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1.
Am J Surg Pathol ; 2019 Nov 22.
Artigo em Inglês | MEDLINE | ID: mdl-31764221

RESUMO

The diagnosis of nodal marginal zone lymphoma (NMZL) can be challenging, with the differential diagnosis including other low-grade B-cell lymphomas, reactive hyperplasia, and even some cases of peripheral T-cell lymphoma (PTCL). PTCL may have a perifollicular growth pattern mimicking NMZL. We and others have noted an atypical distribution of T-follicular helper (TFH) cells in some cases of NMZL. This study was prompted by the diagnosis of NMZL in several cases in which a marked increase of TFH cells, as determined by staining for programmed death-1 (PD1), had prompted suspicion for a diagnosis of PTCL. We analyzed PD1 staining in 48 cases of NMZL to characterize the extent and pattern of the PD1-positive infiltrate. Three main patterns of PD1 staining were identified: follicular pattern (peripheral, n=16; central, n=9; mixed, n=3), diffuse pattern (n=4), and a reduced or normal staining pattern in residual follicles (n=16). A comprehensive analysis of other TFH markers was undertaken in 14 cases with a high content of PD1-positive cells that were confirmed as B-cell lymphoma by clonality analysis. We describe in detail 5 of these cases in which PTCL was an initial consideration. This study illuminates the diverse immunohistochemical patterns encountered in NMZL and highlights a diagnostic pitfall important for diagnostic accuracy.

2.
Am J Surg Pathol ; 43(12): 1720-1725, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31368914

RESUMO

Systemic high-grade B-cell lymphomas (HGBCLs) with MYC gene rearrangements are clinically aggressive. In situ lesions with indolent behavior have not been described to date. We have identified 2 cases of in situ B-cell neoplasms with MYC rearrangements (IS-BCN, MYC) occurring, and focally confined to ≤4 lymphoid follicles in otherwise healthy individuals and without clinical progression despite minimal intervention (surgical only). Morphologically similar to systemic HGBCLs, the low power view of these lesions showed a starry sky pattern with numerous mitotic figures. High power imaging demonstrated these cells to be medium-large in size with irregular nuclear contours, immature chromatin, and prominent nucleoli. Immunophenotypically these cells were light chain restricted, positive for CD20, CD10, c-Myc, and dim or negative for BCL2 with a Ki67 proliferative index of >95%. By fluorescence in situ hybridization studies, we detected MYC translocations in these cells but no rearrangements in BCL2 or BCL6. Microdissection of neoplastic cells in these patients followed by targeted next-generation sequencing identified a mutation in MYC, D2N, and an indel in TNFRSF14. Mutations in ID3 or TCF3 were not identified. Although rare, these lesions should be separated from HGBCLs involving follicles but with systemic spread which has been previously described. Unlike systemic lymphomas with MYC gene rearrangements, these in situ B-cell neoplasms with MYC rearrangements did not require systemic therapy and no progression has been seen in either patient beyond 1 year (29 and 16 mo). Our work offers pathologic and biologic insight into the early process of B-cell neoplasia.

3.
Int J Gynecol Pathol ; 2019 Jul 03.
Artigo em Inglês | MEDLINE | ID: mdl-31274698

RESUMO

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a self-limited histiocytic disorder of unclear etiology which most commonly presents with cervical lymphadenopathy. Purely extranodal presentation of RDD is uncommon, and isolated intralymphatic/intravascular confinement of this entity has not previously been described. We report a 16-yr-old female who presented with vaginal swelling and mass-like enlargement of the right labia. The mass had been present for nearly a year without pain or tenderness. Clinically, the lesion was thought to be a Bartholin gland cyst. Following surgical resection, histologic examination demonstrated a hypocellular myxedematous stroma with a mixture of ectatic thin and thick-walled vessels within which there were numerous collections of histiocytes, lymphocytes, and plasma cells. The histopathologic differential diagnosis included localized vulvar lymphedema, a specialized genital tract neoplasm, and childhood asymmetric labium majus enlargement. The histiocytes showed occasional plasma cells and lymphocytes within their cytoplasm, consistent with emperipolesis. Immunohistochemical studies showed that the histiocytes expressed CD163 and S100, while ERG and D2-40 highlighted their intralymphatic confinement, ultimately leading to the diagnosis of intralymphatic RDD. Intralymphatic RDD may present as vulvar lymphedema and can potentially mimic other myxedematous neoplasms of the vulvovaginal region.

