21/555. Meningeal involvement by a transformed mycosis fungoides following Hodgkin's disease. 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. ( info) |
| lymphomatoid papulosis (LyP) is a chronic self-healing cutaneous eruption which is clinically benign but histologically malignant. Lesions occur episodically over the trunk and limbs. We describe four patients with regional LyP. All were male, with a range in age at onset from 12 to 47 years. In all cases, lesions were confined to a segmental unilateral area. Two patients had type A and two type B LyP. We have long-term follow-up on one patient whose lesions were limited to the right buttock for more than 20 years before more widespread lesions developed. Another patient with lesions on the left flank had mycosis fungoides limited to the same region. Only one other case of LyP presenting in a regional distribution has previously been described. ( info) |
23/555. Novel solutions to the problems encountered in electron irradiation to the surface of the head. A novel treatment for two patients with mycosis fungoides involving all or most of the skin surface of the head is described using large overlapping low energy electron beams. Shielding of previously treated and uninvolved areas was achieved by encapsulating cerrobend within a thermoplastic shell in one patient. In the other patient, GE Saturnes unique asymmetric electron field definition facility was used. Satisfactory dose uniformity was demonstrated by the computation of dose distributions on the full summed electron pencil beam model on the Target Series 2 treatment planning system. Verification of the calculated dose homogeneity was confirmed with lithium fluoride (TLD-100) thermoluminescent dosimetry. The relatively simple treatment set-up was accomplished easily on a routine basis and was well tolerated by the patients, both of whom have been in complete remission since their treatment. ( info) |
24/555. Invisible mycosis fungoides: A diagnostic challenge. We describe a 76-year-old woman who presented persistent generalized pruritus as the only cutaneous manifestation of a cutaneous T-cell lymphoma (mycosis fungoides). No cutaneous lesions were observed throughout the patient's course. skin biopsies obtained from normal-looking pruritic skin revealed a discrete perivascular lymphocytic infiltrate in the upper dermis and focal intraepidermal clusters of atypical lymphoid cells (Pautrier's microabscesses). PCR analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement. Sequencing of the PCR monoclonal product identified the J(8)V(2)C(2) TCR gene rearrangement. This observation illustrates the existence of a peculiar and exceedingly rare form of mycosis fungoides characterized only by persistent pruritus unresponsive to several therapeutic approaches. The diagnostic difficulties of this rare variant are stressed. ( info) |
| A patient with mycosis fungoides (MF) bullosa had a rapidly growing, painful necrotic mass on the left ankle which extended by peripheral bulla formation, clinically resembling pyoderma gangrenosum. Histopathology confirmed MF bullosa with both intraepidermal and subepidermal bulla formation. ( info) |
| Allogeneic hematopoietic stem cell transplantation should be considered as a therapeutic option for patients with generalized erythoderma or tumor stage MF. Indeed, the only curative option for MF may be an allogeneic transplant. bone marrow transplantation (2000) 25, 111-113. ( info) |
27/555. Meningeal mycosis fungoides: cytologic and ultrastructural aspects. Mycosis cells were identified in the pre-morbid cerebrospinal fluid of a patient with neurological symptoms and mycosis fungoides (MF). light and electron microscopic examination at autopsy confirmed leptomeningeal involvement by mycosis fungoides. The cellular morphology of the non-cutaneous infiltrates supports the concept that mycosis fungoides retains a unique histopathology in its dissemination to the viscera. The importance of cerebrospinal fluid cytology in patients with mycosis fungoides is emphasized. ( info) |
28/555. T-cell lymphoproliferative disorder of vitreous associated with mycosis fungoides. 59-year-old man with a history of mycosis fungoides developed loss of visual acuity and visual field in the left eye. Epiretinal lesions were present in the right eye and multifocal choroidal lesions, optic disc edema, and vitritis were present in the left eye. A diagnostic vitrectomy was performed and cytologic examination of the vitreous confirmed the diagnosis of T-cell lymphoproliferative disorder. Systemic and intrathecal chemotherapy resulted in marked improvement in ocular signs and symptoms. At last follow-up, the patient was found to have improved visual acuity in the left eye; however, significant worsening of his systemic condition developed and he died shortly thereafter. ( info) |
| We report a 67-year-old woman with mycosis fungoides (MF) who was receiving subcutaneous injections of recombinant interferon alpha-2b (IFN-alpha). After 2 months of IFN-alpha treatment, bullous lesions appeared on her trunk and extremities. A skin biopsy specimen from the trunk revealed histopathologic features of bullous MF. Bullous lesions with specific infiltrates of MF are very rare; to the best of our knowledge, this is the first case showing specific bullous lesions of MF developed under IFN-alpha treatment. copyright (R) 2000 S.Karger AG, Basel ( info) |
| We describe a 68-year-old man with plaque stage mycosis fungoides (MF) for 8 years. He developed tumorous lesions of granulomatous MF (GrMF) and generalized granuloma annulare (GA) after a previously indolent clinical course. Since then, the clinical course was aggressive with involvement of the bone marrow and lymph nodes, and leukemic change occurred. Systemic chemotherapy was given, but the patient died 9 months later due to neutropenic fever and septic shock. GA in malignant lymphoma has been reported most frequently in association with Hodgkin's disease. To the best of our knowledge, GA associated with GrMF has never been reported in the English language literature. The prognostic significance of the association of granulomatous inflammation and malignancy is reviewed. copyright (R) 2000 S.Karger AG, Basel ( info) |