Cutaneous Lymphoma

Primary cutaneous marginal zone lymphoma

2009-10-20T04:26:00.000-07:00

Primary cutaneous marginal zone lymphoma

Crit Rev Oncol Hematol. 2009 Oct 9

Dalle S, Thomas L, Balme B, Dumontet C, Thieblemont C.

Service de dermatologie, Université Claude Bernard Lyon I, Hospices Civils de Lyon, Lyon, France.

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) is included in the group of extranodal marginal zone B-cell lymphoma involving mucosal sites. Many evidences suggest that chronic antigen stimulation is a key-player in its pathogenesis. While Helicobacter pylori seems not to be implicated in PCMZL, Borrelia Burgdorferi's role is still matter of debate since the results are discordant between European and North American/Asian countries. However Borrelia subspecies are different between the studied areas and this difference could be a confounding factor. Then ubiquitous candidate antigen is still missing. Beyond these discrepancies the treatment of diffuse PCMZL has been recently improved. If local therapies (surgery, radiation) are the gold standard for localized disease, rituximab can also be considered as an alternative for disseminated or plurifocal PCMZL.

Elsevier

Primary cutaneous marginal zone B-cell lymphoma: clinical and histological aspects

Pathologica. 2009 Feb

Khaled A, Sassi S, Fazaa B, Ben Hassouna J, Ben Romdhane K, Kamoun MR.

Department of Dermatology, Charles Nicolle Hospital, Tunis, Tunisia. aida.khaled@rns.tn

According to the WHO-EORTC classification of cutaneous lymphomas, primary cutaneous marginal zone B-cell lymphoma are now well characterized. We report here a case of primary cutaneous marginal zone B-cell lymphoma in a 51 year-old man in which the diagnosis was made using both histology and immunopathology. The patient had no remarkable medical history, no history of either acute inflammation or insect bite, and presented with a 5 cm solitary asymptomatic erythematous firm, multinodular and infiltrated plaque on the back for 12 months. Histological examination and immunohistochemical study of a cutaneous biopsy provided a differential diagnosis between B cell lymphoma and lymphocytoma cutis. Full body work up revealed no signs of extracutaneous dissemination. The patient underwent surgical excision of the nodule. Histological examination showed a histological and immunophenotyping profile typical of primary cutaneous marginal zone B-cell lymphoma. The lesion was completely excised with clear margins and no recurrence occurred after a 12 month-follow-up period. Primary cutaneous marginal zone B-cell lymphoma are low-grade lymphomas that have an indolent course and a high tendency to recur. They should be differentiated from lymphocytoma cutis and from the other types of cutaneous B cell lymphomas that have a different course and prognosis.

PMID: 19771768 [PubMed - in process]

Vitreous metastases of primary cutaneous B-cell lymphoma.

2009-10-20T04:22:00.000-07:00

Vitreous metastases of primary cutaneous B-cell lymphoma.

Ocul Immunol Inflamm. 2009 Sep-Oct

Chong DY, Johnson MW, Shen D, Chan CC, Callanan DG.

Texas Retina Associates, Arlington, TX, USA.

Purpose: To describe two cases of vitreous metastases of primary cutaneous B-cell lymphoma (PCBCL). Methods: Observational case series. Results: A 73-year-old man and an 81-year-old woman, both with a history of PCBCL, diffuse large cell type, presented with decreased visual acuity due to vitritis. Both patients underwent vitreous biopsy that demonstrated B-cell lymphoma, large cell type, and confirmed metastases of cutaneous B-cell lymphoma to the vitreous. Conclusion: PCBCL, diffuse large cell type, is a rare form of non-Hodgkin lymphoma that can metastasize to the vitreous without visible chorioretinal involvement.

PMID: 19831568 [PubMed - in process]

IRF4 Gene Rearrangements Define a Subgroup of CD30-Positive Cutaneous T-Cell Lymphoma

2009-10-12T13:17:00.000-07:00

IRF4 Gene Rearrangements Define a Subgroup of CD30-Positive Cutaneous T-Cell Lymphoma:

A Study of 54 Cases

J Invest Dermatol. 2009 Oct

Pham-Ledard A, Prochazkova-Carlotti M, Laharanne E, Vergier B, Jouary T, Beylot-Barry M, Merlio JP.

Department of Histology and Molecular Pathology of Tumors, University Bordeaux 2, Bordeaux, France.

Correspondence: Professor Jean-Philippe Merlio, Department of Histology and Molecular Pathology, University Bordeaux 2, 146 rue Leo Saignat, Bordeaux 33076, France. E-mail:jp.merlio@u-bordeaux2.fr

The identification of IFN regulatory factor 4 gene (IRF4) translocation in 8 out of 14 cases of cutaneous anaplastic large cell lymphomas (C-ALCLs) (Leukemia, 2009; 23(3):574-80) prompted us to study IRF4 locus status in different cutaneous T-cell lymphoma (CTCL) subtypes. Fluorescence in situ hybridization (FISH) was used with break-apart dual-color probes. Sixty samples from 54 patients were analyzed with 23 C-ALCL, 11 transformed mycosis fungoides (T-MF), 7 lymphomatoid papulosis (LyP), and 13 Sézary syndrome (SS) cases. IRF4 immunostaining was performed in 32 cases. We observed a split FISH signal pattern indicating a translocation at the IRF4 locus in 6 out of 23 C-ALCL (26%) and 2 out of 11 T-MF (18.2%) cases. Extra copies of the IRF4 locus were found in 4 out of 13 SS, 1 out of 11 T-MF, and 1 out of 23 C-ALCL cases, corresponding to either aneuploidy, chromosome 6 trisomy, or 6p partial gain. The IRF4 expression was not correlated with the presence of IRF4 alteration in C-ALCL or T-MF. Interestingly, IRF4 rearrangements define a subgroup of CD30-positive C-ALCL and T-MF cases. Conversely, T-MF cases with IRF4 rearrangements may correspond to the development of C-ALCL in MF patients and not to large cell transformation. Investigation of the biological pathways triggered by IRF4 rearrangements and/or expression in CTCL will provide a biological basis for IRF4-directed therapy.Journal of Investigative Dermatology advance online publication, 8 October 2009; doi:10.1038/jid.2009.314.

