Sézary Syndrome After Successful Treatment of Hodgkin's Disease

Büchner, S.

doi:10.1001/archderm.1981.01650010056027

Cited by 7 publications

(2 citation statements)

References 29 publications

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“…In most cases, the HD followed the MF, but the occurrence of MF in patients with previous HD has also been occasionally reported. 3–6 In our patient, the HD preceded the diagnosis of MF but lesions of parapsoriasis (considered as latent MF 7 ) had existed for many years. The HD itself, and the chemotherapy, may have altered the infiltrating cells in the patches, allowing the diagnosis of MF.…”

Section: Discussionmentioning

confidence: 72%

Meningeal involvement by a transformed mycosis fungoides following Hodgkin's disease

Beylot‐Barry¹,

Dubus²,

Vergier³

et al. 1999

Br J Dermatol

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A 58-year-old man had long-standing lesions of presumed large plaque parapsoriasis. Following treatment for nodal Hodgkin's disease (HD), these became more infiltrated, with a diagnosis of mycosis fungoides (MF). A few months later, nodules appeared on the right leg, which was lymphoedematous after inguinal irradiation for HD. Histopathological examination showed CD3+, CD30-, CD15- large pleomorphic lymphocytes, leading to the diagnosis of transformed MF. The cutaneous lesions were successfully treated with topical nitrogen mustard and interferon alfa-2b then methotrexate, but his general health worsened with depression and malaise, without specific neurological symptoms or extracutaneous spreading of the lymphoma. Cerebral computed tomographic scan revealed a cerebellar subdural collection, arachnoid cyst and quadriventricular hydrocephaly, initially considered to be non-specific. After a few weeks, clinical symptoms of intracranial hypertension appeared, and a cerebrospinal fluid (CSF) examination revealed meningeal involvement by the lymphoma. These cells were CD3-negative and the diagnosis was confirmed by polymerase chain reaction (PCR) study, which revealed an identical clonal rearrangement of the T-cell receptor gamma gene between cutaneous biopsies and the CSF. Repeated intrathecal injections of methotrexate and cranial irradiation were performed and the patient was still alive after 13 months. This case illustrates the possible meningeal involvement of MF that may be preceded by atypical and mild neurological or psychiatric symptoms, which may be dissociated from the evolution of the cutaneous lesions. Moreover, PCR study may be useful for both diagnosis and monitoring.

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Section: Discussionmentioning

confidence: 72%

Meningeal involvement by a transformed mycosis fungoides following Hodgkin's disease

Beylot‐Barry¹,

Dubus²,

Vergier³

et al. 1999

Br J Dermatol

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“…Cutaneous T‐cell lymphoma usually antedates the development of other malignancies, 7 , 8 , 12 , 13 although occasionally the reverse has been reported. 16 , 21 This suggests that Sézary syndrome itself may in some way contribute to a predisposition for the development of second malignancies. Sézary syndrome is characterized by a malignant proliferation of T‐helper cells and, although the CD4 count may be markedly elevated, this results from the expansion of a neoplastic T‐cell clone and there is a relative lymphopenia of normal T cells leading to a degree of immunosuppression which may be severe.…”

Section: Discussionmentioning

confidence: 99%

Systemic Hodgkin’s lymphoma in a patient with Sézary syndrome

Scarisbrick

Child

Spittle

et al. 2000

British Journal of Dermatology

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We report a case of a 71-year-old male with Sézary syndrome diagnosed in 1996 who subsequently developed systemic Hodgkin's lymphoma. His only past treatment was bath psoralen plus ultraviolet A. He has since been treated with multiagent chemotherapy (ChlVPP/PABLOE) which induced a remission in his Hodgkin's disease. Eighteen months later he remains in remission from Hodgkin's disease but the Sézary syndrome remains active. He has also developed a squamous cell carcinoma on the upper lip. Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by a malignant proliferation of CD4-positive cells in the skin and peripheral circulation. The CD4 count may be markedly elevated but this results from expansion of a neoplastic T-cell clone and there is a relative lymphopenia of normal T cells leading to a degree of immunoparesis. Immunosuppression is known to be associated with an increased rate of malignancies and this may account for the occurrence of Hodgkin's disease and squamous cell carcinoma in this patient with Sézary syndrome.

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Two lymphomas: A potential diagnostic dilemma

Robinson

Berry

1989

I.J.M.S.

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We report a patient with mycosis fungoides of 20 years standing who developed mixed cellularity Hodgkin's disease. Full investigation, including biopsy, is essential when recurrent mycosis fungoides is suspected to outrule a second lymphoma as the results may affect management. Case History A Caucasian male presented aged 41 years with an eczematous rash affecting his trunk and upper and lower limbs. There was no lymphadenopathy/organomegaly or mucosal disease. Biopsy showed mucosis fungoides. This was controlled over the next two decades with simple emulsifying creams and topical corticosteroids, the disease remaining confined to the skin. Nineteen years after the diagnosis of mucosis fungoides, he developed an isolated left groin node, biopsy of which showed mixed cellularity Hodgkin's disease. Staging investigations were undertaken and the patient was found to have stage 1A disease (Ann Arbor). He was treated with combination chemotherapy (Nitrogen Mustard, Vincristine, Procarbazine and Prednisolone) and has had no recurrence of his Hodgkin's lymphoma, follow-up being seven years. His mycosis fungoides skin lesions improved temporarily with the cytotoxic therapy, but have subsequently progressed to the tumorous stage. Only temporary improvements in these lesions have resulted from total skin electron therapy, local electron irradiation and P.U.V.A.

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Sézary Syndrome After Successful Treatment of Hodgkin's Disease

Cited by 7 publications

References 29 publications

Meningeal involvement by a transformed mycosis fungoides following Hodgkin's disease

Meningeal involvement by a transformed mycosis fungoides following Hodgkin's disease

Systemic Hodgkin’s lymphoma in a patient with Sézary syndrome

Two lymphomas: A potential diagnostic dilemma

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