Sonja Michaelis

Intra-lesional low-dose interferon α2a therapy for primary cutaneous marginal zone B-cell lymphoma

Primary cutaneous marginal zone lymphomas (pcMZL) belong to the primary cutaneous B-cell lymphoma (pCBCL) group. They are characterized by their restriction to the skin and a high likelihood of recurrence after various treatment modalities. First-line therapy consists of surgery and radiotherapy. These therapies may not always be indicated in young patients or in patients with pCBCL located in the face, where surgery or radiotherapy may leave disfiguring scars or radioderma. Eight patients with pcMZL were treated with intra-lesional injections of 3 million units of recombinant IFNα2a three times per week. The patients either did not want to undergo or did not qualify for surgical excision or radiotherapy due to inappropriate localization or age. All patients experienced complete tumor regression after a mean of 8.5 weeks (range 3 – 20). Two patients relapsed 4 and 12 months after treatment, respectively, but tumor regression was repeated after additional cycles of intra-lesional IFNα2a. All other patients remained free of disease. Thus, intra-lesional IFNα2a may represent a valuable alternative to surgery and radiotherapy as first-line treatment of pcMZL.

Hepatitis C and G viruses in B‐cell lymphomas of the skin

Background:  The etiology of B‐cell lymphoproliferative disorders (LPDs) of the skin has still to be elucidated. So far, Borrelia sp. has been identified as the causative agent of some cases of B‐cell LPDs of the skin. Apart from bacterial pathogens, recent studies suggested that also flaviviruses, in particular hepatitis C (HCV) and G (HGV) viruses, may be involved in the pathogenesis of B‐cell non‐Hodgkins lymphomas (NHLs). Most studies were performed in patients with known HCV infection, but the overall frequency of HCV‐ and HGV‐RNA in tumoral tissue of primary cutaneous B‐cell lymphomas (CBCLs) is unknown.

Primary cutaneous lymphoproliferative disorders with dual lineage rearrangement.

We present a series of 15 cases of cutaneous lymphoma and pseudolymphoma with dual lineage rearrangement identified among approximately 1200 cases of cutaneous lymphoproliferative disorders assessed in our 4 institutions during the last 8 years in which the results of both T-cell receptor and immunoglobulin heavy chain rearrangement investigations were available. On the basis of the clinicopathologic information, the cases were retrospectively subdivided into 2 categories: (1) cases with definite features of cutaneous lymphoma or pseudolymphoma (n = 11) and (2) cases with unclassifiable disease (n = 4). The detection of dual genotype in the first group did not influence the final diagnosis; 7 cases represented cutaneous B-cell lymphomas, 3 pseudolymphomas, and 1 case lymphomatoid papulosis. The presence of monoclonal T-cell receptor-gene rearrangements in these cases may be explained either by monoclonal or oligoclonal expansion of exuberant T cells (or B cells in case of lymphomatoid papulosis) or by lineage infidelity. Three patients with unclassifiable disease had several clinical and histopathologic features in common. They were elderly, presented with solitary lesions, were in good general health and histopathologically demonstrated a dense multinodular infiltrate containing approximately an equal number of T and B cells and a high number of histiocytes forming granulomas, with prominent granulomatous features in 2 cases. B cells were either scattered with the infiltrate or formed collections vaguely resembling follicles; Reed-Sternberg-like cells were seen in 2 cases. B cells showed expression neither of immunoglobulin light chain. The T-cell component was represented mainly by small, well-differentiated lymphocytes or slightly pleomorphic cells, with some medium-sized convoluted cells. Epstein-Barr virus was not detected by polymerase chain reaction. The exact classification of these cases is unknown; they differ histopathologically from previously published cases of bigenotypic cutaneous lymphomas. They may merely represent a growth or reactive pattern, but, on the other hand, may be low-grade lymphomas. If so, they may be histopathologically related to cutaneous Hodgkin disease, T-cell/histiocyte-rich large B-cell lymphoma, or composite lymphomas. Further reports are needed to identify these lesions to clarify their nature and biologic potential.

Subcutaneous infection with Mycobacterium abscessus in a renal transplant recipient.

Transplant recipients show a high incidence of infections, often with atypical manifestations mainly because of drug-related immunosuppression. Mycobacterial infections can be life-threatening in immunosuppressed patients due to the risk of dissemination. There have been only few reports of cutaneous infections with Mycobacterium abscessus among immunosuppressed patients. We present a rare case with a sporotrichoid skin infection with M. abscessus in a renal recipient. The value of immunohistochemical detection of mycobacteria using an anti-BCG antibody in the diagnostic work-up skin biopsies is demonstrated. Long-term antibiotic treatment resulted in complete remission, although recurrences with atypical presentation were observed.

Cutaneous malignant lymphomas: Update 2006

Cutaneous lymphomas represent a unique group of lymphomas and are the second most frequent extranodal lymphomas. As with other neoplasias, the pathogenesis is based mainly on a stepwise accumulation of mutations of suppressor genes and oncogenes caused by genetic, environmental or infectious factors. The diagnostic work‐up includes clinical, histological, imaging and hematological investigations and in many cases immunohistochemical and molecular biological analyses.

