T lymphoblastic leukaemia with an unusual Burkitt lymphoma morphology--a case report (original) (raw)
Related papers
Problematic 'high grade' lesions in lymphoproliferative pathology
Materia Medica, 2012
High error rate in primary lymphoma diagnosis by generalist pathologists has imposed a requirement for a redesign of diagnostic services in this subspecialty. 1 In all developed countries and in most of the countries in transition, diagnosis of lymphoid proliferations has become centralised, relying on regional panels of experts and dedicated specialised laboratories. However, the initial steps in the management and subspecialist referral of patients with suspected lymphoma still rely on diagnostic skills of general histopathologists. Their detailed awareness of classification changes, diagnostic requirements and standards is essential for the success of the diagnostic pathway. Here a range of scenarios are highlighted where the outcome of the initial pathological assessment, before any specialist investigations have been carried out, could "sidetrack" the referral process and adversely affect management. The term "High Grade" in this context is used for lesions histologically characterised by high pleomorphism and "blastic" appearance and also for processes with an aggressive clinical behaviour. Over the past two decades the wealth of accumulated knowledge on the biology of lymphoid cells and lymphomas culminated in a series of classifications which emphasised the need for extensive immunophenotypic and genetic interrogation of lymphoid proliferation in the course of pathological diagnosis. 2 as a consequence gone past are the days when treatment of lymphomas could commence after morphological assessment alone. On morphological grounds so many different aggressive lymphomas may show striking similarity. Burkitt lymphoma (BL), blastoid variant of mantle cell lymphoma, lymphomas of the "grey zone" between BL and diffuse large B-cell lymphoma (dLBCL), lymphoblastic lymphoma, plasmacytoid dendritic cell neoplasm and many others may show very similar morphology. This morphological mimicry is further complicated by similarities aggressive lymphoid malignancies may in certain circumstances show with non-haematological malignancies and reactive, inflammatory conditions. Examples of this contentious spectrum are provided together with an update of the most recent classification changes and the impact this has made on the practicalities of pathological diagnosis and management. Abundant reactive lymphoid infiltrate is seen in a range of different tumours: Follicular dendritic cell tumour / sarcoma is an example of this category. This is an uncommon entity displaying a spectrum of biological behaviour involving lymph nodes and a range of extranodal sites. The tumour cells amongst the abundant reactive lymphoid infiltrate could show spindle cell morphology, epithelioid or reed-sternberg-cell features. accurate diagnosis relies on the recognition of the specific immunophenotype (Cd21, Cd23, Cd35, clusterin). 2-4 This is of benefit only if this tumour is included in the initial differential diagnosis which should also consider tumours such as classical Hodgkin lymphoma, T-cell rich B-cell lymphoma, "lymphoepithelioma-like" carcinoma, inflammatory myofibroblastic tumour, metastatic germ cell tumour, medullary carcinoma of breast, "B-type" thymomas, inflammatory pleomorphic sarcoma or interdigitating dendritic cell sarcoma. Aggressive lymphomas may be negative for commonly used lymphoid lineage markers: ALK positive large B-cell lymphoma is a rare entity characterised by a high degree of pleomorphism and epithelioid morphology. 5 This aggressive lymphoma in addition displays an aberrant phenotype, lacking expression of Cd45 and other lineage markers. The initial use of broad immunocytochemical screens may classify this lymphoma as undifferentiated malignancy. a range of haematolymphoid neoplasms may display loss of expression or are by definition characterised by the absence of markers generally considered to be robust lineage discriminators. such tumours are myeloma, plasmablastic lymphomas, anaplastic large cell lymphoma and classical Hodgkin lymphoma which may all pose a difficult differential diagnosis with non-haematological malignancies. Plasma cell myeloma as well as other haematological malignancies may also aberrantly express cytokeratins, which in the context of paucity of expression of other B-cell lineage markers could be highly confusing. 6 In addition, a common tumour such as small cell carcinoma of lung on occasions expresses
Histoplasmosis mimicking childhood non-Hodgkin lymphoma
Medical and Pediatric Oncology, 1979
Cell surface markers are becoming increasingly important in the diagnosis of malignant lymphoid diseases. We present a case of pulmonary histoplasmosis with a pleural effusion. The differential diagnosis included non-Hodgkin lymphoma because the pleural fluid cells were cytologically identical to convoluted lymphoblasts; the cells also formed rosettes with sheep erythrocytes at 37'C, suggesting that they were malignant thymus-derived lymphoblasts. Since cultures of pleural fluid were negative for bacteria and fungi, the correct diagnosis of histoplasmosis was made only after conventional histology identified Histoplasma capsulatum organisms in pleural nodules. Thus, until we have a better understanding of the significance of cell surface markers, we should continue to rely on conventional histology for the diagnosis of lymphomas.
