Cutaneous and systemic plasmacytosis.
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Aged↗
Biomedical subjects
Filiz Sen
Publications and source records attributed to Filiz Sen.
Epistaxis and severe weakness in a patient with multiple myeloma. Therapy-related acute myeloid leukemia, pure erythroid leukemia.
Therapy-related acute myeloid leukemias arise as a result of cytotoxic chemotherapy and/or radiation therapy. The most common types of acute myeloid leukemia arising in this setting are acute myeloid leukemia with maturation, and lesser numbers of acute myelomonocytic leukemia, acute monocytic leukemia, acute erythroleukemia, or acute megakaryocytic leukemia. We present a patient with multiple myeloma who was treated with melphalan and 4 years later developed acute erythroid leukemia. The morphologic diagnosis of pure erythroid leukemia developing in the setting of multiple myeloma may be challenging.
Acute Disease↗
Primary hepatic anaplastic large-cell lymphoma diagnosed by fine-needle aspiration biopsy.
Anaplastic large-cell lymphoma (ALCL) presenting as primary hepatic lymphoma is exceedingly rare. Here, we report a case of primary hepatic ALCL, which was diagnosed by cytologic evaluation and concurrent immunohistochemical studies on the material obtained by fine-needle aspiration biopsy. The aspirate smears revealed many loosely dispersed, large atypical cells, some of them with kidney-/horseshoe-shaped and doughnut-shaped nuclei and abundant amphophilic cytoplasm. The nuclei in some of the other cells were often eccentrically located and appeared to be convoluted and even multilobulated. These large atypical cells displayed T-cell immunophenotype and were immunoreactive with CD30 antibody. The diagnosis was confirmed by a core-needle biopsy. This is the first case of primary hepatic ALCL reported in the English cytological literature.
Adult↗
Tenascin and microvessel stromal changes in patients with non-Hodgkin's lymphoma are isolated to the sites of disease and vary in correlation to disease activity.
This study investigated stromal changes in expression of tenascin and vasculogenesis in lymphoma. Documenting the dynamic nature of the stromal changes in lymphoma in relation to response to therapy is helpful in planning new therapies directed at these targets. Two hundred and sixty one samples from 111 patients with varying types of non-Hodgkin's lymphoma were reviewed and examined using immunohistochemistry techniques. Samples were stained for factor VIII - related antigen for microvessel density (MVD) analysis and anti-tenascin antibody for qualitative assessment of the stromal expression. Multiple samples from the same patient were taken at the same point in time to assess whether stromal changes were limited to sites of disease. Multiple samples were examined over the course of a patient's illness to assess whether the stromal changes were modulated according to disease activity. There was a significant increase in tenascin expression and MVD in the sites of disease compared with uninvolved sites (p = 0.01 and p < 0.0001, respectively). In patients who responded to therapy, there was a decrease in the expression of tenascin (p = 0.0049) and MVD (p < 0.0001), and in those with disease progression there was an increase in the tenascin expression (p = 0.0050) and MVD (p < 0.0001). Our results suggest stromal changes are isolated to the sites of disease within patients, allowing targeted therapies to be developed. Further, stromal changes correlate with disease response over the course of the patient's disease. This new finding may have implications for the timing of anti-stromally directed therapies.
Blood Vessels↗
Calcifying fibrous pseudotumor of the neck: diagnostic challenges of a rare benign lesion.
Neck masses, frequently encountered by physicians, comprise a vast range of diagnoses, with malignancy being the greatest concern. Calcifying fibrous pseudotumor (CFP) is a rare lesion with unknown pathogenesis, characterized pathologically by a predominance of abundant hyalinized collagenous tissue with focal lymphoplasmacytic infiltrate and psammomatous or dystrophic calcifications. We present the case of a 29-year-old woman who presented with a 4-cm left neck mass, accompanied by constitutional symptoms of vague weakness and lethargy. After the lesion failed to respond to a course of antibiotic therapy, fine-needle aspiration was performed, the pathology of which was indeterminate. The concern was that the lesion was a lymphoproliferative disorder-further workup was performed. CT of the chest, abdomen, and pelvis revealed no evidence of adenopathy or neoplasms. Subsequently, an incisional biopsy was performed, suggesting a diagnosis of CFP. Magnetic resonance imaging with contrast, performed to delineate the anatomy, revealed the lesion in the left neck, deep to the left clavicle, that extended superiorly into the supraclavicular fossa. Complete surgical removal of the lesion was successfully performed, with immunophenotyping confirming the initial diagnosis of CFP. We present a case report of cervical CFP, discuss the approach to neck masses, and review the recent literature on this rare, benign entity.
Adult↗
Cytogenetic findings in blastoid mantle cell lymphoma.
