Publications by authors named "Werner Kempf"

Background: In recent classifications several cutaneous lymphomas were reclassified as lymphoproliferative disorder (LPD). These include primary cutaneous CD4-positive small/medium T-cell LPD (PCSM-TCLPD), primary cutaneous acral CD8+ T-cell LPD (acral CD8+ TLPD) and primary cutaneous marginal zone LPD (PCMZLPD; still classified as primary cutaneous marginal zone lymphoma (PCMZL) in the 5th edition of the WHO classification). The results of a survey among 30 cutaneous lymphoma centres on the effects of this new terminology on clinical management revealed considerable heterogeneity and emphasized the need to develop uniform recommendations for management and treatment of these disorders.

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Background: Primary cutaneous anaplastic large cell lymphoma (pcALCL) and lymphomatoid papulosis (LyP) are indolent CD30-positive lymphoproliferative disorders that rarely express TCR-γδ. However, primary cutaneous gamma-delta T-cell lymphoma (pcGDTCL), characterized by TCR-γδ expression on neoplastic cells, is a rare, aggressive cutaneous T-cell lymphoma with a poor prognosis. Accurate differentiation is essential due to distinct clinical behavior and treatment.

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Based on histologic and genetic patterns, the current World Health Organization (WHO) classification distinguishes six subtypes of lymphomatoid papulosis (Lyp). The aim of our article was to analyze the frequency of histologic and immunohistochemical features of different Lyp subtypes reported in the literature. We used PubMed advanced search builder to systematically review and evaluate English and German literature of Lyp from 1968 to April 2022.

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Article Synopsis
  • * Radiation oncologists must make tailored decisions for patient treatment, but these choices would benefit from more clinical evidence and research.
  • * The article calls for a prospective trial to better understand how different radiation doses affect patient outcomes in primary cutaneous lymphoma treatment.
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Background: Cutaneous leishmaniasis (CL) incidence in Switzerland is rising due to factors like migration and globalization. The aim of this work was to investigate CL frequency in Switzerland and identify clinical and histopathological difficulties in diagnosing CL in a non-endemic country.

Patients And Methods: This retrospective study evaluated the clinical and histopathological characteristics of all CL cases from two dermatopathology laboratories between 2000 and 2022.

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  • Globally, while people are living longer, many experience a decline in health due to age-related diseases, highlighting the need for better classification systems to address these issues.
  • A consensus meeting with 150 experts established criteria for identifying ageing-related pathologies, requiring a 70% agreement for approval among participants.
  • The agreed criteria focus on conditions that progress with age, contribute to functional decline, and are backed by human studies, setting a foundation for future classification and staging efforts.
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Background: The subcellular distribution of CD30 on mast cells and the presence of eosinophils in cutaneous mastocytosis require further investigation, especially as the cell surface expression of CD30 is critical for the therapeutic response of systemic mastocytosis to brentuximab vedotin.

Objective: Investigation of 147 biopsy specimens from 143 patients with cutaneous mastocytosis for mast cell density and distribution, frequency of CD30 expression, CD30 staining patterns, and presence and distribution of eosinophils. Correlation with clinical patterns.

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  • * Primary cutaneous B-cell lymphomas have undergone a significant review, leading to the establishment of new classifications, including a distinct category for cutaneous follicle center lymphomas.
  • * The document emphasizes the role of genomic and molecular data in improving classifications, although further research is needed to fully characterize these factors in primary cutaneous lymphomas.
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  • Primary cutaneous lymphomas are rare skin cancers that do not have lymphomas outside the skin when they are diagnosed, and there is currently no data on this condition in Nepal.
  • This study looked at 15 cases of cutaneous lymphomas diagnosed at a hospital in Kathmandu, with patients having a median age of 45 years and more males than females.
  • The findings showed that 87% of the lymphomas were T-cell types, with mycosis fungoides being the most common subtype, while only 13% were B-cell lymphomas.
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  • Lymphomatoid papulosis (LyP) is a rare skin disorder affecting T-cells, and there's limited information about its impact on children.
  • A study analyzed data from 87 pediatric patients diagnosed with LyP over a 24-year period, noting that most cases were initially misdiagnosed and the average age at onset was 7 years.
  • The prognosis appears positive, with a 100% overall survival rate, although 10% of patients had associated blood cancers, indicating a significantly higher risk of malignancy compared to the general youth population.
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(1) Background: Various cutaneous adverse drug reactions (ADRs) are observed with the implementation of mRNA COVID-19 vaccines. To gain insight into the clinicopathologic features, we analyzed the correlation of histological and clinical data in 48 patients with these ADRs. (2) Methods: Single-center retrospective study in patients with ADRs after mRNA COVID-19 vaccination (mRNA-1273 and BNT162b2 vaccines).

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  • Lymphomatoid papulosis (LyP) can present in various ways and may be misdiagnosed as aggressive skin lymphomas, especially those involving γδ T-cells.
  • A study analyzed 26 cases of LyP with γδ T-cell expression, noting that most patients were treated with topical steroids or had no treatment.
  • The findings highlighted significant differences in how LyP presents, underlining the importance of recognizing specific clinical features and markers to avoid misdiagnosis.
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Leukemia cutis corresponds to skin infiltration by malignant hematopoietic cells. It is most commonly reported in acute myeloid leukemia, particularly in subtypes with a monocytic component. Its clinical manifestations are extremely variable, and histopathologic diagnosis of cutaneous leukemic infiltrates may be challenging.

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Purpose: Dedifferentiated melanoma (DedM) poses significant diagnostic challenges. We aimed to investigate the clinical, histopathological and molecular features of DedM. Methylation signature (MS) and copy number profiling (CNP) were carried out in a subgroup of cases.

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Background: Mycosis fungoides is the most common subtype of cutaneous T-cell lymphoma, in which the expression of cluster of differentiation 30 (CD30)+ subtype can now be treated with the CD30 antibody conjugate brentuximab vedotin. Diagnostic methods are based on immunohistochemical (IHC) staining followed by manual assessment by pathologists, which is always a subjective calculation. QuPath, an open-source software for digital pathology image analysis, satisfies the requirements of objective approaches.

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  • The overview discusses the upcoming 5th edition of the WHO Classification of Haematolymphoid Tumours, specifically focusing on lymphoid neoplasms, while myeloid and histiocytic neoplasms will be covered separately.
  • Key changes from the previous 4th edition include a hierarchical reorganization of entities, modifications in their nomenclature, and revisions to diagnostic criteria and subtypes.
  • The new edition will also see the deletion of certain entities, the introduction of new ones, and the inclusion of tumour-like lesions and germline predisposition syndromes related to lymphoid neoplasms.
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Aims: To evaluate the frequency, subcellular localization, and variability of CD30 expression in mycosis fungoides.

Methods: This retrospective multicenter study investigated 135 biopsy specimens of 95 patients with mycosis fungoides for CD30 expression and CD30 staining pattern in relation to histomorphologic criteria, eosinophils, and tumour stage. We used two different CD30 cutoffs: (i) any CD30 positivity (important for in-label treatment with an anti-CD30 antibody-based drug), and (ii) ≥10% (most commonly used cutoff in therapy studies).

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  • The study presents the largest series of granulomatous pigmented purpuric dermatosis (GPPD), a rare skin condition that can mimic other serious diseases like cutaneous T-cell lymphoma.
  • It involves analyzing the histopathological features of nine GPPD cases compared to ten other types of pigmented purpuric dermatoses.
  • The findings indicate GPPD primarily affects adult males' lower extremities and shows distinct histological traits, including a specific immune cell profile.
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