Nature

Abbreviations:

ALCL, anaplastic large cell lymphoma; ALK, anaplastic lymphoma kinase; C-ALCL, cutaneous anaplastic large cell lymphoma; CTCL, cutaneous T-cell lymphoma; FISH, fluorescencein situ hybridization; IRF4, IFN regulatory factor 4; LyP, lymphomatoid papulosis; MF, mycosis fungoides; MM, multiple myeloma; MUM1, multiple myeloma antigen 1; PTCL, peripheral T-cell lymphoma; T-MF, transformed mycosis fungoides

Primary cutaneous marginal zone B-cell lymphoma: clinical and histological aspects.

2009-09-29T04:42:00.000-07:00

Primary cutaneous marginal zone B-cell lymphoma: clinical and histological aspects.

Pathologica. 2009 Feb

Khaled A, Sassi S, Fazaa B, Ben Hassouna J, Ben Romdhane K, Kamoun MR.

Department of Dermatology, Charles Nicolle Hospital, Tunis, Tunisia. aida.khaled@rns.tn

PubMed

Anetoderma in cutaneous marginal-zone B-cell lymphoma.

2009-09-29T04:37:00.000-07:00

Anetoderma in cutaneous marginal-zone B-cell lymphoma.

Clin Exp Dermatol. 2009 Sep

Zattra E, Pigozzi B, Bordignon M, Marino F, Chiarion-Sileni V, Alaibac M.

Department of Dermatology, University of Padua, Padua, Italy.

Correspondence to Dr Mauro Alaibac, Dermatology Unit, University of Padua, Via C. Battisti 206, 35128, Padova, Italy
E-mail: mauro.alaibac@unipd.it

Summary: Anetoderma is a rare condition, consisting of well-circumscribed areas of slack skin, in which dermal elastic fibres are destroyed or deficient. We present the case of a 45-year-old man with a 25-year history of deep nodules and plaques gradually progressing to areas of anetoderma. Histological examination found an infiltrate composed of neoplastic cells with lymphoplasmocytoid morphology. The cells were positive for CD20, CD38 and CD138, and there was a monoclonal kappa light chain gene rearrangement of plasma cells. A diagnosis of cutaneous marginal-zone B-cell lymphoma was made. The pathogenesis of anetoderma remains unknown, but it is possible that cytokines or other soluble factors produced by the infiltrating lymphocytes have a role in this process.

Wiley InterScience

Hodgkin lymphoma with cutaneous involvement

2009-09-21T22:46:00.000-07:00

Hodgkin lymphoma with cutaneous involvement
May 2009

Hodgkin lymphoma with cutaneous involvement Cyrus C Hsia MD FRCPC1, Kang Howson-Jan MD FRCPC1, Kamilia S Rizkalla MD FRCPC2Dermatology Online Journal 15 (5): 5 1. Department of Medicine, Division of Hematology2. Department of PathologyLondon Health Sciences Centre, London, Ontario, Canada. chsia@uwo.ca

Abstract
We report a case of a 54-year-old previously healthy man with Hodgkin lymphoma who presented initially with a solitary cutaneous ulcer. Unlike non-Hodgkin lymphoma subtypes, skin involvement of Hodgkin lymphoma is extremely rare. Furthermore, the prognosis of Hodgkin lymphoma with skin infiltration is felt to be extremely poor. Contrary to other reports, this case demonstrates that a good response with standard therapy is possible.

Introduction
In contrast to non-Hodgkin lymphoma subtypes, skin involvement of Hodgkin lymphoma (HL) is extremely rare [1, 2]. Furthermore, the prognosis when cutaneous involvement is present is felt to be extremely poor in HL [2]. We report a case of a patient with HL who presented with a solitary cutaneous ulcer. Although his diagnosis was delayed, he obtained a good response with adequate standard therapy.

Case
A 54-year-old previously healthy man noticed an ulceration in his left gluteal area. Initially, the ulcer was the size of a quarter, but subsequently enlarged to 10 cm over the course of several months. The lesion had areas of necrotic and loose granulation tissue. Marked left inguinal lymphadenopathy with associated scrotal, penile, and left leg edema developed. These were also associated with mild intermittent fevers and significant weight loss.

Two separate biopsies of the ulceration were performed and both were reported as non-specific dermatitis. However, upon review by a hematopathologist (KSR, one of the authors) the diagnosis of classical Hodgkin lymphoma, nodular sclerosis subtype, was made. The biopsies revealed multinucleated giant cells with large nuclei and several nucleoli, the characteristics of Reed-Sternberg cells, underneath a large area of ulceration and necrosis. These cells were present in the typical background of small mature lymphocytes, eosinophils, and plasma cells.

A biopsy of a left inguinal lymph node confirmed the diagnosis of nodular sclerosis HL. Similar to the skin lesion, Reed-Sternberg cells were again present and stained positively for both CD15 and CD30. The lymph node had a typical nodular pattern surrounded by broad collagen bands. Staging CT scan of the abdomen showed massive conglomerated retroperitoneal lymphadenopathy. Bone marrow was otherwise unremarkable. He was diagnosed with stage 3B disease and was started on chemotherapy with ABVD (Adriamycin, Bleomycin, Vinblastine, and Dacarbazine) with an overall good response. After six weeks of treatment his ulcer had completely healed and his disease was deemed to be in complete remission after six cycles of therapy.

