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Judith A Ferry

Publications and source records attributed to Judith A Ferry.

12 recordsLinked to original sources

Burkitt's lymphoma: clinicopathologic features and differential diagnosis.

Burkitt's lymphoma is a highly aggressive lymphoma identified and described in the last century by Denis Burkitt in Africa, in areas endemic for malaria. Since its description in African children, it has been recognized outside areas with endemic malaria, frequently also in children as well as among individuals with an underlying immunodeficiency. Since its initial designation as Burkitt's lymphoma, this type of lymphoma and lymphomas closely resembling it have received a variety of names in different classifications of lymphomas and leukemias: undifferentiated lymphoma, Burkitt's and non-Burkitt's type in the modified Rappaport Classification, malignant lymphoma, small non-cleaved cell, Burkitt's type in the Working Formulation, Burkitt's lymphoma and high-grade B-cell lymphoma, Burkitt-like in the REAL Classification, and acute lymphoblastic leukemia, L3 type in the FAB Classification. With the publication of the WHO Classification of Haematopoietic and Lymphoid Tumors, the nomenclature of this lymphoma has come full circle, and it is once again known simply as Burkitt's lymphoma. In recent years, efforts have focused on improving therapy for this rapidly proliferating neoplasm while minimizing, to the extent possible, treatment-associated toxicity. These efforts have led to the development of high-intensity, short-duration combination chemotherapy that has proven extremely effective for a high proportion of Burkitt's lymphoma patients. The differential diagnosis of Burkitt's lymphoma is broad, and precise diagnosis based on histologic, immunophenotypic, and genetic features remains the critical first step in planning appropriate therapy.

Burkitt Lymphoma↗

ELF4 is fused to ERG in a case of acute myeloid leukemia with a t(X;21)(q25-26;q22).

We report a novel chromosomal translocation in AML, t(X;21)(q25-26;q22), resulting in a fusion transcript between two ETS domain family members, ELF4 (at Xq25) and ERG (at 21q22). ERG has been associated previously with other fusion partners, specifically FUS and EWSR1, and implicated in both AML and Ewing's sarcoma. RT-PCR analysis of RNA isolated from bone marrow samples from the patient demonstrates that the translocation occurs within intron 1 of ERG isoform 1 (ERG-1) and intron 2 of ELF4 resulting in an in-frame fusion joining exon 2 from ELF4 with exon 2 of ERG. This is the first reported case of an ELF4-ERG fusion and identification of the specific ERG exon involved in the fusion that differentiates ERG isoforms. In addition, this case also directly implicates a new role for ELF4 in cancer.

Acute Disease↗

One patient, two lymphomas. Simultaneous primary gastric marginal zone lymphoma and primary duodenal follicular lymphoma.

We report the case of a 73-year-old man who presented with a 2- to 3-month history of epigastric discomfort and guaiac-positive stool. An upper gastrointestinal endoscopy revealed a diffuse erythematous nodular mucosa and submucosal thickening in the stomach. Diffuse mucosal nodularity was also found in the second portion of the duodenum. A complete workup with histologic, immunohistochemical, and molecular studies revealed 2 distinct, apparently unrelated lymphomas, namely, a gastric marginal zone B-cell lymphoma (mucosa-associated lymphoid tissue type) in a background of Helicobacter pylori gastritis and a grade 1/3 duodenal follicular lymphoma. The patient was then treated with an H pylori eradication regimen. No therapy was given for his duodenal follicular lymphoma because his symptoms were thought to be due to the gastric disease and because the duodenal lesion was small. A 6-month follow-up with upper gastrointestinal endoscopy revealed only focal biopsy scarring in the stomach and an apparently normal duodenum. The follow-up biopsies revealed significant regression of his mucosa-associated lymphoid tissue lymphoma, but persistence of his duodenal follicular lymphoma. The combination of these 2 lymphomas in the same patient and the different clinical responses to antibiotic treatment make this case unique.

Aged↗

Ocular adnexal lymphoma: clinical behavior of distinct World Health Organization classification subtypes.