A case of T/null anaplastic large cell lymphoma arising in lung
Bosnian journal of basic medical sciences / Udruženje basičnih mediciniskih znanosti = Association of Basic Medical Sciences, 2006
Anaplastic large cell lymphoma (ALCL) is a rare non-Hodgkin, T-cell lymphoma, representing only 2-3% of all lymphoid neoplasm's in adults according to World Health Organization (WHO). CD30 antigen-positive, large neoplastic cells characterize ALCL. We present here a 46-year-old male with pulmonary ALCL previously diagnosed with Hodgkin disease. Microscopically, atypical bi-and multinucleated cells with frequent mitoses were present. The neoplastic cells were large and had clear cytoplasm, large vesicular nuclei, and prominent nucleoli. Immunophenotypic analysis revealed LCA, vimentin and CD30 positivity. ALK immunostaining was negative. Immunohistochemical profile was consistent with ALK negative ALCL. The progression of Hodgkin lymphoma to aggressive non-Hodgkin lymphoma (ALCL in this case) is well known entity. After the diagnosis was established, our patient immediately had been referred to the Department of Hematology in order to get appropriate chemotherapy, necessary in su...
IP innovative publication pvt ltd, 2020
Introduction: Burkitt lymphoma is an aggressive type B-cell NHL. It is an infrequently occurring aggressive B-cell NHL, which occurs in children and young adults and it is probably the fastest growing tumor in humans, with exuberant proliferation. However, it is now one of the most curable conditions in the developed world. Objective: The aim of this study to analyze the clinico-pathologic, immunophenotypic, cytogenetic and molecular pathology of cases diagnosed as Burkitt lymphoma/leukemia. Materials and Methods: All cases diagnosed as Burkitt lymphoma/leukemia (BL) between2008 and 2016 were included in this study. All the relevant data was collected from Pathology and Oncology records. Peripheral blood, bone marrow smears and histopathology slides were reviewed. Stains like Giemsa, Oil -Red-O and Hematoxylin and Eosin (H&E) were reviewed. Immunohistochemistry with lymphoma markers were done on tissue sections. Immunophenotyping on marrow aspirates was done with 4-color flowcytometry. Flourescence In-situ Hybridization (FISH) assay was performed on some bone marrow aspirate samples. Results: During this study period, 37 cases were diagnosed as Burkitt leukemia / Lymphoma. There were25 male and 12 female (M: F: 2.2:1). Ten patients had hepatosplenomegaly and 8 had lymphadenopathy. B-type symptoms in 21 patients and raised LDH levels in 17 cases were observed. There were 25 patients diagnosed on bone marrow studies (21 fresh cases and 4 cases of relapse). Twelve patients were diagnosed as high grade NHL on histopathological examination (HPE). Flow analysis was done in 6 cases, which were positive for CD10, CD19, and CD20. Immuno histochemistry on biopsy specimens was done in 12 cases and 3 trephine biopsy sections were positive for B-cell lymphoma markers with high Ki67. FISH analysis was performed on 5 specimens and showed c -MYC rearrangement and t (8-14) translocation Conclusion: Burkitt lymphoma/leukemia is an aggressive variant of B cell NHL. It has classical morphology with monomorphic large lymphoid cells with prominent punched out vacuoles, positive for lipid vacuoles. Even with aggressive chemotherapy, seven cases were in remission and majority patients expired. As per our knowledge this is the largest case series from India.
Journal of Clinical Oncology, 2010
The WHO classification subdivides large granular lymphocytic leukemias (LGLLs) into two subtypes, T and natural killer (NK) LGLLs, with distinct immunophenotypes and clinical presentations. 1 Although arising from a common progenitor cell, NK cells are bone marrow-derived innate immune cells capable of cytokine production and cytotoxicity, and T cells are thymic-derived, antigen-specific lymphocytes that express a T-cell receptor (TCR). 2 Natural killer T (NKT) cells, a subset of T cells, express NK-associated antigens such as CD16, CD56, and CD57, in addition to a CD1d-restricted, semi-invariant TCR. 3 The point at which NKT cells branch from other TCR rearranged cells is not clearly defined. However, this is believed to be mediated through CD1d thymic-dependant signaling in CD4 CD8 double-positive thymocyte precursors. 4,5 NK LGLL has a CD3 -, CD8 ϩ , CD16 ϩ , CD56 ϩ phenotype, with variable expression of CD57. It presents in teenagers and young adults with "B" symptoms, lymphadenopathy, hepatosplenomegaly, lymphocytosis, anemia, and thrombocytopenia and typically follows an aggressive clinical course. 6 T-cell LGLLs are CD3 ϩ , CD8 ϩ , CD16 ϩ with a clonal TCR rearrangement and defective Fas (CD95) -mediated apoptosis. 7,8 Median age of onset is 55 years, presenting with splenomegaly, neutropenia, and recurrent infections. It is associated with rheumatoid arthritis and follows an indolent and protracted clinical course. 9 We describe the case of a teenage patient with T-cell LGLL, who despite having a CD56 immunophenotype, initially showed an aggressive clinical course more in keeping with an NKT leukemia. We discuss the reasoning behind his conservative management and the observed changes in lymphocyte count and serum cytokine/chemokine levels over an 18month period. There have been no reported cases of an initially aggressive presentation with subsequent spontaneous regression.