Discussion
Since the first description by Sir Thomas Hodgkin in 1832, the diagnosis, classification, and management of HL have evolved dramatically [3]. Hodgkin lymphoma represents a small but significant proportion of malignancies worldwide and has a bimodal age distribution [1, 3]. The etiology and pathogenesis of this disease remains unknown [1, 3]. A few risk factors such as familial predisposition, infections with Epstein-Barr virus or human immunodeficiency virus, and immune suppression have been implicated [1, 2, 3]. This disease has been divided into two major groups according to the World Health Organization: the rare nodular lymphocyte-predominant and classical Hodgkin lymphoma [1]. Classical HL contains four subtypes: nodular sclerosis, mixed cellularity, lymphocyte rich, and lymphocyte depleted [1]. Each subtype has its own clinical features, pathological characteristics and prognosis [1, 3].

The most common clinical manifestation of classical HL is painless lymphadenopathy; over 80 percent of involved lymph nodes are found above the diaphragm [1, 2]. Patients may also describe painful lymphadenopathy, pruritus, and constitutional symptoms [1, 2]. Rarely, extranodal regions include the spleen, liver, lung, and bone marrow [1]. It is extremely rare to have extranodal involvement of other organs, the central nervous system, or the integument – as in our patient [2-8].

It is known that non-specific skin involvement such as pruritus, hyperpigmentation, urticaria, erythoderma, or acquired ichthyosis occurs fairly commonly, in 17-53 percent [2, 4]. However, these are thought to be paraneoplastic syndromes rather than direct tumor infiltration [2]. Direct tumor involvement is extremely rare and reported in a number of case reports [2, 4, 5, 6, 7, 8]. In 1906, Grosz described the first case of a patient with Hodgkin disease and multiple brownish, "lentil to walnut-sized," ulcerating nodules [2, 4]. Since then, the incidence has been reported to occur in 0.5-3.4 percent of Hodgkin lymphoma patients [2, 4]. Recently, Introcaso et al. have described another case and thoroughly reviewed the literature on cutaneous HL and showed that the number of cases had reportedly diminished over time [4]. It was felt this was likely due to improved treatments and the utilization of stem cell transplantation for relapsed disease [2, 4]. Frequently in cutaneous HL, the presentation is single or multiple dermal or subcutaneous nodules that become ulcerated and involve the skin area over the chest [4, 5, 6]. The mechanism or mechanisms for the skin involvement in HL are not known but postulated to be retrograde lymphatic spread from tumor-involved lymph nodes, direct extension into skin by tumor cells in underlying lymph nodes, or hematogenous spread of the tumor [2, 4].

The diagnosis of classic HL requires careful pathological identification of characteristic binucleated tumor cells (Reed-Sternberg cells), or mononuclear cells (Hodgkin cells) within an inflammatory milieu [1]. These malignant cells represent 0.1-10 percent of all cells in a biopsy, are derived from germinal center B cells in more than 98 percent, and are distributed in a background of reactive cells [1, 3]. Typically, these cells stain positively for CD15 and CD30 but not CD20 [1, 3]. Other than HL, cells resembing Reed-Sternberg cells may be present in other B and T cell lymphomas, carcinomas, melanomas, and sarcomas [1, 3]. In particular, HL must be distinguished from other conditions that present with cutaneous lesions such as mycosis fungoides, granulomatous slack skin disease (CTCL), lymphomatoid papulosis, and anaplastic large cell lymphoma. The latter two may also have CD30 positive cells and require the more specific CD15 positive expression to be differentiated from HL.

Overall, the prognosis in Hodgkin lymphoma is good with greater than an 80 percent five-year survival [1, 3]. However, cutaneous involvement is usually associated with diffuse lymphadenopathy, late stage disease, and poor prognosis [2, 4, 6, 8]. Our case was an exception; the outcome was favorable with a good dermatological response. Rare cases of cutaneous HL with indolent clinical courses have previously been described showing slow progression to generalized lymphadenopathy over years [6, 7]. Thus, it is important to recognize this malignancy, complete the appropriate work-up for staging, and initiate prompt treatment. There are no standardized treatments for Hodgkin lymphoma with skin lesions. Standard treatment is chemotherapy with or without involved field radiation depending on the stage and bulk of the disease [3]. Skin lesions due to Hodgkin lymphoma, as in our case, have been reported to respond to current standard treatment without further therapeutic modalities such as surgical excision, topical therapy, or local cutaneous radiation [2, 4].

Conclusions
Although cutaneous involvement of Hodgkin lymphoma is extremely rare, it merits consideration in the category of atypical causes of non-specific papules, plaques, and ulcers; therefore, enlisting a hematopathologist may be required. Contrary to most reported cases of HL involving skin, our case demonstrates that a good response with adequate therapy is possible.

Acknowledgements: We thank dermatologist Dr. Ronald W. Gottschalk for taking the initial picture. We thank Dr. Barbara Burrall for her constructive review and advice.

Dermatology Online

Denileukin diftitox for the treatment of cutaneous T-cell lymphoma

2009-09-20T12:55:00.000-07:00

Denileukin diftitox for the treatment of cutaneous T-cell lymphoma

Biologics. 2008 Dec

Kaminetzky D, Hymes KB.
Division of Hematology/Oncology, New York University School of Medicine, New York, USA.

Cutaneous T-cell lymphoma/mycosis fungoides (CTCL/MF) is a rare lymphoproliferative disorder which can present as an indolent or as an aggressive process involving skin, lymph nodes, and blood. In stages IA, IB and IIA, it is usually managed with topical medications and phototherapy. If there is progression despite application of these treatments, or if the patient presents with a higher stage of disease, systemic chemotherapy or retinoids, rexinoids, biologic response modifiers are often necessary. Consequently, patients are often treated with a sequence of modalities and drugs. Denileukin diftitox (DD, Ontak(R)) is a targeted immunotoxin which has biological activity against malignancies expressing the IL-2 receptor. In addition to its unique mechanism of action, DD has a toxicity profile which does not overlap with most commonly used chemotherapeutic agents. CTCL/MF has been found be particularly susceptible to treatment with this agent. This review will describe the development DD, its proposed mechanism of action, the clinical trials which identified its utility in the treatment of CTCL/MF, the common toxicities encountered with this agent, and the management of these toxicities. In addition the incorporation of DD in the sequential treatment of CTCL/MF and data suggesting potential combination therapies employing this novel agent will be discussed.

