We herein present an overview of the upcoming 5th edition of the World Health Organization Classification of Haematolymphoid Tumours focussing on lymphoid neoplasms. Myeloid and histiocytic neoplasms will be presented in a separate accompanying article. Besides listing the entities of the classification, we highlight and explain changes from the revised 4th edition. These include reorganization of entities by a hierarchical system as is adopted throughout the 5th edition of the WHO classification of tumours of all organ systems, modification of nomenclature for some entities, revision of diagnostic criteria or subtypes, deletion of certain entities, and introduction of new entities, as well as inclusion of tumour-like lesions, mesenchymal lesions specific to lymph node and spleen, and germline predisposition syndromes associated with the lymphoid neoplasms.
- MeSH
- hematologické nádory * MeSH
- lidé MeSH
- lymfom * patologie MeSH
- Světová zdravotnická organizace MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
Lichen aureus is a variant of pigmented purpuric dermatoses. The usual histopathology of lichen aureus is characterized by a subepidermal dense, band-like lymphocytic infiltrate, extravasated erythrocytes, and hemosiderin deposits. We report three patients with lichen aureus on the extremities with similar clinical, dermoscopic, and histopathological findings characterized by a dense band-like relatively deep dermal infiltrate accompanied by extravasation of erythrocytes and hemosiderin deposits occasioning a resemblance to a lymphoproliferative disorder.
- MeSH
- dermatoskopie metody MeSH
- diferenciální diagnóza MeSH
- dospělí MeSH
- erytrocyty patologie MeSH
- hemosiderin analýza MeSH
- imunohistochemie metody MeSH
- kožní nemoci patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- lymfocyty patologie MeSH
- lymfoproliferativní nemoci metabolismus patologie MeSH
- plazmatické buňky patologie MeSH
- poruchy pigmentace patologie MeSH
- pseudolymfom komplikace patologie MeSH
- purpura diagnóza patologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
Pseudolymphomatous infiltrates in Borrelia infection of the skin most commonly manifest with dense B-cell infiltrates and plasma cells. Cutaneous infiltrates of B-cell chronic lymphocytic leukemia (B-CLL) may accumulate at sites of infection, including Borrelia infection. We report an unusual constellation in a patient with synchronously diagnosed B-CLL and Borrelia infection of skin presenting with a dense dermal T-cell-rich infiltrate masking specific leukemic infiltrates of neoplastic B cells in the context of B-CLL harboring t(14;18)(q32;q21). Specific cutaneous involvement by B-CLL was confirmed by the detection of t(14;18)(q32;q21) (BCL2-IGH) using FISH in neoplastic B cells within the skin infiltrates. Borrelia burgdorferi (sensu lato) DNA detected by nested polymerase chain reaction in the skin biopsy and serological findings proved Borrelia infection. Complete resolution of the cutaneous infiltrates was observed after antibiotic treatment. This case demonstrates that Borrelia infection of the skin may present with dense T-cell-rich infiltrates mimicking cutaneous T-cell lymphoma and masking the synchronous presence of neoplastic B cells in the context of B-CLL.
- MeSH
- chronická lymfatická leukemie komplikace genetika patologie MeSH
- hybridizace in situ fluorescenční MeSH
- imunofenotypizace MeSH
- imunohistochemie MeSH
- lidé MeSH
- lidské chromozomy, pár 14 * MeSH
- lidské chromozomy, pár 18 * MeSH
- lymeská nemoc komplikace imunologie patologie MeSH
- nádory kůže komplikace genetika patologie MeSH
- polymerázová řetězová reakce MeSH
- senioři MeSH
- separace buněk MeSH
- T-lymfocyty imunologie MeSH
- translokace genetická MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
BACKGROUND: With the exception of erythema migrans, Borrelia infection of the skin manifests much more commonly with B cell-rich infiltrates. T cell-rich lesions have rarely been described. OBJECTIVE: We report a series of 6 patients with cutaneous borreliosis presenting with T cell-predominant skin infiltrates. METHODS: We studied the clinicopathologic and molecular features of 6 patients with T cell-rich skin infiltrates. RESULTS: Half of the patients had erythematous patchy, partly annular lesions, and the other patients had features of acrodermatitis chronica atrophicans. Histopathology revealed a dense, band-like or diffuse dermal infiltrate. Apart from small, well differentiated lymphocytes, there were medium-sized lymphocytes with slight nuclear atypia and focal epidermotropism. An interstitial histiocytic component was found in 4 cases, including histiocytic pseudorosettes. Fibrosis was present in all cases but varied in severity and distribution. In 5 patients, borrelia DNA was detected in lesional tissue using polymerase chain reaction studies. No monoclonal rearrangement of T-cell receptor gamma genes was found. LIMITATIONS: This retrospective study was limited by the small number of patients. CONCLUSION: In addition to unusual clinical presentation, cutaneous borreliosis can histopathologically manifest with a T cell-rich infiltrate mimicking cutaneous T-cell lymphoma. Awareness of this clinicopathologic constellation is important to prevent underrecognition of this rare and unusual presentation representing a Borrelia-associated T-cell pseudolymphoma.
