Abstract:The term “Primary Cutaneous B-Cell Lymphoma” (PCBCL) comprehends a variety of lymphoproliferative disorders characterized by a clonal proliferation of B-cells primarily involving the skin. The absence of evident extra-cutaneous disease must be confirmed after six-month follow-up in order to exclude a nodal non-Hodgkin's lymphoma (NHL) with secondary cutaneous involvement, which may have a completely different clinical behavior and prognosis. In this article, we have summarized the clinico-pathological features… Show more
“…Primary cutaneous FCL and DLBCL‐LT are two different B‐cell lymphoma subtypes whose etiologic factors are largely unknown and characterized by a very different clinical course (La Selva et al, ). The onset of specific types of non‐Hodgkin lymphomas (NHL) in the setting of autoimmune diseases has been widely debated (Ekström Smedby et al, ) and, regarding pemphigus, a large cross‐sectional study has shown a significant association between pemphigus and chronic leukemia, multiple myeloma, and NHL (Kridin et al, ).…”
It is known that individuals with immune dysregulation have an increased risk of non‐Hodgkin lymphoma. This association has been proven for pemphigus as well as for other autoimmune disease. We describe the development of cutaneous B‐cell lymphoma in two patients affected by long‐standing pemphigus vulgaris and pemphigus foliaceus (i.e., characterized by histological and immunopathological features different from those of paraneoplastic pemphigus). In both cases, a therapy with rituximab allowed to achieve the complete remission for the lymphoproliferative disease (never recurred at follow up) and a substantial long‐term improvement of the clinical manifestations of pemphigus, although persistent to serological disease and occasional recurrences. We suggest that clinicians should consider that patients with long‐standing pemphigus, both vulgaris and foliaceus, may develop primary cutaneous B‐cell lymphomas, as shown in our report, and in these cases the treatment with rituximab is elective, providing a therapeutic option for both low‐grade or follicular, CD20‐positive, B‐cell non‐Hodgkin lymphomas and pemphigus. Nevertheless, as shown in our cases, a constant surveillance for pemphigus is necessary.
“…Primary cutaneous FCL and DLBCL‐LT are two different B‐cell lymphoma subtypes whose etiologic factors are largely unknown and characterized by a very different clinical course (La Selva et al, ). The onset of specific types of non‐Hodgkin lymphomas (NHL) in the setting of autoimmune diseases has been widely debated (Ekström Smedby et al, ) and, regarding pemphigus, a large cross‐sectional study has shown a significant association between pemphigus and chronic leukemia, multiple myeloma, and NHL (Kridin et al, ).…”
It is known that individuals with immune dysregulation have an increased risk of non‐Hodgkin lymphoma. This association has been proven for pemphigus as well as for other autoimmune disease. We describe the development of cutaneous B‐cell lymphoma in two patients affected by long‐standing pemphigus vulgaris and pemphigus foliaceus (i.e., characterized by histological and immunopathological features different from those of paraneoplastic pemphigus). In both cases, a therapy with rituximab allowed to achieve the complete remission for the lymphoproliferative disease (never recurred at follow up) and a substantial long‐term improvement of the clinical manifestations of pemphigus, although persistent to serological disease and occasional recurrences. We suggest that clinicians should consider that patients with long‐standing pemphigus, both vulgaris and foliaceus, may develop primary cutaneous B‐cell lymphomas, as shown in our report, and in these cases the treatment with rituximab is elective, providing a therapeutic option for both low‐grade or follicular, CD20‐positive, B‐cell non‐Hodgkin lymphomas and pemphigus. Nevertheless, as shown in our cases, a constant surveillance for pemphigus is necessary.
“…Since lymphoma is a systemic disease, treatment for multicentric and cutaneous lymphoma involves systemic chemotherapy [2,12,20]. The best response rates have been seen when using multidrug protocols incorporating doxorubicin [1,2,12]; however, in cases of cutaneous lymphoma, responses to treatment may be short lived.…”
Section: Case Reports In Veterinary Medicinementioning
A 9-year-old male castrated mixed-breed dog from the West Indies was presented for multiple, nonpainful, nodular, circumscribed, subcutaneous masses located on the dorsum, lateral thorax, head, forelimbs, and scrotum. En bloc surgical resection of a mass on the right paw, left forehead, and left medial forelimb with proportional margins was performed. Three punch biopsies were taken from the masses located along the right lateral flank. Histopathologic and immunohistochemistry (IHC) examination of the skin lesions revealed a diagnosis of subcutaneous B cell lymphoma. Thoracic radiographs and abdominal ultrasound were negative for signs of gross metastatic disease. Chemotherapeutic intervention included intravenous doxorubicin (30 mg/m 2 ) administered at 3-week intervals for 3 treatments and oral prednisone (2 mg/kg/d) for 3 weeks. There were no complications following the chemotherapy protocol. As of 3 years, there has been no regrowth of the tumors and the patient continues to be cancer free. To date, this is the first reported case of subcutaneous B cell lymphoma diagnosed in a dog treated successfully with gross tumor resection and chemotherapy.
“…In 1996, the term "primary cutaneous large B-cell lymphoma of the leg" was proposed, which was used to classify it as a distinctive subgroup in the EORTC classification. In the last WHO-EORTC classification, this entity was finally defined PCDLBCL, leg type to reflect the predominant but not exclusive anatomic location of the lesions (Swerdlow, et al, 2008. Selva, et al, 2017.…”
Section: Clinics and Pathologymentioning
confidence: 99%
“…BCL-2, MUM1/IRF4, and FOX-P1 are strongly expressed regardless of the location of the skin lesions. A study suggests that cell-of origin is germinal center experienced and superantigen driven selected B-cell, in a stage between germinal center B-cell and plasma cell (Swerdlow, et al , 2008. Selva, et al, 2017.…”
Section: Phenotype/cell Stem Originmentioning
confidence: 99%
“…PCDLBCL, LT represents 5%-10% of all PCBCLs commonly presenting in elderly females (male:female 1:3-4), with a peak incidence in the seventh decade of life (Swerdlow, et al , 2008. Selva, et al, 2017.…”
Primary cutaneous diffuse large B-cell lymphoma (PCDLBCL) leg type is an aggressive type of CBCL, characterized by skin lesions mainly on the legs and a predominance of diffuse sheets of centroblasts and immunoblasts. Given the poor prognosis, old age at onset, frequent relapses, and extracutaneous spread, PCDLBCL, leg type is a distinct type of cutaneous lyphoma. Here the clinicopathology of extranodal lymphomas will be discussed.
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