A subset of mantle cell lymphoma (MCL) tumors has blastoid morphology, and a number of morphologic variants of blastoid MCL have been described in the literature. In this report, we document the cytogenetic findings in 27 cases of blastoid MCL. Conventional cytogenetic analyses were performed on bone marrow aspirates involved by MCL from 27 patients. There were 14 men and 13 women with a median age of 63 years (range, 40-79 years). Diagnostic tissue biopsy and bone marrow specimens were reviewed, and cases were divided into 2 morphologic groups: classic (12 cases) and pleomorphic (15 cases), as defined in the World Health Organization classification. All tumors had an immunophenotype compatible with MCL, were positive for cyclin D1, and carried the t(11;14). Twenty-four cases had complex karyotypes with 3 or more chromosomal abnormalities in addition to the t(11;14). In classic blastoid MCL, abnormalities of chromosomes 13, 18, and 8 were most common. In pleomorphic blastoid MCL, abnormalities of chromosomes 13, 17, and 3 were most frequent. Chromosome 22 abnormalities were detected exclusively in the pleomorphic group. Tumors in which the neoplastic cells showed prominent nucleoli had a significantly higher frequency of chromosome 17 abnormalities (P = 0.03). We conclude that blastoid MCL tumors often show complex cytogenetic aberrations. Some abnormalities correlate with morphologic features, suggesting that morphologic variants of blastoid MCL may arise via different molecular pathways.
Adult↗
Apoptosis and proliferation in subcutaneous panniculitis-like T-cell lymphoma.
Subcutaneous panniculitis-like T cell lymphoma (SPTCL), designated recently as a distinct clinicopathologic entity in the World Health Organization Classification, is a neoplasm composed of cytotoxic T-cells that preferentially involves subcutaneous adipose tissue. Histologically, SPTCL is characterized by extensive karyorrhectic debris and tumor necrosis suggesting that apoptotic mechanisms are involved in its pathogenesis. We assessed the apoptotic index (AI) and proliferation rate (PR) of 13 cases of SPTCL by TUNEL test and Ki-67 immunostaining, respectively. We also immunohistochemically assessed for expression of BCL-2 (anti-apoptosis), BAX (pro-apoptosis), and P53 and correlated the results with apoptosis and proliferation. We detected a high AI (median 8.1%) in 11 cases of SPTCL, and 12 cases had low BCL-2 and high BAX expression. BCL-2 expression inversely correlated with AI (P <.001) and BAX (P <.001). We found a low PR (cutoff > or = 25%) in eight (61%) cases. There was an inverse correlation between AI and PR (r = -.58, P =.04). Ten cases were assessed for P53; immunostaining results were heterogeneous but P53 expression correlated with large cell cytologic features. Our findings demonstrate that SPTCLs have a high AI that may be explained by differential expression of BCL-2 and BAX in the neoplastic cells.
Adult↗
Mantle cell lymphoma involving skin: cutaneous lesions may be the first manifestation of disease and tumors often have blastoid cytologic features.
We describe five cases of mantle cell lymphoma involving skin. Three patients initially presented with skin lesions but had evidence of widespread disease at time of diagnosis or with relatively short follow-up. One patient was known to have disseminated disease before he developed skin lesions. One patient presented with a solitary skin nodule on the thigh and has developed multiple smaller nodules on the same leg, but no other sites of disease over 30 months of clinical follow-up. This case fulfills the criteria for primary cutaneous lymphoma as proposed by the European Organization for Research and Treatment of Cancer. Biopsy of the skin lesions in all cases showed predominantly dermal and focally subcutaneous lymphoid infiltrates, preferentially perivascular and periadnexal in four cases, and nodular in one case. The tumors were composed of small- to medium-sized lymphocytes with irregular nuclear contours. Four cases had blastoid and one case had typical cytologic features. Immunophenotypic studies showed that all cases were positive for CD20 and cyclin D1, and four of five were positive for CD5. Four cases, including the CD5-negative case, had evidence of the t(11;14) shown by either fluorescence in situ hybridization methods performed on skin tumors or conventional cytogenetic analysis performed on involved bone marrow. We conclude that mantle cell lymphoma can involve skin, usually as a manifestation of disseminated disease, and is often associated with blastoid cytologic features. Rare cases of mantle cell lymphoma may arise in skin.
Aged↗
Apoptotic rate in peripheral T-cell lymphomas. A study using a tissue microarray with validation on full tissue sections.
Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of non-Hodgkin lymphomas with a wide spectrum of clinicopathologic features, and apoptosis mechanisms may have a role in lymphomagenesis. We assessed apoptotic rate (AR) in 112 PTCLs using a tissue microarray developed in our laboratory and a modified terminal deoxynucleotidyl transferase-mediated dUTP nick-end labeling assay. The mean AR was 1.47% +/- 1.38% for the entire group of PTCLs (range, 0.06%-5.15%), and AR varied significantly among different tumor types. In mycosis fungoides, the mean AR was 0.74%; angioimmunoblastic T-cell lymphoma, 1.02%; PTCL, not otherwise specified, 1.38%; cutaneous anaplastic large cell lymphoma (ALCL), 1.41%; anaplastic lymphoma kinase protein (ALK)-negative ALCL, 1.43%; extranodal natural killer/T-cell lymphoma of nasal type, 2.04%; ALK-positive ALCL, 2.95%; and enteropathy-type T-cell lymphoma, 3.06%. Mean AR was higher in PTCL with large cell vs small/medium cell morphologic features (1.66% +/- 1.1% vs 0.99% +/- 1.0%). In a subset of 33 PTCLs, the tissue microarray results comparedfavorably with those obtained in full tissue sections. We conclude that the highest ARs in PTCLs are found in enteropathy-type T-cell lymphoma and ALK-positive ALCL, and that AR can be assessed reliably by using a tissue microarray.
Anaplastic Lymphoma Kinase↗
Multiple myeloma in association with sarcoidosis.
The association of sarcoidosis with Hodgkin disease and non-Hodgkin lymphoma is well known. However, multiple myeloma also can occur rarely in association with sarcoidosis. We describe a patient with sarcoidosis who subsequently developed multiple myeloma. The patient was a 49-year-old woman with a 4-year history of severe, chronic, active sarcoidosis involving her lungs, lymph nodes, eyes, and bone marrow. During the initial clinical workup, a serum monoclonal paraprotein was detected and bone marrow examination revealed a slight increase in plasma cells (4%), in addition to noncaseating granulomas. Thus, the diagnoses of monoclonal gammopathy of undetermined significance and sarcoidosis were established simultaneously. She sought medical attention for her current illness when she developed low back pain and weakness of her lower extremities. Serum protein electrophoresis and immunofixation revealed a monoclonal paraprotein, immunoglobulin (Ig) G kappa type, and quantification revealed an IgG level of 46.67 g/L (normal, 5.88--15.73 g/L). Bone marrow aspiration and biopsy revealed multiple myeloma and sarcoidosis. Including this patient, 11 cases of sarcoidosis and multiple myeloma have been reported to date, including 3 patients with monoclonal gammopathy of undetermined significance preceding the onset of multiple myeloma. In this case, as in most of the cases reported previously, sarcoidosis preceded the development of multiple myeloma.
Antineoplastic Agents, Alkylating↗
Chronic lymphocytic leukemia with t(14;18) and trisomy 12.
The chromosomal abnormality t(14;18) is most commonly associated with neoplasms of follicular center cell origin. However, t(14;18) also has been reported in rare cases of chronic lymphocytic leukemia (CLL). In 2215 cases of CLL studied by conventional cytogenetics in our institution, we identified 2 cases of CLL carrying t(14;18). Both patients were men, aged 52 and 71 years at the time of diagnosis. One patient presented with leukemia and the other primarily with nodal disease. In addition to t(14;18), trisomy 12 (+12) was identified in the same clone in both cases. Atypical morphologic features were identified: case 1 contained more than 15% lymphoid cells with cleaved nuclei, whereas case 2 contained more than 15% plasmacytoid lymphoid cells. The immunophenotype of case 2 was also unusual for CLL, showing weak CD23 expression and FMC7 positivity. We identified 6 other t(14;18)-carrying CLL cases in the literature; 2 had t(14;18) as the sole abnormality and 2 contained +12 as the additional abnormality. To conclude, cases of CLL carrying t(14;18) are exceedingly rare, and +12 appears to be the most common cytogenetic abnormality coexisting with t(14;18) in CLL.
Aged↗
Molecular genetic methods in the diagnosis of hematologic neoplasms.
Leukemias and lymphomas are monoclonal neoplasms that arise as a result of molecular abnormalities. These abnormalites are diverse but can be grouped into two general categories, chromosomal translocations that usually result in oncogene activation and inactivation of tumor suppressor genes. Recent advances in our understanding of chromosomal translocations have led to improved classification of leukemias and lymphomas. For example, the t(9;22)(q34;q11) is now considered a defining feature of chronic myeloid leukemia, and the t(2;5)(p23;q35) defines a clinically and biologically unique subset of anaplastic large cell lymphomas. In this review, we focus on chromosomal translocations in hematologic neoplasms and the techniques used for their detection. We also briefly discuss tumor suppressor genes and assessment of clonality in lymphoid neoplasms.
Blotting, Southern↗