to 55% of cases. [11][12][13][14] However, to our knowledge, the transition of SS into MF has not been reported yet.…”

Section: Resultsmentioning

confidence: 99%

Transition of Sézary syndrome into mycosis fungoides after complete clinical and molecular remission under extracorporeal photophoresis

Assaf

Hummel²,

Zemlin³

et al. 2004

J Clin Pathol

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Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common clinical variants of cutaneous T cell lymphoma. Although thought to be closely related to mature T helper cells, the relation between the neoplastic cells in MF and SS is still not fully clarified. This report describes a patient with complete remission of SS under treatment with extracorporeal photophoresis (ECP), who subsequently developed typical plaques of MF and large cell lymphoma (LCL). Serial polymerase chain reaction analyses confirmed identical T cell receptor β and γ gene rearrangements in SS, MF, and LCL, and complete disappearance of the circulating malignant T cell clone from the peripheral blood after ECP. These findings indicate that the neoplastic cells in SS, MF, and LCL are derived from a common precursor T cell, despite the change in clinical phenotype.

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Section: Resultsmentioning

confidence: 99%

Transition of Sézary syndrome into mycosis fungoides after complete clinical and molecular remission under extracorporeal photophoresis

Assaf

Hummel²,

Zemlin³

et al. 2004

J Clin Pathol

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“…7,8,10,23,24,27,28,32,33,34,36 Granulomatous infiltration has recently been reported in a subset of cutaneous lymphoproliferative disorders with dual lineage rearrangement. 18 Some of these patients also evidenced large binucleated cells raising the differential diagnosis of Hodgkin disease.…”

Section: Disscusionmentioning

confidence: 99%

Hyperplasia of Hair Follicles and Other Adnexal Structures in Cutaneous Lymphoproliferative Disorders

Kazakov¹,

Белоусова

Kacerovská³

et al. 2008

American Journal of Surgical Pathology

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We studied 53 cutaneous lymphoproliferative disorders, all of which manifested hair follicle hyperplasia. There were 42 cases conforming to the description of pseudolymphomatous folliculitis (PLF) and 11 cases of authentic lymphomas including mycosis fungoides, CD30+ anaplastic large cell lymphoma, diffuse large B-cell lymphoma, B-cell small cell lymphoma/leukemia, and peripheral T-cell lymphoma, not otherwise specified. All patients with PLF clinically presented with a solitary nodule preferentially involving the face. Beside hair follicle hyperplasia, the typical features were a dense infiltrate of small well-differentiated lymphocytes, lymphoplasmacytoid cells, plasma cells, and epithelioid histiocytes forming tiny granulomas. Some unusual or worrisome features recognized included eccrine/apocrine duct hyperplasia, subcutis/muscle infiltration, lymphocyte "smudging," single file infiltration, and large atypical cells. Immunohistochemically, T-cell predominant cases dominated in the series. All 34 tested cases revealed a polyclonal pattern of kappa and lambda immunoglobulin (Ig) light chain expression. In 4 cases, scattered CD30+ cells were identified. Monoclonal rearrangements of T-cell receptor (TCR) and IgH genes were detected in 19 and 3 cases respectively, including 1 case with dual T-cell receptor/IgH rearrangement. Three of 30 tested cases proved positive for herpes simplex virus-1, whereas herpes simplex virus-2 always tested negative. Of 31 cases tested for Borrelia burgdorferi, 30 specimens were negative. In 9 cases, fluorescent in situ hybridization for t(11;18) and t(14;18) revealed none of the above translocations. The most common treatment modality was surgical removal. Forty patients with a mean follow-up of 3.7 years included 39 patients with no evidence of disease and 1 individual with local recurrence. The comparison of "clonal cases of PLF" and those with polyclonal population or in which clonality remained undetermined revealed no differences between the 2 groups in the clinical presentation, pathologic, and immunohistochemical features. We conclude that hyperplasia of hair follicles and other adnexa can be seen not only in the condition currently known as PLF, but also in genuine cutaneous lymphomas and may be just a happenstance secondary to a basic pathologic process.

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“…Median time from diagnosis of MF to transformation was found to be 6.5 years, and the survival from transformation to death was 22 months. 15,16 Molecular studies based on T-cell receptor analysis have demonstrated that the large-cell infiltrate in transformed MF/Sézary syndrome represents evolution from the original malignant clone. 7 In Sézary syndrome, transformation clinically manifests with large tumors, which may ulcerate.…”

Section: Discussionmentioning

confidence: 99%

Transformation of Sézary Syndrome Into CD30+ Anaplastic Large T-Cell Lymphoma After Alemtuzumab Therapy With Evidence of Clonal Unity

Nevet

Zuckerman

Sahar

et al. 2015

The American Journal of Dermatopathology

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Alemtuzumab is a humanized mouse antibody targeting the CD52 cell surface, which has been effective in patients with advanced stage mycosis fungoides (MF) including erythrodermic MF and Sézary syndrome. There are a few descriptions of large cell transformation after its administration. A young patient with an acute onset of Sézary syndrome treated initially unsuccessfully with fludarabine and cyclophosphamide and later on successfully with alemtuzumab has been described. Three weeks after the beginning of therapy, however, she developed transformed T-cell lymphoma indistinguishable from CD30 anaplastic large-cell lymphoma. After bone marrow transplantation, the transformed CD30 cutaneous T-cell lymphoma recurred as a transformed CD30 plaque MF. All 3 types of lesions showed the same T-cell receptor clonal gene rearrangement, which supports the notion that Sézary syndrome, CD30 anaplastic large-cell lymphoma, and MF are interrelated.

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A case of Sezary's syndrome associated with granulomatous lesions, myelodysplastic syndrome and transformation into CD30-positive large-cell pleomorphic lymphoma

Cited by 26 publications

References 43 publications

Transition of Sézary syndrome into mycosis fungoides after complete clinical and molecular remission under extracorporeal photophoresis

Transition of Sézary syndrome into mycosis fungoides after complete clinical and molecular remission under extracorporeal photophoresis

Hyperplasia of Hair Follicles and Other Adnexal Structures in Cutaneous Lymphoproliferative Disorders

Transformation of Sézary Syndrome Into CD30+ Anaplastic Large T-Cell Lymphoma After Alemtuzumab Therapy With Evidence of Clonal Unity

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