4.
Blood Adv ; 2(5): 481-491, 2018 03 13.
Artigo em Inglês | MEDLINE | ID: mdl-29496669

RESUMO

Castleman disease (CD) is a rare lymphoproliferative disorder subclassified as unicentric CD (UCD) or multicentric CD (MCD) based on clinical features and the distribution of enlarged lymph nodes with characteristic histopathology. MCD can be further subtyped based on human herpes virus 8 (HHV8) infection into HHV8-associated MCD, HHV8-/idiopathic MCD (iMCD), and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin change (POEMS)-associated MCD. In a subset of cases of UCD, an associated follicular dendritic cell sarcoma (FDCS) may be seen. Although numerous reports of the clinical and histologic features of UCD, MCD, and FDCS exist, an understanding of the genetic and epigenetic landscape of these rare diseases is lacking. Given this paucity of knowledge, we analyzed 15 cases of UCD and 3 cases of iMCD by targeted next-generation sequencing (NGS; 405 genes) and 3 cases of FDCS associated with UCD hyaline vascular variant (UCD-HVV) by whole-exome sequencing. Common amplifications of ETS1, PTPN6, and TGFBR2 were seen in 1 iMCD and 1 UCD case; the iMCD case also had a somatic DNMT3A L295Q mutation. This iMCD patient also showed clinicopathologic features consistent with a specific subtype known as Castleman-Kojima disease (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly [TAFRO] clinical subtype). Additionally, 1 case of UCD-HVV showed amplification of the cluster of histone genes on chromosome 6p. FDCS associated with UCD-HVV showed mutations and copy number changes in known oncogenes, tumor suppressors, and chromatin structural-remodeling proteins.


Assuntos
Hiperplasia do Linfonodo Gigante/genética , Sarcoma de Células Dendríticas Foliculares/genética , Sequenciamento de Nucleotídeos em Larga Escala , Adolescente , Adulto , Idoso , Análise Mutacional de DNA , Exoma/genética , Feminino , HIV , Herpesvirus Humano 8 , Humanos , Linfonodos/patologia , Masculino , Pessoa de Meia-Idade , Adulto Jovem
5.
PLoS One ; 11(3): e0151735, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26991267

RESUMO

We sought to address the significance of isolated follicles that exhibit atypical morphologic features that may be mistaken for lymphoma in a background of reactive lymphoid tissue. Seven cases that demonstrated centroblast-predominant isolated follicles and absent BCL2 staining in otherwise-normal lymph nodes were studied. Four of seven cases showed clonal B-cell proliferations amid a polyclonal B cell background; all cases lacked the IGH-BCL2 translocation and BCL2 protein expression. Although three patients had invasive breast carcinoma at other sites, none were associated with systemic lymphoma up to 44 months after diagnosis. The immunoarchitectural features of these highly unusual cases raise the question of whether a predominance of centroblasts and/or absence of BCL2 expression could represent a precursor lesion or atypical reactive phenomenon. Differentiating such cases from follicular lymphoma or another mimic is critical, lest patients with indolent proliferations be exposed to unnecessarily aggressive treatment.


Assuntos
Linfócitos B/citologia , Cromossomos Humanos Par 14/genética , Cromossomos Humanos Par 18/genética , Linfonodos/patologia , Linfoma Folicular/patologia , Proteínas Proto-Oncogênicas c-bcl-2/genética , Neoplasias da Mama/patologia , Proliferação de Células/genética , Criança , Feminino , Humanos , Cadeias kappa de Imunoglobulina/genética , Cadeias lambda de Imunoglobulina/genética , Hibridização in Situ Fluorescente , Linfoma Folicular/genética , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Translocação Genética/genética
6.
Hum Pathol ; 48: 9-17, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26772393

