Linfoma anaplásico de células T grandes primario cutáneo CD30+. Serie de nueve casos / Primary cutaneous CD30+ anaplastic large T-cell lymphoma. Series of nine cases

Resumen Introducción: El linfoma anaplásico de células T grandes CD30+ es un linfoma primario cutáneo en el cual no hay evidencia de enfermedad sistémica; para su diagnóstico es necesario el estudio histopatológico. Objetivo: Presentar los casos diagnosticados en el Departamento de Dermatología del Hospital General "Dr. Manuel Gea González" con linfomas anaplásicos de células T grandes primarios cutáneos CD30+ durante un periodo de 24 años. Método: Estudio retrospectivo en el que realizó estadística descriptiva. Se recopiló información de sexo, edad, características clínicas, resultados de pruebas complementarias, tratamientos previos y actuales, reportes de los estudios histopatológicos y de inmunohistoquímica. Resultados: Entre 29 309 expedientes, se encontraron nueve casos (0.000034 %) con diagnóstico de linfoma anaplásico de células T CD30+. Se hizo la confirmación del diagnóstico histopatológico e inmunohistoquímico por dos dermatopatólogos certificados. La edad promedio fue de 61.2 años, hubo predominio del sexo femenino y de lesión papular o nodular y topografía variada como presentación clínica inicial. Conclusiones: El pronóstico del linfoma anaplásico de células T grandes CD30+ en la población estudiada fue dependiente del estadio clínico. El tratamiento en etapas tempranas tiene resultados favorables.

Abstract Introduction: CD30+ anaplastic large T cell lymphoma is a cutaneous primary lymphoma in which there is no evidence of systemic disease; histopathological study is required for its diagnosis. Objective: To present the cases diagnosed with primary cutaneous CD30+ anaplastic large T-cell lymphoma over a 24-year period in Hospital General "Dr. Manuel Gea González" Department of Dermatology. Method: Retrospective study. Descriptive statistics was carried out. Information was collected on gender, age, clinical characteristics, complementary test results, previous and current treatments, histopathological studies reports and immunohistochemistry test results. Results: Of 29 309 records, nine patients (0.000034%) with a diagnosis of CD30+ anaplastic T cell lymphoma were found. Histopathological and immunohistochemical diagnosis was confirmed by two certified dermatopathologists. Average age was 61.2 years, and there was a predominance of the female gender, with initial clinical presentation as a papular or nodular lesion and varied topography. Conclusions: The prognosis of CD30+ anaplastic large T cell lymphoma in the studied population was dependent on clinical stage. The treatment at early stages has favorable results.

Cutaneous CD30 positive anaplastic large cell lymphoma

An adult Caucasian male presented with a solitary abscess-like lesion on his left thigh. The lesion later on ulcerated and developed a central eschar. The initial differential diagnoses included deep mycosis, insect bite reaction or atypical mycobacterial infection. Histology confirmed it to be a CD30 positive anaplastic large cell lymphoma. Haematologyoncology screen was carried out and the patient started on local field radiotherapy. Localized lymphomatous lesions can be treated with surgical excision or local radiotherapy. Systemic treatments are reserved for generalized disease. The prognosis depends on the extent of involvement

Clinically unsuspected Hodgkin's lymphoma diagnosed primarily from bone marrow trephine biopsy: report of six cases.

Bone marrow may be the initial or rarely the only site of involvement in Hodgkin's lymphoma. A high index of suspicion is required to pick up the histopathological changes of Hodgkin's lesions in the bone marrow like necrosis, presence of Reed-Sternberg cell or its variant in a polymorphic background infiltrate, focal fibrosis and myxoid change especially in the absence of classical clinical picture. Bone marrow with immunohistochemistry has a valuable role in the staging and in the diagnosis of primary medullary Hodgkin's lymphoma. B-symptoms may easily masquerade as an infectious process as in all our cases the patients had fever as a presenting feature, in four of them tuberculosis was suspected clinically and two had received antitubercular therapy elsewhere. We report six human immunodeficiency virus-negative patients diagnosed over a period of 5 years in which the initial diagnosis of Hodgkin's lymphoma was suggested from bone marrow histology.

Anaplastic large cell lymphoma (ALCL) presenting as primary bone and soft tissue sarcoma--a study of 12 cases.

This study highlights the rare presentation of anaplastic large cell lymphoma as primary bone and soft tissue tumour. Twelve cases were studied. Clinical impression was non Hodgkin's lymphoma in 4 cases, sarcoma in 6 (osteosarcoma-2, Ewing's/primitive neuroectodermal tumour-1, and sarcoma NOS-3), and tuberculosis of thoracic spine in 1 and the last case involving the rib had a differential diagnosis of tuberculosis and NHL. Histology revealed round cells with eosinophilic cytoplasm and pleomorphic nuclei. Immunohistochemically all tumours were CD30 positive and 8 of 9 cases (88.9%) showed ALK-1 positivity. The pleomorphic cytomorphology ofALCL leads to confusion with the more frequent bone and soft tissue sarcomas affecting the musculoskeletal system. A high index of suspicion is necessary to initiate the correct panel of immunohistochemical markers to first confirm the lymphomatous nature of this tumour and to subsequently subclassify. This alone will lead to an accurate recognition of ALCL and the appropriate chemotherapy.

Interfollicular Hodgkin's disease: an uncommon pattern that may cause diagnostic difficulty.