Full text article:

DovePress

Cutaneous T cell lymphoma-mycosis fungoides and Sezary syndrome: an update

2009-09-17T09:20:00.000-07:00

Cutaneous T cell lymphoma-mycosis fungoides and Sezary syndrome: an update

G Ital Dermatol Venereol. 2009 Aug

Parker SR, Bethaney JV.
Department of Dermatology, Emory University, Atlanta, GA, USA srsingh@emory.edu.

Mycosis fungoides (MF) is the most common form of cutaneous T cell lymphoma (CTCL) and, for unclear reasons, its incidence appears to be increasing. Due to significant differences in biological behavior, cutaneous T cell lymphomas have recently been reclassified. MF and Sezary syndrome (SS) are now separately classified as indolent and aggressive CTCLs, respectively. The cause of MF and SS remains elusive. Because establishing the diagnosis of MF, particularly in early stages of disease, is often difficult, a guideline with clinical and histological diagnostic criteria has recently been proposed. With the exception of very early stage disease, no cure is available. However, the number of available therapeutic options has increased considerably in recent years, and partial and complete remissions are achievable. Prognosis and selection of appropriate therapy largely depends on stage of disease. A detailed practice guideline with an algorithmic, stage-based approach to therapy has recently been put forth and will likely aid clinicians in managing patients with MF and SS. Despite the indolent nature in most individuals, the disease has a tremendous psychological impact, not only because of the visible nature of the skin lesions, but also due to the rarity of the disease and its chronicity. Knowledge of this disease, therapeutic options and expectations of therapy will greatly enhance care of afflicted patients.

PubMed

A case of recurrent pseudolymphomatous folliculitis: A mimic of cutaneous lymphoma

2009-06-08T07:14:00.000-07:00

A case of recurrent pseudolymphomatous folliculitis: A mimic of cutaneous lymphoma
J Am Acad Dermatol. 2009 Jun

Kwon EJ, Kristjansson AK, Meyerson HJ, Fedele GM, Tung RC, Sellheyer K, Tuthill RJ, Honda KS, Gilliam AC, McNiff JM.

Department of Dermatology, Case Western Reserve University, Cleveland, Ohio, USA.

Pseudolymphomatous folliculitis is a rare entity. We present a 62-year-old man with a recurrent solitary nodule on his nose requiring multiple excisions. Microscopic examination of the excisions showed a dense lymphocytic infiltrate containing numerous histiocytes and S100+, CD1a+ dendritic cells that surrounded and infiltrated hypertrophic hair follicles. Diffuse sheets of CD3+ T cells and nodular clusters of CD20+ B cells were also seen. There was normal reactive pattern of follicular centers. Light chain restriction was not detected. T-cell receptor and immunoglobulin heavy chain gene rearrangements by polymerase chain reaction revealed negative findings. A diagnosis of pseudolymphomatous folliculitis was made based on the hypertrophic hair follicles, periadnexal S100+ and CD1a+ dendritic cells, and negative clonal gene rearrangement study findings. This case of recurrent pseudolymphomatous folliculitis is instructive because of the resemblance to cutaneous lymphomas and cutaneous lymphoid hyperplasias, and the need for correct diagnosis to avoid overtreatment of this indolent condition.

Elsevier/ScienceDirect

Cutaneous large B-cell lymphoma of the leg: presenting initially as mononeuritis multiplex.

2009-06-08T07:11:00.000-07:00

Cutaneous large B-cell lymphoma of the leg: presenting initially as mononeuritis multiplex.
Singapore Med J. 2009 May

Ho SL, Tang BY, Chai J, Tan SH.
National Skin Centre, 1 Mandalay Road, Singapore. sheunling@yahoo.com

We report a rare case of primary B-cell lymphoma of the leg presenting with mononeuropathy multiplex. A 79-year-old Chinese woman who was being investigated for mononeuritis multiplex had an incidental finding of indurated erythematous plaques on the breast and left leg. A skin biopsy from the nodular area on the right breast showed a dense and diffuse infiltrate of atypical cells with large, round, hyperchromatic nuclei with prominent nucleoli. These atypical lymphocytes were CD20+, Bcl-2+ and Mum-1+. A diagnosis of diffuse large B-cell lymphoma, leg type involving the breast and leg with extracutaneous involvement, was made. This case highlights the importance of a full systemic and cutaneous examination in patients presenting with progressive, painful peripheral neuropathy

PubMed

Juvenile onset of primary low-grade cutaneous B-cell lymphoma

2009-05-19T08:36:00.000-07:00

Juvenile onset of primary low-grade cutaneous B-cell lymphoma
Br J Dermatol. 2009 Mar 26

Amitay-Laish I, Feinmesser M, Ben-Amitai D, David M, Manor Y, Kidron D, Barzilai A, Hodak E.
Department of Dermatology, Rabin Medical Center, Beilinson Hospital, Petah Tikva 49100, Israel.

Summary Background: Cutaneous lymphomas rarely occur in children and adolescents, and are mostly of the T-cell lineage. Low-grade primary cutaneous B-cell lymphoma (CBCL) is extremely rare in individuals under 18 years old. Only 11 patients under 20 years old have been reported in the literature.

Objectives: To evaluate the number of patients younger than 18 years with primary CBCL diagnosed at our centre and to investigate its clinicopathological features, treatment and course in this age group.

Methods: We reviewed the files of all 90 patients with primary CBCL who attended the Department of Dermatology of our tertiary care university-affiliated centre from 1992 to 2007.