RESUMO

Composite diffuse large B-cell lymphomas (DLBCLs) with peripheral T-cell lymphomas (PTCLs) are rare co-occurrences with poorly understood pathologic features. Herein, we describe 15 distinct cases of DLBCL occurring in association with PTCL, including angioimmunoblastic T-cell lymphoma (AITL; n = 12) and PTCL, not otherwise specified (n = 3). Sheets of large B cells were seen in all cases, with Hodgkin/Reed-Sternberg-like (HRS-L) cells present in 6 cases. When compared to cases of AITL without DLBCL, HRS-L cells were more frequently seen in cases of AITL with DLBCL (P = .02). Epstein-Barr virus (EBV) expression was seen in 10 of 15 cases, and in those with HRS-L cells, EBV expression was detected invariably in at least a subset of the HRS-L cells. MYC gene rearrangements were consistently absent, although 6 of the 10 cases showed MYC overexpression by immunohistochemistry in the neoplastic B cells; a frequency significantly increased compared to other cases of DLBCL not associated with a T-cell lymphoma: 29 of 166 (P = .005). In addition, when MYC was overexpressed in DLBCL, it was also weakly present in the HRS-L cells. The increased and frequent morphologic presence of HRS-L cells in association with this composite lymphoma raises a possible link between their occurrence and DLBCLs in PTCLs; furthermore, the frequent detection of MYC protein expression and EBV infection in these cases suggests a possible role of these pathways in B-cell lymphomagenesis.


Assuntos
Infecções por Vírus Epstein-Barr/epidemiologia , Linfoma Difuso de Grandes Células B/patologia , Linfoma de Células T/patologia , Neoplasias Primárias Múltiplas/patologia , Infecções Tumorais por Vírus/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Infecções por Vírus Epstein-Barr/complicações , Feminino , Herpesvirus Humano 4 , Humanos , Imuno-Histoquímica , Hibridização In Situ , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/virologia , Linfoma de Células T/complicações , Linfoma de Células T/virologia , Masculino , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/complicações , Neoplasias Primárias Múltiplas/virologia , Proteínas Proto-Oncogênicas c-myc/biossíntese , Análise Serial de Tecidos , Infecções Tumorais por Vírus/complicações
7.
Hum Pathol ; 46(11): 1655-61, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26410017

RESUMO

Progressive transformation of germinal centers (PTGC) has been frequently described in association with Hodgkin lymphoma, particularly nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). The aim of this study was to evaluate morphologic features of PTGC for better delineation of PTGC from early involvement by NLPHL. A total of 160 cases of PTGC were evaluated and included in the following 3 groups: 93 patients with PTGC who never developed a lymphoma, 23 patients with synchronous PTGC and NLPHL, and 44 patients with PTGC with antecedent or subsequent history of lymphoma. By histopathologic evaluation, 5 patterns of PTGC that reflected progressive dismantling of germinal centers were identified. There was no difference in the distribution of patterns 1 to 4 among the 3 groups of PTGC; however, in patients showing synchronous involvement of PTGC and NLPHL, pattern 5, which resembles a naïve B-cell follicle, was significantly more frequently observed (14/23) when compared with patients with PTGC who never developed a lymphoma (30/93; P = .0161). Furthermore, recognition of the spectrum of immunoarchitectural patterns of PTGC, including architectural and cytologic features, was helpful to better differentiate nodules involved by PTGC from NLPHL.


Assuntos
Linfócitos B/patologia , Transformação Celular Neoplásica/patologia , Centro Germinativo/patologia , Doença de Hodgkin/patologia , Linfoma/patologia , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
9.
Hum Pathol ; 46(1): 74-83, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25456392

RESUMO

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is characterized by nodular or nodular and diffuse growth of scattered large neoplastic B cells associated with follicular dendritic cell (FDC) meshworks. Variant patterns, which at least focally show a T-cell-rich background, and rare cases lacking FDC meshworks that overlap with T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) are also recognized. We reviewed 195 cases spanning the diagnostic spectrum of NLPHL and THRLBCL and identified 5 cases with distinctive features that were difficult to classify according to the World Health Organization criteria or previously described variants. Clinically, they involved peripheral and central lymph node sites or the mediastinum, and the majority also had recurrent disease. Four cases showed large T-cell-rich nodules with fibrosis, and 1 showed diffuse THRLBCL-like pattern with a minor component of nodularity. All cases completely lacked FDC meshworks despite a prominent nodular growth pattern. Large atypical cells in all cases were CD20+ CD30- CD15- B cells, although a small subset (<10%) of CD30+ and CD15+ large cells were seen in 1 case. In 4 cases, the background mainly contained CD4+ PD-1+ or CD57+ T cells that ringed large atypical B cells. In 1 case, B-cell predominance and a ringing pattern of CD57+ T cells were noted in nodules, whereas they were lacking in the diffuse areas. Recognition of these variant cases expands the spectrum between NLPHL and THRLBCL and points to the need for further refinement of diagnostic criteria for appropriate classification and clinical management.