- MeSH
- akrodermatitida etiologie MeSH
- bakteriální nemoci kůže diagnóza imunologie patologie MeSH
- Borrelia burgdorferi izolace a purifikace MeSH
- chronická lymfatická leukemie komplikace MeSH
- diferenciální diagnóza MeSH
- DNA bakterií izolace a purifikace MeSH
- dospělí MeSH
- erytém etiologie MeSH
- fibróza MeSH
- histiocyty patologie MeSH
- kousnutí klíštětem komplikace mikrobiologie patologie MeSH
- kožní T-buněčný lymfom diagnóza MeSH
- kůže imunologie mikrobiologie patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- lymeská nemoc komplikace diagnóza imunologie patologie přenos MeSH
- pseudolymfom diagnóza imunologie patologie MeSH
- receptory antigenů T-buněk gama-delta genetika MeSH
- retrospektivní studie MeSH
- senioři MeSH
- T-lymfocyty patologie MeSH
- zvířata MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- MeSH
- antigeny CD279 analýza MeSH
- antigeny CD4 analýza MeSH
- antigeny CD8 analýza MeSH
- lidé MeSH
- mycosis fungoides imunologie MeSH
- nádorové biomarkery analýza MeSH
- nádory kůže imunologie MeSH
- T-lymfocyty pomocné-indukující imunologie MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- dopisy MeSH
- komentáře MeSH
: Primary cutaneous marginal zone lymphoma (PCMZL) is one of the most common cutaneous B-cell lymphomas. It affects mostly patients in their fourth decade and manifests with multifocal nodules mostly on the arms and upper trunk in more than half of the patients. PCMZL is, however, rare in children and adolescents, with only 20 cases reported in patients aged 20 and younger. The authors present 3 cases of PCMZL in teenagers. The patients were 2 girls aged 18 and 13 and a 17-year-old boy. Two patients presented with multiple lesions involving various anatomic sites, whereas in 1 patient, 2 small closely opposed papules on the abdomen were seen. Histopathologically, the characteristic appearance of PCMZL was found in 3 of 4 specimens, with nodular infiltrates composed of small lymphocytes in the interfollicular compartment, reactive germinal centers, and plasma cells in small clusters mainly at the periphery of the infiltrates, whereas 1 specimen showed a dense lymphocytic infiltrate with small granulomas. Clonality was demonstrated by monotypic immunoglobulin light chain expression and/or monoclonal rearrangement of the immunoglobulin heavy chain genes. No Borrelia burgdorferi was identified on serology or by polymerase chain reaction in any of the cases. Treatment included excision or administration of antibiotics with complete remission in all the 3 patients indicating that PCMZL in children and young adolescents follows the same indolent course with a tendency for recurrences, but excellent prognosis as in adults. The pertinent literature on PCZL in childhood and adolescence is reviewed.