PURPOSE: To evaluate the clinical behavior and treatment outcome of ocular adnexal lymphomas classified by the World Health Organization system, with emphasis on marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). MATERIALS AND METHODS: The clinicopathologic materials from 98 consecutive patients treated for ocular adnexal lymphoma were reviewed. Fourteen patients had prior lymphoma and 84 patients had primary disease (75% Stage I, 6% Stage III, and 19% Stage IV). Radiation (photons/electrons) was administered to 102 eyes to a median dose of 30.6 Gy. The mean follow-up was 82 months. RESULTS: The most common subtypes among the primary patients were MALT (57%) and follicular (18%) lymphoma. The 5-year actuarial local control rate in 102 irradiated eyes was 98%. Among the low-grade lymphomas, the 5-year local control rate correlated with the radiation dose in the MALT lymphoma subgroup (n = 53): 81% for <30 Gy and 100% for > or =30 Gy (p <0.01). For the non-MALT low-grade lymphomas such as follicular lymphoma (n = 30), the local control rate was 100% regardless of dose. For 39 Stage I MALT lymphoma patients treated with radiation alone, the distant relapse-free survival rate was 75% at 5 years and 45% at 10 years. Distant relapses were generally isolated and successfully salvaged by local therapy. The overall survival for this subgroup was 81% at 10 years, with no deaths from lymphoma. CONCLUSIONS: Dose-response data suggest that the optimal radiation dose for MALT lymphoma of the ocular adnexa is 30.6-32.4 Gy in 1.8-Gy fractions and follicular lymphoma is adequately controlled with doses in the mid-20 Gy range. The substantial risk of distant relapse in Stage I ocular adnexal MALT lymphoma underscores the importance of long-term follow-up for this disease and the need for additional comparative studies of MALT lymphoma of different anatomic sites.

Adolescent↗

Hodgkin and non-Hodgkin lymphoma of the head and neck: clinical, pathologic, and imaging evaluation.

Lymphomas are subdivided into HL and NHL and are more specifically classified into subtypes of HL or NHL according to the WHO classification. HLs involve the lymph nodes predominantly and only approximately 5% arise in extranodal sites, whereas 30% of NHLs present in extranodal sites. Imaging studies, including CT and MR imaging, cannot distinguish [figure: see text] HL from NHL, and cannot differentiate their various subtypes, necessitating a pathologic diagnosis. Clinical parameters, however, can be helpful in differentiating the two broad categories of lymphomas, and subtypes of lymphomas have predilections for different sites within the head and neck. HL is most commonly located in the lymph nodes of the neck and mediastinum. Marginal-zone lymphoma has an affinity for the ocular adnexa, salivary glands, larynx, and the thyroid gland. Diffuse large B-cell lymphoma is commonly encountered in the paranasal sinuses, mandible, maxilla, and Waldeyer ring. Burkitt lymphoma occurs more frequently in children and young adults and frequently affects the maxilla and mandible, with a greater distribution of involvement at a lower frequency. On imaging studies, the lymph nodes of HL and NHL are homogeneous and variable in size, with an average diameter from 2 to 10 cm. They may enhance slightly to moderately, display necrosis before and after treatment, and display calcification post-treatment. NHL in extranodal sites in the head and neck (nasopharynx, Waldeyer ring, oral cavity, and larynx) manifests frequently as a submucosal mass accompanied [figure: see text] by polypoid, bulky masses with a smooth mucosal surface. Clinically aggressive lymphomas, such as Burkitt lymphoma, diffuse large B-cell lymphoma, and NK-/T-cell lymphomas are characterized by destruction of the maxilla, mandible, and bones around the paranasal sinuses, which is indistinguishable from bony destruction in other malignant tumors, such as SCC. Contrast CT is indicated for evaluation of cervical lymph nodes; the chest, including the mediastinum; the pelvic cavity; paranasal sinuses; and orbits. CT is also useful for detection of bone destruction involving the base of the skull, paranasal sinuses, and the mandible or maxilla. MR imaging is preferred for the assessment of extension of lymphomas to different fascial spaces (parapharyngeal, masticator, infratemporal fossa, tongue, and nasopharynx) and for intracranial extension. Lymphomas are isodense to muscle on CT and circumscribed with distinct margins that occasionally display extranodal extension with less-well-defined margins and areas of necrosis within the tumor matrix. Lymphomas appear low in signal intensity on T1-weighted images and low to high in signal intensity on T2-weighted images, with variable, but usually low, enhancement following introduction of Gadolinium-DTPA (Gd-DTPA) contrast material.