Assuntos
Linfócitos B/imunologia , Células Dendríticas Foliculares/imunologia , Doença de Hodgkin/diagnóstico , Linfonodos/imunologia , Linfoma Difuso de Grandes Células B/diagnóstico , Linfócitos T/imunologia , Adulto , Idoso , Biomarcadores Tumorais/análise , Biópsia , Diagnóstico Diferencial , Fibrose , Doença de Hodgkin/classificação , Doença de Hodgkin/imunologia , Doença de Hodgkin/patologia , Humanos , Imuno-Histoquímica , Imunofenotipagem , Linfonodos/patologia , Linfoma Difuso de Grandes Células B/classificação , Linfoma Difuso de Grandes Células B/imunologia , Linfoma Difuso de Grandes Células B/patologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes
10.
J Cutan Pathol ; 42(1): 46-9, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25384366

RESUMO

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon non-Hodgkins primary cutaneous lymphoma that typically presents as subcutaneous nodules on the extremities or trunk. Here, we report an unusual case of systemic panniculitic T-cell lymphoma with predominantly mesenteric extra-cutaneous involvement and an aggressive clinical course with histopathologic and immunophenotypic features that mimic SPTCL. This case serves to expand the body of literature regarding systemic SPTCL-like disorders with prominent extra-cutaneous involvement.


Assuntos
Linfoma Cutâneo de Células T/patologia , Linfoma de Células T/patologia , Paniculite/patologia , Gordura Subcutânea/patologia , Adulto , Diagnóstico Diferencial , Humanos , Masculino , Prognóstico , Neoplasias Cutâneas/patologia
11.
Diagn Pathol ; 9: 144, 2014 Jul 21.
Artigo em Inglês | MEDLINE | ID: mdl-25047073

RESUMO

BACKGROUND: Detection of B cell clonality is useful for assisting in the diagnosis of B cell lymphomas. Clonality assessment can be accomplished through evaluation of KAPPA and LAMBDA light chain expression. Currently, only slide based methods are available for the majority of patient biopsies and do not detect light chain protein or mRNA in many B-cell lymphomas. Herein we evaluated a new method, known as colorimetric in situ hybridization (CISH), with improved sensitivity and multiplexing capacity, for its usefulness in clonality detection in mature B cell malignancies. METHODS: The KAPPA and LAMBDA ISH was performed on a Ventana Benchmark XT utilizing two color chromogenetic detection. The probes comprised 2 haptenated riboprobes each approximately 500 base pairs long directed against the conserved regions of either KAPPA or LAMBDA mRNA. The dual colors consisted of silver deposition (black) for KAPPA light chain and a novel (pink) chromogen for LAMBDA light chain. Following optimization, CISH allowed visualization of mRNA in benign B cells in reactive tissues including germinal center, mantle zone, and post-germinal center cells. We then identified 79 cases of B cell lymphoma with formalin-fixed paraffin-embedded (FFPE) biopsies including: follicular (36 cases), mantle cell (6 cases), marginal zone (12 cases), lymphoplasmacytic (6 cases), small lymphocytic (4 cases), and diffuse large B cell (15 cases), which were selected on the basis of either prior flow cytometry or immunohistochemistry (IHC) results to serve as the predicate, "gold standard," comparator. RESULTS: 39/79 (49.4%) cases were classified as KAPPA and 29/79 (36.7%) as LAMBDA light chain restricted; while 9/79 (11.3%) cases were classified as indeterminate. Of the 70 cases with KAPPA or LAMBDA light chain restricted CISH, 69/70 (98.6%) were concordant with the reference method, while 1/70 (1.4%) was discordant. CONCLUSIONS: Optimized CISH detected lower levels of mRNA than can be visualized with current slide based methods, making clonality assessment in FFPE biopsies possible for mature B cell neoplasms. In this preliminary study, CISH was highly accurate compared to flow cytometry or IHC. CISH offers the possibility of wider applicability of light chain ISH and is likely to become a useful diagnostic tool. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1430491067123856.