- MeSH
- antibiotika antitumorózní terapeutické užití MeSH
- biopsie MeSH
- dermatochirurgické výkony MeSH
- imunohistochemie MeSH
- indukce remise MeSH
- lidé MeSH
- lymfom z B-buněk marginální zóny * chemie genetika patologie terapie MeSH
- mladiství MeSH
- mnohočetné primární nádory * chemie genetika patologie terapie MeSH
- nádorové biomarkery analýza genetika MeSH
- nádory kůže * chemie genetika patologie terapie MeSH
- výsledek terapie MeSH
- Check Tag
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
Cutaneous γ/δ+ T-cell lymphoma (CGD-TCL) is a rare but aggressive lymphoma associated with a poor prognosis in most patients. The clinicopathological spectrum is variable including predominantly epidermotropic infiltrates manifesting with patches and plaques or tumors with dermal and/or subcutaneous infiltrates. The diagnosis of CGD-TCL requires the demonstration of a γ/δ+ phenotype by immunohistochemistry. We report 2 patients with epidermotropic cutaneous T-cell lymphomas displaying a γ/δ+ phenotype, but exhibiting an indolent course. In one patient, the clinical presentation was similar to mycosis fungoides in patch and plaque stage, but recurrent blister formation within the lesions was observed accompanied by fever and arthralgias, whereas the second patient presented with 2 localized erosive plaques on the left temple and dense epidermotropic and dermal diffuse and folliculotropic infiltrates of atypical small-to-medium-sized lymphocytes. These cases corroborate the view that expression of a γ/δ+ phenotype in cutaneous T-cell lymphomas per se does not portend a worse prognosis and that CGD-TCL may represent a clinically and prognostically heterogeneous group.
- MeSH
- fenotyp MeSH
- imunohistochemie MeSH
- kožní T-buněčný lymfom chemie patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádorové biomarkery analýza MeSH
- nádory kůže patologie MeSH
- prognóza MeSH
- receptory antigenů T-buněk gama-delta analýza MeSH
- senioři nad 80 let MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
BACKGROUND: CD30 is expressed in aggressive and Epstein-Barr virus-associated forms of B-cell non-Hodgkin lymphomas, but is rarely expressed by the majority of tumor cells in primary cutaneous B-cell lymphomas (CBCLs). The expression of CD30 in CBCLs may be at risk for misinterpretation as an unequivocal indicator of a highly aggressive form of the disease. OBJECTIVE: We report 4 cases of low malignant primary cutaneous follicle center lymphoma (PCFCL) with diffuse and strong expression of CD30 by the majority of neoplastic cells. RESULTS: The patients included 3 men and 1 woman with tumors on the scalp (3 patients) and chest wall (1 patient). The histologic examinations revealed a mixed, diffuse, and follicular growth pattern with CD20(+), bcl-6(+), and bcl-2(-) tumor cells. Seventy percent to 90% of the tumor cells expressed CD30. Clonal rearrangement of immunoglobulin heavy chain genes was found in 1 of 4 cases. None of the 3 cases yielded positivity for Epstein-Barr virus RNA. LIMITATIONS: The study is limited by the small number of patients. CONCLUSIONS: This rare variant of CD30(+) PCFCL needs be distinguished from CD30(+) aggressive B-cell lymphomas. CD30 in this variant of CBCLs may serve as a therapeutic target for anti-CD30 antibody-based strategies.
- MeSH
- antigen Ki-1 metabolismus MeSH
- B-buněčný lymfom epidemiologie metabolismus patologie terapie MeSH
- dospělí MeSH
- imunohistochemie MeSH
- komorbidita MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory hlavy a krku epidemiologie metabolismus patologie terapie MeSH
- nádory kůže epidemiologie metabolismus patologie terapie MeSH
- senioři MeSH
- skalp MeSH
- syndrom bazocelulárního névu epidemiologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Mycosis fungoides (MF) displays a broad spectrum of clinical, histological, and phenotypic variants with different prognostic impact. CD8⁺CD56⁺MF is very rare. So far, only 5 patients with CD8⁺CD56⁺ MF were reported to date. All of the previously described patients with this phenotypic variant were adults at the time of diagnosis and often manifested with widespread poikilodermatous skin lesions. Here we describe the clinicopathological features of a juvenile form of CD8⁺CD56⁺ non-poikilodermatous MF in a 10-year-old girl. In our patient, the disease displayed an indolent course and excellent prognosis with complete remission after treatment with UV light, topical steroids, and methotrexate. The differential diagnosis of CD8⁺ epidermotropic lymphocytic infiltrates in children is discussed, and the cases of CD8⁺CD56⁺ MF reported in the literature are reviewed.
- MeSH
- antigeny CD56 imunologie MeSH
- antigeny CD8 imunologie MeSH
- dítě MeSH
- imunofenotypizace MeSH
- lidé MeSH
- mycosis fungoides imunologie patologie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