Head and Neck Neoplasms↗

Posttransplant lymphoproliferative disorder associated with an Epstein-Barr-related virus in cynomolgus monkeys.

BACKGROUND: Human posttransplant lymphoproliferative disorder (PTLD) has been shown to be associated with Epstein-Barr virus (EBV) infection. Primate animal models of PTLD and the use of molecular markers in its diagnosis have not been reported. This study was designed to evaluate the frequency, pathology, and molecular characteristics of PTLD in cynomolgus kidney allograft recipients. METHODS: Over a 5-year period (January 1995 to November 2000), 160 primate renal transplants were performed at the Massachusetts General Hospital (MGH). Of these, all cases (n=9) that developed PTLD were included. H&E stained paraffin sections of all available tissue samples from the cases were evaluated for the presence of PTLD. Immunoperoxidase staining for T cells (CD3), B cells (CD20), kappa and lambda light chains as well as EBV nuclear antigens (EBNA2) and latent membrane proteins (EBV LMP-1) was done on paraffin sections using standard immunohistochemical (IHC) methods. In situ hybridization for EBV encoded RNA (EBER) was performed in all tissue samples with atypical lymphoid proliferations, using a novel EBER nucleotide probe based on consensus gene sequences from EBV and the related herpes lymphocryptoviruses (LCV) infecting baboons and rhesus macaques. RESULTS: Of 160 consecutive primate renal transplants performed at MGH, 5.6% developed PTLD 28-103 days after transplantation. In all cases, the lymph nodes were involved and effaced by an atypical polymorphous lymphoid proliferation of EBER+ B cells, diagnostic for PTLD. Focal staining for EBNA-2 was noted in tumor cells. In 67% (six of nine) the PTLD infiltrates were present in extra nodal sites, notably liver (56%), lung (44%), heart (44%), renal allograft (44%), and native kidney (22%). The spleen was involved by PTLD in all four animals that had not undergone a pretransplant splenectomy. The PTLD morphology was similar in all cases and predominantly of the polymorphous type, however, some of these showed areas that appeared minimally polymorphous. No cases of monomorphic PTLD were seen. CONCLUSIONS: By in situ hybridization, expression of the RNA product, homologous for EBV-encoded RNA (EBER) was identified in the PTLD tumor cells of all cases, indicating latent primate EBV- related infection. This report identifies a novel animal model of EBV associated PTLD in the setting of kidney transplantation, with valuable implications for managing and understanding human PTLD and oncogenesis.

Animals↗

Epstein-Barr virus-negative precursor B cell lymphoblastic lymphoma after liver transplantation: a unique form of posttransplant lymphoproliferative disease.

BACKGROUND: We report a liver transplant patient with Epstein-Barr virus-negative, precursor B cell lymphoblastic lymphoma diagnosed 6 months after transplantation. PATIENT: The patient was a 13-year-old boy with acute, fulminant hepatic failure of unknown etiology who underwent cadaveric liver transplantation. RESULTS: Six months after the transplant, the patient developed non-tender cervical lymphadenopathy 2 days after a reduction in prednisone dosage. The adenopathy worsened despite withdrawal of immunosuppression, and a biopsy showed precursor B cell lymphoblastic lymphoma. All investigations for Epstein-Barr virus were negative. The patient responded well to chemotherapy and is currently in complete remission 24 months after diagnosis. CONCLUSIONS: Precursor B cell lymphoblastic lymphoma has not been previously reported as a form of posttransplant lymphoproliferative disease. We discuss this unique form of posttransplant lymphoproliferative disease and briefly review the clinical and pathological spectrum of posttransplant lymphoproliferative disease.

Adolescent↗

Angioimmunoblastic T-cell lymphoma.

Angioimmunoblastic T-cell lymphoma (AIL-TCL) is a rare subtype of lymphoma, making up only 1% to 2% of nonHodgkin's lymphomas; however, it accounts for a major subset of peripheral T-cell lymphomas. Angioimmunoblastic T-cell lymphoma has clinical and pathologic features that set it apart from other B- and T-cell lymphomas. More recent studies have delineated the immunophenotypic and genetic features of this unusual lymphoma, and have tentatively identified the cell of origin of this neoplasm.

Adult↗