Assuntos
Cadeias kappa de Imunoglobulina/análise , Cadeias lambda de Imunoglobulina/análise , Hibridização In Situ/métodos , Linfoma de Células B/diagnóstico , RNA Mensageiro/análise , Citometria de Fluxo , Humanos , Cadeias kappa de Imunoglobulina/genética , Cadeias lambda de Imunoglobulina/genética , Imuno-Histoquímica , Linfoma de Células B/genética
12.
Int J Gynecol Pathol ; 33(4): 432-6, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24901405

RESUMO

We report a unique case of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) involving the uterus. A 63-yr-old female with a history of parathyroid adenoma and cavernous sinus meningioma underwent total abdominal hysterectomy for a possible uterine malignancy. The histologic findings consisted of a nodular, mass-like infiltration of the myometrium by clusters, cords, and sheets of CD163-positve, S100-positive histiocytes with lymphocytophagocytosis (emperipolesis). The cells were negative for CD1a and langerin. Occasional plasma cells and erythrocytes were also present. Most of the histiocytes had pale, vacuolated, or foamy cytoplasm. In all cases, the nuclei were small and eccentric. No mitotic figures were identified. Two prior cases of Rosai-Dorfman disease have been reported in the female genital tract: 1 in the cervix and 1 in the bilateral ovaries. Rosai-Dorfman disease should be added to the differential diagnosis of histiocyte-rich lesions in the female genital tract. The diagnosis should be strongly considered in the presence of the characteristic histology with lymphocytophagocytosis (emperipolesis). A limited immunohistochemical panel consisting of CD163, S100, and CD1a and/or langerin will confirm the diagnosis in most cases.


Assuntos
Histiocitose Sinusal/patologia , Doenças Linfáticas/patologia , Doenças Uterinas/patologia , Útero/patologia , Feminino , Histiócitos/patologia , Humanos , Pessoa de Meia-Idade
13.
Am J Surg Pathol ; 38(12): 1655-63, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-24921642

RESUMO

Reactive immunoblastic proliferations can histologically mimic classical Hodgkin lymphoma (CHL), and show diffuse CD30 expression in large cells. The lack of expression of CD15 in a subset of CHL further complicates their separation from immunoblastic proliferations. Loss of expression of B-cell transcription factors is frequently exploited in making a diagnosis of CHL; however, the staining patterns of B-cell transcription factors in immunoblastic proliferations have not been extensively studied. Thirty-three cases of reactive immunoblastic proliferations were evaluated using a panel of immunohistochemistry for CD30, CD15, CD20, CD3, κ, λ, CD45RB, MUM1, PAX5, OCT2, and BOB.1, as well as Epstein-Barr virus (EBV)/EBV-encoded ribonucleic acid in situ hybridization. A newly developed dual-color chromogenic in situ hybridization technology for detection of κ/λ mRNAs was also used. The majority of immunoblasts expressed CD30 in 14 of 33 (42%) cases; none expressed CD15. Loss or weak expression of at least 1 transcription factor in B immunoblasts, most commonly PAX5, was noted in 24 of 29 (83%) cases. A polytypic light chain expression pattern was detected by immunohistochemistry in 14 of 22 (63.6%) cases and by dual-color chromogenic in situ hybridization in 9 of 10 (90%) cases studied. EBV-encoded ribonucleic acid was detected in 8 of 33 (24.2%) cases, 5 of which were clinically unrelated to infectious mononucleosis. We conclude that B-cell transcription factors can show loss or weak expression in a significant proportion of reactive immunoblastic proliferations, and, therefore, staining for B-cell transcription factors together with CD30 should be interpreted with caution before a diagnosis of CHL is made.