Study of HHV‐8 DNA sequences in archival biopsies from lesional skin of Kaposi's sarcoma, various mesenchymal tumors and related reactive conditions

Background: HHV‐8 has been identified as the causative agent of Kaposis sarcoma (KS) and some lymphoproliferative disorders. In addition, there are anecdotal reports on the presence of HHV‐8 in other tumors, especially cutaneous epithelial and mesenchymal neoplasms. The aim of the study was to ascertain the value of identification of HHV‐8 viral DNA sequences in routinely processed, formalin‐fixed, paraffin‐embedded tissues for the diagnosis of Kaposis sarcoma and other mesenchymal tumors.

Unusual manifestation of specific cutaneous involvement by B-cell chronic lymphocytic leukemia: Spontaneous regression with scar formation

We report a patient with specific cutaneous involvement by B-cell chronic lymphocytic leukemia, who demonstrated unusual clinical features during the course of the disease, namely several spontaneous regressions of skin lesions with the formation of scars. In addition, histologically proven keratoacanthoma was found. During the follow-up period of approximately 1.5 years, the patient experienced several recurrences of skin lesions and their partial spontaneous regression. The scars persisted and remained unchanged. We hypothesize that vascular injury combined with edema could have accounted for dermal ischemia and the subsequent development of the scarring lesions.

Cutaneous Lymphomas Unusual Cases 2

Mature T-cell and NK-cell Neoplasms.- Familial mycosis fungoides involving a father and daughter.- A case of mycosis fungoides with polyarthritis showing clonal identity in skin and synovial tissue.- Vesicular mycosis fungoides.- Unusual case of mycosis fungoides clinically mimicking a dermatophytosis subsequently evolving in aggressive CD30- cutaneous lymphoma.- Sclero-atrophic mycosis fungoides with a cytotoxic atypical phenotype.- CD56+ early mycosis fungoides.- Sezary syndrome with t(2 5) translocation, as developed after prolonged cyclosporine therapy for actinic reticuloid.- CD8+ Lymphomatoid papulosis with simultaneous Type A, Type B, and Type C lesions.- Lymphomatoid papulosis with a NK-cell phenotype.- Anaplastic large cell lymphoma associated with acquired ichthyosis.- Anaplastic large cell T-cell lymphoma.- Primary cutaneous histiocyte and neutrophil rich CD30+/CD56+ anaplastic large cell lymphoma of the scalp with prominent angio- and neuroinvasion.- Late relapse of primary cutaneous CD30+ anaplastic large cell lymphoma confirmed by T-cell receptor (TCR) PCR analysis.- Peripheral CD4+ T-cell lymphoma with cytotoxic phenotype.- CD8+ disseminated small and medium pleomorphic T-cell lymphoma with blood involvement and secondary hemophagocytic syndrome.- CD8+ cutaneous T-cell lymphoma with an indolent course.- Primary cutaneous pleomorphic small/medium-sized T-cell lymphoma revealed by a plantar callus.- Atypical poorly differentiated cutaneous T-cell lymphoma with an angiocentric growth pattern.- Angiocentric non-Hodgkins lymphoma of the leg.- ?/? cutaneous T-cell lymphoma.- Epidermotropic and subcutaneous ?/? T-cell lymphoma.- Cutaneous T-cell lymphoma presenting as reticular erythematous mucinosis.- Nasal NK-cell lymphoma preceded by a puffy eyelid and swollen cheek due to intramuscular infiltration of Epstein-Barr virus-infected cells.- Epstein-Barr virus-associated lymphoma mimicking hydroa vacciniforme: Final diagnosis of a case reported in 1986.- Epstein-Barr virus-positive blastoid NK-cell lymphoma.- Mature B-cell Neoplasms.- Primary cutaneous marginal zone B-cell lymphoma with secondary anetoderma in a patient with Sjogrens syndrome.- Follicular lymphoma with follicular dendritic cell overgrowth.- Diffuse large B-cell lymphoma with cutaneous and ocular involvement.- EBV-positive cutaneous B-cell lymphoproliferative disease after imatinib mesylate (Glivec).- B-cell chronic lymphocytic leukemia revealed by an erythroderma.- B-cell chronic lymphocytic leukemia (B-CLL) at the site of Borrelia burgdorferi infection.- Scarring leukemia cutis.- Lymphoma-associated insect bite-like reaction arising in a patient with mantle cell lymphoma.- Immature Hematopoietic Malignancies.- Blastic NK-cell lymphoma.- Subcutaneous splenosis of the abdominal wall.- Post-transplant lymphoproliferative disorder.

Extracutaneous transformation into a high-grade lymphoma: a potential pitfall in the management of patients with Sézary syndrome.

Background  Transformation into a high‐grade lymphoma in cutaneous T‐cell lymphoma (CTCL) occurs in approximately 25% of cases and is associated with an aggressive clinical course.

T‐zone lymphoma with cutaneous involvement: a case report and review of the literature

Summary T‐zone lymphoma (TZL) is a rare subtype of nodal peripheral T‐cell lymphoma characterized by a clonal expansion of T‐zone lymphocytes accompanied by a proliferation of other T‐zone constituents. Non‐specific cutaneous alterations are seen in about one‐third of all cases, but specific cutaneous involvement is extremely rare. We present a case of TZL with secondary skin infiltration, review the literature on cutaneous manifestations of TZL and discuss the differential diagnosis of TZL.