Results: Four patients who met study criteria were identified: three girls and one boy. Mean age at diagnosis was 16.6 years (range 16-17). Three patients had cutaneous marginal zone lymphoma (CMZL), and one had a spindle-cell (sarcomatoid) lymphoma, most probably follicular centre cell type. All were treated with the standard regimen used in adults. The mean duration of follow up was 45 months. No extracutaneous progression was noted. At present two of the four patients are in complete clinical remission.

Conclusions: In Israel, primary CBCL apparently occurs more often in young patients than reported in the literature. CMZL is the most frequent type. Long follow up is mandatory to assess the biological behaviour of CBCL in the paediatric/adolescent age group.

Wiley InterScience

Gemcitabine treatment in cutaneous T-cell lymphoma: a multicentre study of 23 cases

2009-05-19T08:30:00.000-07:00

Gemcitabine treatment in cutaneous T-cell lymphoma: a multicentre study of 23 cases
Br J Dermatol. 2009 May

Jidar K, Ingen-Housz-Oro S, Beylot-Barry M, Paul C, Chaoui D, Sigal-Grinberg M, Morel P, Dubertret L, Bachelez H.

Service de Dermatologie, Centre Hospitalier Victor Dupuy, 95100 Argenteuil, France.

Background: Primary cutaneous T-cell lymphomas (CTCLs) are malignancies characterized by a clonal T-cell infiltrate involving the skin. CTCLs often show resistance to conventional antineoplastic chemotherapy. Gemcitabine is a pyrimidine analogue which has shown efficacy and a favourable safety profile in solid tumours and haematological malignancies.

Objectives: We report a multicentre retrospective study of 23 patients who received gemcitabine for advanced-stage CTCL and emphasize the high incidence of serious unusual adverse events. Methods We collected data from 23 patients with refractory CTCL (14 mycosis fungoides, six Sézary syndrome and three other CTCL). Gemcitabine was given weekly within a 21- or 28-day schedule. Response was evaluated after three and six cycles of chemotherapy. For each patient, all adverse events were recorded.

Results: Of the 16 patients who received at least three cycles of gemcitabine, 10 achieved a response (62.5%). Only five patients reached the sixth cycle of treatment and four still had a favourable response. Haematological toxicity was recorded in 15 cases with severe grade 3 or 4 neutropenia in seven patients (30%) and six serious infections (26%). Other serious adverse events were observed in six cases (26%): one haemolytic-uraemic syndrome, one severe capillary leak syndrome, one acute heart failure related to cardiac arrhythmia, two bullous and erosive dermatitis, and one recurrent influenza-like syndrome with altered general condition.

Conclusions: Our study confirms the early efficacy of gemcitabine in advanced-stage CTCL. However, our results contradict the safety profile of gemcitabine previously reported and underline the high incidence of severe complications including visceral and cutaneous involvement.

WileyInterScience

Primary cutaneous anaplastic large cell lymphoma of the face presenting as posttraumatic maxillary sinusitis.

2008-12-30T07:10:00.000-08:00

Primary cutaneous anaplastic large cell lymphoma of the face presenting as posttraumatic maxillary sinusitis.
J Craniofac Surg. 2008 Nov

Chen RF, Chen CT, Liao HT, Chen CH, Chen YR.
Division of Trauma Plastic Surgery, Department of Plastic and Reconstruction Surgery, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Chang Gung Institute of Technology, Taoyuan, Taiwan.

Primary cutaneous anaplastic large cell lymphoma (ALCL) is an uncommon disease. It has various presentations that may mislead the diagnosis and cause delay of treatment. We report a 12-year-old boy who experienced persistent swollen and painful right cheek after blunt facial injury. The patient was first treated for maxillary sinusitis according to clinical history, but the pathology turned out to be primary cutaneous ALCL. Symptoms improved after chemotherapy with complete remission. No recurrence was noted during the follow-up period. The presentations of primary cutaneous ALCL of the face may mimic posttraumatic maxillary sinusitis. Any patient with facial prolonged symptoms or nonhealing wound should be excluded from this entity.

Lippincott, Williams & Wilkins

Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma.

2008-12-30T07:02:00.000-08:00

Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma.
Arch Dermatol. 2008 Jun

Gerami P, Rosen S, Kuzel T, Boone SL, Guitart J.
Department of Dermatology, Northwestern University Feinberg School of Medicine, and The Robert H. Lurie Comprehensive Cancer Center, Northwestern University, Chicago, Illinois, USA.

OBJECTIVES: To study the clinical features, therapeutic responses, and outcomes in patients with folliculotropic mycosis fungoides (FMF) and to compare our single-center experience of 43 patients with the findings from the Dutch Cutaneous Lymphoma Group.

SETTING: A single-center experience from the Northwestern University Multidisciplinary Cutaneous Lymphoma Group.

PATIENTS: Forty-three patients with FMF were included in the study and compared with 43 age- and stage-matched patients with classic epidermotropic mycosis fungoides (MF) with similar follow-up time.

RESULTS: Folliculotropic mycosis fungoides showed distinct clinical features, with 37 patients having facial involvement (86%) and only 6 having lesions limited to the torso (14%). The morphologic spectrum of lesions is broad and includes erythematous papules and plaques with follicular prominence with or without alopecia; comedonal, acneiform, and cystic lesions; alopecic patches with or without scarring; and nodular and prurigolike lesions. Sixty-five percent of patients had alopecia, which in 71% of cases involved the face. Severe pruritus was seen in 68% of patients. In general, patients responded poorly to skin-directed therapy and in almost all cases required systemic agents to induce even a partial remission, including patients with early-stage disease. Overall survival was poor. Patients with early-stage disease (< or ="IIA)"> or =IIB) had an outcome similar to those patients in the control group with conventional epidermotropic MF of a similar stage.