Assuntos
Linfócitos B/patologia , Diagnóstico Diferencial , Doença de Hodgkin/diagnóstico , Pseudolinfoma/diagnóstico , Fatores de Transcrição/biossíntese , Adolescente , Adulto , Idoso , Linfócitos B/metabolismo , Biomarcadores Tumorais/análise , Criança , Feminino , Humanos , Imuno-Histoquímica , Hibridização In Situ , Masculino , Pessoa de Meia-Idade , Pseudolinfoma/metabolismo , Fatores de Transcrição/análise , Adulto Jovem
14.
Am J Surg Pathol ; 38(9): 1298-304, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24618611

RESUMO

Although indolent T-lymphoblastic proliferations (iT-LBP) are rare, this diagnosis should be excluded in any patient with an extrathymic proliferation of immature TdT+T cells. Unlike T-lymphoblastic leukemia/lymphoma, patients with iT-LBP do not require chemotherapy. We report a case of iT-LBP with disseminated multinodal involvement in an otherwise healthy 49-year-old woman. Multiple lymph node biopsies were performed over the course of several months demonstrating persistent and anatomically diffuse involvement. Over 18 months, and without therapy, she has remained healthy, and her lymphadenopathy significantly improved. No bone marrow or peripheral blood involvement was ever identified. Atypical T cells showed an immunophenotypic spectrum of T-cell antigen expression with partial CD33 on a subset of T cells detected by both flow cytometry and immunohistochemistry. Both T-cell clonality and Human Androgen Receptor Assay (HUMARA) studies, performed on lymph node biopsy specimens, were negative. This case represents the first detailed clinical, morphologic, molecular, and immunophenotypic description of disseminated multinodal involvement by nonclonal iT-LBP with partial CD33 expression on T cells.


Assuntos
Biomarcadores Tumorais/análise , Proliferação de Células , Linfonodos/imunologia , Ativação Linfocitária , Transtornos Linfoproliferativos/imunologia , Lectina 3 Semelhante a Ig de Ligação ao Ácido Siálico/análise , Linfócitos T/imunologia , Adulto , Biópsia , Diagnóstico Diferencial , Feminino , Citometria de Fluxo , Humanos , Imuno-Histoquímica , Imunofenotipagem , Linfonodos/patologia , Transtornos Linfoproliferativos/patologia , Valor Preditivo dos Testes , Prognóstico , Linfócitos T/patologia , Fatores de Tempo
15.
Blood ; 122(22): 3599-606, 2013 Nov 21.
Artigo em Inglês | MEDLINE | ID: mdl-24009234

RESUMO

Primary gastrointestinal (GI) T-cell lymphoma is an infrequent and aggressive disease. However, rare indolent clonal T-cell proliferations in the GI tract have been described. We report 10 cases of GI involvement by an indolent T-cell lymphoproliferative disease, including 6 men and 4 women with a median age of 48 years (range, 15-77 years). Presenting symptoms included abdominal pain, diarrhea, vomiting, food intolerance, and dyspepsia. The lesions involved oral cavity, esophagus, stomach, small intestine, and colon. The infiltrates were dense, but nondestructive, and composed of small, mature-appearing lymphoid cells. Eight cases were CD4(-)/CD8(+), 1 was CD4(+)/CD8(-), and another was CD4(-)/CD8(-). T-cell receptor-γ chain gene rearrangement identified a clonal population in all 10 cases. There was no evidence of STAT3 SH2 domain mutation or activation. Six patients received chemotherapy because of an initial diagnosis of peripheral T-cell lymphoma, with little or no response, whereas the other 4 were followed without therapy. After a median follow-up of 38 months (range, 9-175 months), 9 patients were alive with persistent disease and 1 was free of disease. We propose the name "indolent T-LPD of the GI tract" for these lesions that can easily be mistaken for intestinal peripheral T-cell lymphoma, and lead to aggressive therapy.


Assuntos
Gastroenteropatias/patologia , Transtornos Linfoproliferativos/patologia , Linfócitos T/patologia , Adolescente , Adulto , Idoso , Antígenos CD/metabolismo , Diagnóstico Diferencial , Linfoma de Células T Associado a Enteropatia/imunologia , Linfoma de Células T Associado a Enteropatia/patologia , Feminino , Gastroenteropatias/genética , Gastroenteropatias/imunologia , Neoplasias Gastrointestinais/imunologia , Neoplasias Gastrointestinais/patologia , Rearranjo Gênico da Cadeia gama dos Receptores de Antígenos dos Linfócitos T , Humanos , Linfoma de Células T Periférico/imunologia , Linfoma de Células T Periférico/patologia , Transtornos Linfoproliferativos/genética , Transtornos Linfoproliferativos/imunologia , Masculino , Pessoa de Meia-Idade , Mutação , Fator de Transcrição STAT3/genética , Fator de Transcrição STAT3/metabolismo , Linfócitos T/imunologia , Terminologia como Assunto
16.
Blood ; 122(6): 981-7, 2013 Aug 08.
Artigo em Inglês | MEDLINE | ID: mdl-23777769