Interfollicular Hodgkin's Disease is characterised by reactive follicular hyperplasia with involvement of the interfollicular area of lymph node by Hodgkin's lymphoma. It represents a peculiar pattern of focal involvement of lymph node and does not constitute a classical subtype. Its importance rests in the fact that it can be misinterpreted as one of the many causes of reactive hyperplasia of lymph node and not as Hodgkin's disease. Eleven cases of interfollicular Hodgkin's disease were diagnosed in a period of five years. Majority of the patients were less than twenty years and all had localised lymphadenopathy. Lymph node biopsy showed follicular hyperplasia with expanded interfollicular area. Careful search of the interfollicular area showed infiltration by inflammatory cells and scattered Reed-Sternberg and Hodgkin's cells. Immunohistochemistry with CD 15 and CD 30 highlighted the atypical cells. This report emphasises on the problems in diagnosis of interfollicular Hodgkin's disease.

The role of p53 and anaplastic lymphoma kinase genes in the progression of cutaneous CD30(+) lymphoproliferative diseases.

BACKGROUND AND OBJECTIVES: Molecular events that precede transformations from lymphomatoid palulosis (LyP) to mycosis fungoides (MF) or to cutaneous anaplastic large cell lymphoma (ALCL) in the CD 30(+) cutaneous lymphoproliferative diseases (LPDs) are not known. Altered p(53) gene may be responsible since overexpression of the p(53) gene product has been reported in higher, but not in lower grades of cutaneous lymphomas. Expression of the anaplastic lymphoma kinase (ALK) gene product has also been described as an important prognostic indicator in ALCL. ALK positive systemic nodal ALCL are associated with a good prognosis. However, primary cutaneous ALCL that are ALK negative have a better overall survival. The current study was done to see if mutated p(53) gene or ALK reactivity were poor prognostic indicators in those patients with CD 30(+) cutaneous LPD who showed progression of the disease. METHODS: Mutations of the p(53) gene and expression of the ALK gene product were analysed in 36 patients (23 of LyP and 13 of CD30(+) cutaneous ALCL). Follow up data were available up till 5 yr in all patients. RESULTS: Clinical progression or histological transformation in sequential biopsy specimens was found in 9 of 36 patients. Transformation occurred in 5 patients (4 from LyP to ALCL and 1 from MF to ALCL) and clinical progression in 4 patients with ALCL. Mutations of the p(53) gene were found in two biopsy specimens of LyP. ALK gene products were not detected in any of the biopsy specimens of LyP and primary cutaneous ALCL. INTERPRETATION AND CONCLUSION: Although 9 of 36 patients with cutaneous CD30(+) LPDs had progression of their disease, neither mutations of the p(53) gene nor ALK immunoreactivity were found in any of these biopsies. The two cases of LyP that had mutated p(53) gene in their biopsy specimens showed no progression of their disease in the 5 yr follow up period. It appears that these molecular events may not play any significant role in the pathogenesis, progression or transformation of cutaneous CD30(+) LPD.

A Case of Primary Gastric CD30-Positive Anaplastic Large-Cell Lymphoma

Gastric CD30-positive anaplastic large-cell lymphoma is a very rare disease. It is sometimes difficult to distinguish it from undifferentiated carcinoma, sarcoma and so on. We report here on a case of primary gastric anaplastic large-cell lymphoma. A 50-yr-old woman complained of epigastric pain and severe chest pain for 1 week. The gastroendoscopic examination revealed geographic mucosal irregularities with shallow ulceration at the antrum. She underwent a total gastrectomy. The gross finding of the resected stomach was an 8 x 4.5 cm sized ulceroinfiltrative lesion at the pyloric antrum along the lesser curvature. The microscopic examination revealed diffuse and solid proliferations of large atypical cells with pleomorphic nuclei. Immunohistochemically, the tumor cells were positive for CD30, vimentin and CD3, and this was a finding compatible with anaplastic large-cell lymphoma. To the best of our knowledge, this is the first such reported case in Korea.

Epstein-Barr Virus-Associated Peripheral T-Cell Lymphoma involving Spleen in a Renal Transplant Patient

The incidence of posttransplantation lymphoproliferative disorders (PTLDs) has increased in recent years. Although rare, various types of T-cell lymphoma have been reported and their association with Epstein-Barr virus (EBV) has been compared with B-cell PTLDs. We report a case of splenic peripheral T-cell lymphoma occurring in a 47-yr-old male patient 7 yr after renal allograft transplantation. The spleen showed sinusoidal proliferation of focal CD30 positive, large, atypical lymphoid cells. Positivity for CD3 and cytolytic granule-associated proteins was also demonstrated in the tumor cells, while anaplastic large cell lymphoma kinase (ALK) and CD8 were not expressed. Strong nuclear signals for EBV mRNA were noted by EBER1 in situ hybridization. A molecular genetic study demonstrated a rearrangement of the gamma T-cell receptor gene. To our knowledge, this case is unique in terms of a posttransplant T-cell lymphoma that shows focal CD30, cytolytic granule-associated proteins, and EBV positivity.

Mediastinal Castleman's disease complicated by follicular dendritic cell tumour.

We describe a rare case of follicular dendritic cell tumour which had arisen over the background of hyaline vascular type of Castleman's disease at the mediastinal location. Constellation of histology and immunohistochemistry using CD21 antibody and non-reactivity to CD15, CD30, cytokeratin and epithelial membrane antigen helped us diagnose this case. The literature is reviewed, specially with reference to the genesis of follicular dendritic cell neoplasm at the backdrop of Castleman's disease and its clinical relevance.