CONCLUSIONS: The morphologic spectrum of clinical presentation for FMF is broad and distinct from those in conventional MF. This is at least partially attributed to the ability of FMF to simulate a variety of inflammatory conditions afflicting the follicular unit. The disease course is aggressive, and many patients, including those with early disease, show a poor outcome particularly between 10 and 15 years after the initial onset of disease. Response to skin-directed therapy is poor even in early-stage disease, and our best results were seen with psoralen plus UV-A (PUVA) therapy with oral bexarotene or PUVA with interferon alfa. These findings corroborate those of the Dutch Cutaneous Lymphoma Group and further validate the classification of FMF as a distinct entity.

PubMed

A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy.

2008-12-30T07:00:00.000-08:00

A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy.
J Am Acad Dermatol. 2009 Jan

Jacob R, Scala M, Fung MA.
Department of Radiation Oncology, University of California-Davis, Sacramento, California, USA.

Syringotropic cutaneous T-cell lymphoma (SCTCL) is a variant of mycosis fungoides that is characterized by the presence of lymphocytic infiltration of eccrine glandular structures and the absence of the classic features of MF, such as epidermotropism and Pautrier's microabscesses. We report a patient with SCTCL who presented with multiple hypopigmented patches and localized alopecia who was treated with local radiotherapy with excellent local control. In our experience, local electron radiation offers local control in early stage SCTCL, and it may be possible to reserve systemic therapy for more advanced stages of the disease.

PMID: 19103368 [PubMed - in process]

Primary cutaneous T-cell-rich B-cell lymphoma: a case report and literature review.

2008-12-30T06:55:00.000-08:00

Primary cutaneous T-cell-rich B-cell lymphoma: a case report and literature review.
Acta Dermatovenerol Alp Panonica Adriat. 2008 Dec

Venizelos ID, Tatsiou ZA, Mandala E.
Pathology Department, Hippokration Hospital, 5 Exadactylou Street, 54635 Thessaloniki, Greece. ivenizel@otenet.gr.

T-cell-rich B-cell lymphoma (TCRBCL) is a recently recognized B-cell lymphoma variant, characterized by a minor population of neoplastic B-cells existing in a background of predominant reactive T-lymphocytes. It is a rare entity, accounting for approximately 1 to 2% of all non-Hodgkin's lymphomas. It has both nodal and extranodal presentation. Primary cutaneous TCRBCL is an extremely rare lymphoma and only 16 cases have been documented thus far in the medical literature. We report the case of a 46- year-old man that presented with a slowly-growing, painless skin nodule on the left temporofrontal region of the scalp. A complete surgical excision was performed and histological examination revealed diffuse infiltration of the dermis by TCRBCL. A complete surgical excision of the skin lesion and systemic chemotherapy seems to have been effective because the patient is disease-free 2 years after the initial diagnosis was made. This study reports a very rare case of TCRBCL presented primarily in the skin. Because of its rarity, it is especially important to make the correct diagnosis using the appropriate immunohistochemical stains and apply the proper therapy.

PMID: 19104743 [PubMed - in process]

Lymphomatoid papulosis in childhood: six case reports and a literature review

2008-11-24T04:42:00.000-08:00

Lymphomatoid papulosis in childhood: six case reports and a literature review
Ann Dermatol Venereol. 2008 Oct

Bories N, Thomas L, Phan A, Balme B, Salameire D, Thurot-Guillou C, Dalle S.

Service de dermatologie, hôpital Hôtel-Dieu, 1 place de l'Hôpital, Lyon cedex 02, France.

BACKGROUND: Lymphomatoid papulosis (LyP) is a rare lymphoproliferative disorder. It is now included in the World Health Organisation (WHO) classification of cutaneous lymphomas. Although frequently described in adults, there have been only a few reported cases of LyP in children; diagnosis is often difficult in this population and no clear guidelines have been established regarding management or monitoring. In this article we report six new cases of LyP in children.

PATIENTS AND METHODS: This is a retrospective study of six children, aged between two and 11 years, seen at the Hôtel-Dieu Hospital in Lyon and at the Grenoble Hospital Centre between 2005 and 2008. Each child underwent skin biopsy for histological and immunohistochemical analysis.

RESULTS: All six children presented papulonodular lesions on the limbs and trunk, in some cases necrotic, present for different times and developing in episodes. Histology and immunolabelling announces in all six cases militated in favour of LyP type A, with large CD30+ and CD15- cells dispersed in an inflammatory dermal infiltrate made up for the most part of lymphocytes, polynuclear neutrophils, eosinophils and a small number of histiocytes.

DISCUSSION: Only around 60 cases of LyP have so far been reported in children, principally type A. Association with malignant lymphoma occurs, with high risk in relation to the general population of the same age. Clinical diagnosis is confirmed histopathology and immunolabelling. There is currently no consensus regarding therapeutic management. First-line treatment generally comprises therapeutic abstention or dermal corticosteroids. Methotrexate and phototherapy constitute possible alternatives but should be used only in very disseminate or debilitating forms of the disease.

CONCLUSION: The presentation and course of LyP of childhood differs very little from the adult form. Cases of associated lymphoma have been reported. Although regular clinical monitoring is recommended, there is no call for routine laboratory testing.

EMConsulte

Primary cutaneous B cell lymphoma presenting as recurrent eyelid swelling.

2008-11-24T04:37:00.000-08:00

Primary cutaneous B cell lymphoma presenting as recurrent eyelid swelling.
Clin Experiment Ophthalmol. 2008 Oct

Pandya VB, Conway RM, Taylor SF.
University of Sydney, Sydney, NSW, Australia.

Primary cutaneous lymphoma represents a distinct clinical entity within the spectrum of haematological malignancy. A case of primary cutaneous B cell lymphoma is reported, presenting in an 87-year-old female with a 2-year history of intermittent swelling and discolouration of the right upper and lower eyelids, in the absence of systemic symptoms. Histopathological examination of an incision biopsy revealed a lymphoid infiltrate in the dermis with immunophenotypic features of B cell lymphoma. Staging investigations confirmed the absence of systemic disease. Treatment with oral chemotherapy was undertaken with a good response. Ophthalmologists should include primary cutaneous lymphoma in the differential diagnosis of recurrent eyelid swelling.