RESUMO

Recent studies report an improvement in overall survival (OS) of patients with follicular lymphoma (FL). Previously untreated patients with grade 1 to 2 FL treated at Stanford University from 1960-2003 were identified. Four eras were considered: era 1, pre-anthracycline (1960-1975, n = 180); era 2, anthracycline (1976-1986, n = 426); era 3, aggressive chemotherapy/purine analogs (1987-1996, n = 471); and era 4, rituximab (1997-2003, n = 257). Clinical characteristics, patterns of care, and survival were assessed. Observed OS was compared with the expected OS calculated from Berkeley Mortality Database life tables derived from population matched by gender and age at the time of diagnosis. The median OS was 13.6 years. Age, gender, and stage did not differ across the eras. Although primary treatment varied, event-free survival after the first treatment did not differ between eras (P = .17). Median OS improved from 11 years in eras 1 and 2 to 18.4 years in era 3 and has not yet been reached for era 4 (P < .001), with no suggestion of a plateau in any era. These improvements in OS exceeded improvements in survival in the general population during the same period. Several factors, including better supportive care and effective therapies for relapsed disease, are likely responsible for this improvement.


Assuntos
Linfoma Folicular/tratamento farmacológico , Linfoma Folicular/mortalidade , Centros Médicos Acadêmicos , Adulto , Idoso , Idoso de 80 Anos ou mais , Antraciclinas/uso terapêutico , Anticorpos Monoclonais Murinos/uso terapêutico , Antineoplásicos/uso terapêutico , California , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Rituximab , Análise de Sobrevida , Resultado do Tratamento , Adulto Jovem
17.
Adv Anat Pathol ; 20(3): 137-40, 2013 May.
Artigo em Inglês | MEDLINE | ID: mdl-23574769

RESUMO

In recent years, a new pathologic entity has emerged: indolent T-lymphoblastic proliferation (iT-LBP). iT-LBPs share immunophenotypic similarities with T-lymphoblastic lymphoma; however, T-lymphoblastic proliferations are clinically indolent, and unlike the malignant counterpart, these expansions of nonclonal terminal deoxynucleotidyl transferase (TdT)+ T cells do not require treatment. Here we review the clinical and pathologic features, which are required for an accurate diagnosis of an iT-LBP. We demonstrate specific criteria can be used to accurately diagnose iT-LBP, notably: (1) confluent groups of TdT+ T cells in a biopsy specimen, (2) relative preservation of surrounding normal lymphoid architecture, (3) TdT+ T cells without morphologic atypia, (4) absence of thymic epithelium, (5) nonclonal TdT+ T cells, (6) immunophenotype of developmentally normal immature thymic T cells, and (7) clinical evidence of indolence (follow-up >6 mo without progression).


Assuntos
Linfonodos/patologia , Leucemia-Linfoma Linfoblástico de Células T Precursoras/diagnóstico , Linfócitos T/patologia , Adulto , Idoso , DNA de Neoplasias/análise , Diagnóstico Diferencial , Feminino , Humanos , Hiperplasia , Imunofenotipagem , Masculino , Pessoa de Meia-Idade , Técnicas de Diagnóstico Molecular , Lesões Pré-Cancerosas , Leucemia-Linfoma Linfoblástico de Células T Precursoras/imunologia , Leucemia-Linfoma Linfoblástico de Células T Precursoras/terapia , Prognóstico
18.
Am J Dermatopathol ; 35(2): 270-2, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-22863906

RESUMO

Primary cutaneous gamma delta T-cell lymphoma is a rare diagnosis with only 40 reported cases. We describe a case of cutaneous gamma delta T-cell lymphoma with hemophagocytic syndrome and brain involvement that was not apparent morphologically on skin biopsy and was diagnosed as perifolliculitis and lobular panniculitis. The biopsy was sent later for molecular studies to the University of Washington, which demonstrated a T-cell clone. This case demonstrates that a T-cell clone may be present in a skin biopsy without morphologic or immunophenotypic evidence of lymphoma.


Assuntos
Neoplasias Encefálicas/secundário , Linfo-Histiocitose Hemofagocítica/etiologia , Linfoma Cutâneo de Células T/patologia , Humanos , Linfoma Cutâneo de Células T/imunologia , Masculino , Pessoa de Meia-Idade , Receptores de Antígenos de Linfócitos T gama-delta/metabolismo
19.
Appl Immunohistochem Mol Morphol ; 21(2): 116-31, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22820658

RESUMO

Determining the immunophenotype of hematologic malignancies is now an indispensable part of diagnostic classification, and can help to guide therapy, or to predict clinical outcome. Diagnostic workup should be guided by morphologic findings and evaluate clinically important markers, but ideally should avoid the use of overly broad panels of immunostains that can reveal incidental findings of uncertain significance and give rise to increased costs. Here, we outline our approach to diagnosis of B-cell neoplasms, combining histologic and clinical data with tailored panels of immunophenotyping reagents, in the context of the 2008 World Health Organization classification. We present data from cases seen at our institution from 2004 through 2008 using this approach, to provide a practical reference for findings seen in daily diagnostic practice.


Assuntos
Biomarcadores Tumorais/genética , Imunofenotipagem , Leucemia/diagnóstico , Linfoma de Células B/diagnóstico , Gradação de Tumores/normas , Proteínas de Neoplasias/genética , Antígenos CD/genética , Antígenos CD/imunologia , Linfócitos B/imunologia , Linfócitos B/patologia , Biomarcadores Tumorais/imunologia , Medula Óssea/imunologia , Medula Óssea/patologia , Citometria de Fluxo , Expressão Gênica , Humanos , Imuno-Histoquímica , Leucemia/classificação , Leucemia/imunologia , Leucemia/patologia , Linfoma de Células B/classificação , Linfoma de Células B/imunologia , Linfoma de Células B/patologia , Proteínas de Neoplasias/imunologia , Prognóstico
20.
Am J Surg Pathol ; 36(11): 1619-28, 2012 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-23060347

RESUMO

T-lymphoblastic lymphoma is an aggressive neoplasm requiring prompt clinical treatment. Conversely, indolent T-lymphoblastic proliferation mimics T-lymphoblastic lymphoma but consists of a proliferation of non-neoplastic TdT+ T cells, requiring no treatment. Recently, we identified several cases of indolent T-lymphoblastic proliferations in extrathymic lymphoid tissues: 1 in a patient suffering from Castleman disease (CD) associated with a follicular dendritic cell sarcoma/tumor, 1 in a patient with a history of angioimmunoblastic T-cell lymphoma (AITL), and 1 in association with acinic cell carcinoma. Interestingly, in the case of the patient with a history of AITL, these TdT+ T cells were seen in multiple anatomic sites over the span of 5 years. Here we review these 3 cases and extend our findings by demonstrating that TdT+ T-lymphoblastic populations are increased in lymph nodes of patients with CD (P=0.011), CD in association with follicular dendritic cell tumors, and AITL (P<0.01) compared with other T-cell or B-cell lymphomas or reactive lymph nodes. Finally, analysis of 352 nonhematolymphoid tumors including carcinomas, melanomas, and sarcomas demonstrates that TdT+ T cells are not increased in these tumors. Our studies not only present several detailed cases of indolent T-lymphoblastic proliferations, but also correlate these populations with specific hematologic diseases.


Assuntos
Hiperplasia do Linfonodo Gigante/patologia , DNA Nucleotidilexotransferase/metabolismo , Sarcoma de Células Dendríticas Foliculares/patologia , Células Dendríticas/patologia , Linfadenopatia Imunoblástica/patologia , Linfoma de Células T/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Hiperplasia do Linfonodo Gigante/metabolismo , Proliferação de Células , Sarcoma de Células Dendríticas Foliculares/metabolismo , Células Dendríticas/metabolismo , Feminino , Humanos , Linfadenopatia Imunoblástica/metabolismo , Linfonodos/metabolismo , Linfonodos/patologia , Linfoma de Células T/metabolismo , Masculino , Pessoa de Meia-Idade , Análise Serial de Tecidos , Adulto Jovem
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