Wiley InterScience

Coincidence of cutaneous follicle center lymphoma and diffuse large B-cell lymphoma.

2008-11-24T04:33:00.000-08:00

Coincidence of cutaneous follicle center lymphoma and diffuse large B-cell lymphoma.
Int J Dermatol. 2008 Nov

van Tuyll van Serooskerken AM, Mosterd K, Veraart JC, Vermeer MH, Frank J, van Marion AM.

Department of Dermatology, University Medical Center Maastricht, The Netherlands. atv@sder.azm.nl

Primary cutaneous follicle center lymphoma (PCFCL) is a neoplasm with differentiation of centrocytes and centroblasts presenting in the skin. At the time of initial manifestation, extracutaneous involvement is absent. PCFCL is considered as an indolent variant of primary cutaneous B-cell lymphomas since dissemination to extracutaneous sites is rare and the prognosis is favorable. Here we describe a 30-year-old man who was diagnosed with a cutaneous FCL and did not show extracutaneous affection at the time of occurrence. Six months later, however, he developed a diffuse large B-cell non-Hodgkin lymphoma localized in several lymph nodes of the neck that most likely reflects the occurrence of a second primary tumor in the same patient.

PubMed

Primary cutaneous aspergillosis in a patient with cutaneous T-cell lymphoma.

2008-11-12T05:04:00.000-08:00

Primary cutaneous aspergillosis in a patient with cutaneous T-cell lymphoma.
Mycoses. 2008 Oct 18

Yuanjie Z, Jingxia D, Hai W, Jianghan C, Julin G.
Department of Dermatology and Mycology Center, Changzheng Hospital, Second Military Medical University, Shanghai, China.

We present a case of primary cutaneous aspergillosis caused by Aspergillus terreus in a patient with cutaneous T-cell lymphoma. A 41-year-old woman bruised her left shin and presented with a 1 x 2 cm ecchymosis on the upper third of the left shin for over 6 months. Before mycological examination and sequential biopsies, the patient was erroneously treated with antibiotics and local debridement. After final diagnosis of cutaneous aspergillosis caused by A. terreus cutaneous T-cell lymphoma on the basis of mycological, histopathological and immunohistochemical findings, the patient responded well to oral itraconazole and chemotherapy for over 1 month.

PubMed

Cutaneous B-cell lymphoma

2008-11-04T06:16:00.000-08:00

Cutaneous B-cell lymphoma

What are cutaneous B-cell lymphomas?

Lymphomas are tumours of the lymph nodes and lymphatic system. Extranodal lymphomas are tumours that occur in organs or tissues outside of the lymphatic system. When lymphomas occur in the skin with no evidence of extracutaneous disease at the time of diagnosis, they are called ‘primary’ cutaneous lymphomas. There are many different types of primary cutaneous lymphomas but they can be broadly divided into two categories, cutaneous T-cell lymphomas and B-cell lymphomas. Primary cutaneous lymphomas of B cell type comprise approximately 20% of cutaneous lymphomas.

Cutaneous B-cell lymphomas occur when there is a malignant proliferation of lymphocytes of the B-cell type. Mutation occurring at different points in B cell development leads to differing forms of lymphoma.

Classification of cutaneous B-cell lymphomas

Recently the World Health Organisation (WHO) and European Organization for Research and Treatment of Cancer Classification (EORTC) reached a consensus classification for cutaneous lymphomas1. Primary cutaneous B-cell lymphomas include:

Primary cutaneous follicle centre lymphoma
Primary cutaneous marginal zone B- cell lymphoma
Primary cutaneous diffuse large B-cell lymphoma, leg type
Primary cutaneous diffuse large B-cell lymphoma, other

Primary cutaneous follicle centre (FC) lymphoma

These skin lymphomas typically present as solitary or grouped nodules, tumours, or infiltrative plaques. They occur most commonly in the scalp, forehead, neck, trunk and back. The tumours have an indolent (slow growing) course but progress with time. Neoplastic follicle centre cells, usually a mixture of centrocytes (small and large cleaved follicle centre cells) and centroblasts (large noncleaved follicle centre cells with prominent nucleoli) are seen in the dermis in at least part of the infiltrate, but usually spare the overlying epidermis. Cellular morphology may vary with the age and size of the lesion, with care required to differentiate this group from Primary cutaneous diffuse large B-cell lymphoma, leg type.

Primary cutaneous FC lymphoma needs to be differentiated from a secondary cutaneous lymphoma, which is where a nodal follicular lymphoma has spread to involve the skin (skin metastasis). It appears from molecular biology studies that Primary cutaneous FC lymphoma usually lack expression of the bcl-2 protein, and, the t(14;18) translocation, which if present may suggest systemic follicular lymphoma.

Primary cutaneous FC lymphomas may be treated with radiotherapy or combination chemotherapy. Radiotherapy is the treatment of choice for localised tumours whereas multiple tumours on the skin of different parts of the body (multifocal) may respond better to combination chemotherapy. Patients with primary cutaneous FC lymphomas have a good overall survival rate. Five-year survival is reported to be more than 95%.

Primary cutaneous marginal zone B-cell lymphoma

These are low-grade malignant B-cell lymphomas of the MALT (mucosa-associated lymphoid tissue) type, that occur in the skin as solitary or multiple red to brownish red papules, plaques, or nodules. They are usually located on the trunk or extremities, especially the arms. Histologically, these lymphomas are characterised by the presence of nodular or diffuse infiltrates with small lymphocytes, marginal zone B cells, lymphoplasmacytoid cells and plasma cells localised in the dermis and subcutaneous fat. This group also includes primary cutaneous immunocytomas.

Primary cutaneous immunocytoma and marginal zone B-cell lymphoma are classed as indolent lymphomas with excellent prognosis. Five-year survival rate is close to 100%. Dissemination to extracutaneous sites is very rare, however there are reports of it spreading to other organs and the bone marrow.

Radiotherapy or surgical excision is effective treatment for patients with a solitary or few lesions. Multifocal skin lesions are treated with chemotherapy agents such as chlorambucil, interferon alpha and anti-CD20 antibody (rituximab). As this is an indolent lymphoma, observation is a reasonable approach in some cases.

Primary cutaneous large B-cell lymphoma, leg type

These cutaneous B-cell lymphomas occur mainly in elderly females and present as erythematous red or bluish-red nodules or tumours usually on one or both lower legs. These tumours are more aggressive than large B-cell lymphomas of the head and neck, often disseminate to extracutaneous sites and have a more unfavourable prognosis.

Histologically these lymphomas show a diffuse monotonous population of centroblasts and immunoblasts. This picture may be seen in lesions not arising on the leg, and these can be classified into this group.

Only some localised small tumours may be treated using radiotherapy. Anthracycline-based chemotherapy is used for most skin tumours or when tumours have spread to other parts of the body. Some cases have shown response to the anti-CD20 antibody rituximab. Death usually occurs with disseminated disease.

Primary cutaneous diffuse large B-cell lymphoma, other

This group contains large B-cell lymphomas arising in the skin, which do not belong to the primary cutaneous follicle centre lymphoma group or large B-cell lymphoma, leg type. These lymphomas commonly present with skin lesions on the head, the trunk or the extremities and have an excellent prognosis.

Intravascular large B-cell lymphoma is a well-defined subtype of large B-cell lymphoma. Malignant B lymphoid cells proliferate within the lumen of small blood vessels, primarily in the skin and central nervous system. Lesions appear as erythematous, tender nodules, tumours, and telangiectases mainly on the trunk and lower legs. Cutaneous lesions may be confused with mycosis fungoides, sarcoidosis, blood vessel tumours, or secondary cutaneous involvement by lymphoma or leukaemia. As the disease progresses secondary organ involvement often occurs. Prognosis is poor, particularly if the disease has spread to other parts of the body.

Article

What is cutaneous T-cell lymphoma?

2008-10-26T07:57:00.000-07:00

What is cutaneous T-cell lymphoma?

Cutaneous T-cell lymphoma is a disease in which certain cells of the lymph system (called T-lymphocytes) become cancer (malignant) and affect the skin. Lymphocytes are infection-fighting white blood cells that are made in the bone marrow and by other organs of the lymph system. T-cells are special lymphocytes that help the body's immune system kill bacteria and other harmful things in the body.

The lymph system is part of the immune system and is made up of thin tubes that branch, like blood vessels, into all parts of the body, including the skin. Lymph vessels carry lymph, a colorless, watery fluid that contains lymphocytes. Along the network of vessels are groups of small, bean-shaped organs called lymph nodes. Clusters of lymph nodes are found in the underarm, pelvis, neck, and abdomen. The spleen (an organ in the upper abdomen that makes lymphocytes and filters old blood cells from the blood), the thymus (a small organ beneath the breastbone), and the tonsils (an organ in the throat) are also part of the lymph system.

There are several types of lymphoma. The most common types of lymphoma are called Hodgkin's disease and non-Hodgkin's lymphoma. These types of lymphoma usually start in the lymph nodes and the spleen. See the patient information summaries on adult or childhood non-Hodgkin's lymphoma or adult or childhood Hodgkin's disease for treatment of these cancers.

Cutaneous T-cell lymphoma usually develops slowly over many years. In the early stages, the skin may itch, and dry, dark patches may develop on the skin. As the disease gets worse, tumors may form on the skin, a condition called mycosis fungoides. As more and more of the skin becomes involved, the skin may become infected. The disease can spread to lymph nodes or to other organs in the body, such as the spleen, lungs, or liver. When large numbers of the tumor cells are found in the blood, the condition is called the Sezary syndrome.

If there are symptoms of cutaneous lymphoma, a doctor may remove a growth from the skin and look at it under a microscope. This is called a biopsy.

The chance of recovery (prognosis) and choice of treatment depend on the stage of the cancer (whether it is just in the skin or has spread to other places in the body) and the patient's general state of health.

There are several other types of cancer that start in the skin. The most common are basal cell cancer and squamous cell cancer, which are covered in the PDQ patient information summary on skin cancer. Another type of skin cancer called melanoma is covered in the patient information summary on melanoma. Kaposi's sarcoma, a rare type of cancer that occurs most commonly in patients with the Acquired Immunodeficiency Syndrome (AIDS), also affects the skin. See the PDQ patient information summary on Kaposi's sarcoma for treatment of this cancer. Cancers that start in other parts of the body may also spread (metastasize) to the skin.

Stages of cutaneous T-cell lymphoma

Once cutaneous T-cell lymphoma is found, more tests will be done to find out if cancer cells have spread to other parts of the body. This is called staging. A doctor needs to know the stage of the disease to plan treatment.

The following stages are used for cutaneous T-cell lymphoma:

Stage I

The cancer only affects parts of the skin, which has red, dry, scaly patches, but no tumors. The lymph nodes are not larger than normal.

Stage II

Either of the following may be true: The skin has red, dry, scaly patches, but no tumors. Lymph nodes are larger than normal, but do not contain cancer cells. There are tumors on the skin. The lymph nodes are either normal or are larger than normal, but do not contain cancer cells.

Stage III

Nearly all of the skin is red, dry, and scaly. The lymph nodes are either normal or are larger than normal, but do not contain cancer cells.

Stage IV

The skin is involved, in addition to either of the following:

Cancer cells are found in the lymph nodes.

Cancer has spread to other organs, such as the liver or lung.

Recurrent

Recurrent disease means that the cancer has come back after it has been treated. It may come back where it started or in another part of the body

From: National Cancer Institute - PDQ

For the complete article, including treatment information, please see the link below.

National Cancer Institute