Gail Bentley

Ewing sarcoma vs lymphoblastic lymphoma. A comparative immunohistochemical study.

To develop a practical immunohistochemistry panel for distinguishing lymphoblastic lymphoma from Ewing sarcoma (ES), we evaluated 17 ES and 27 lymphoblastic lymphoma and leukemia cases with antibodies to CD99, terminal deoxynucleotidyl transferase (TdT), leukocyte common antigen (LCA), CD43, CD79a, CD20, CD3, vimentin, and neuron-specific enolase (NSE). Three cases were bone lymphomas, 2 initially misdiagnosed as ES. All cases were CD99+. All lymphomas and leukemias were TdT+ compared to none of the ESs. None of the ESs expressed other lymphocytic markers, which were inconsistently expressed in the lymphomas and leukemias: CD43, 33%; LCA, 30%; CD79a, 19%; CD3, 19%; and CD20, 7%. Of the ESs, 88% were vimentin positive compared with 23% of lymphomas and leukemias. Vimentin was stronger and more diffuse in ES. NSE did not reliably stain any cases. When faced with the differential diagnosis of ES vs lymphoblastic lymphoma, an immunohistochemical panel that includes antibodies to CD99 and TdT is useful. Both epitopes are well preserved in fixed and decalcified tissue. A panel composed of antibodies to CD99 and TdT, in conjunction with other lymphocytic markers and vimentin, is highly sensitive and specific.

Disseminated Cytomegalovirus Disease in Hosts without Acquired Immunodeficiency Syndrome and without an Organ Transplant

We describe 7 histologically proven cases of cytomegalovirus disease in patients without human immunodeficiency virus and without organ transplants, all of whom had associated comorbid conditions. Therapy with ganciclovir generally resulted in a favorable outcome.

Lacunar and Reed-Sternberg–Like Cells in Follicular Lymphomas Are Clonally Related to the Centrocytic and Centroblastic Cells as Demonstrated by Laser Capture Microdissection

Two cases of follicular lymphoma (FL) with numerous large cells resembling the lacunar and Hodgkin and Reed-Sternberg (HRS) cells of classic Hodgkin lymphoma were studied to determine clonal relationships between the HRS-like cells and centrocytic and centroblastic (CCCB) cells. In both cases, CCCB cells were typical of FL; CD45RB, CD20, CD10 and BCL-2 positive. In case 1, the HRS-like cells were positive for CD45RB, CD20, CD10, CD30, OCT2, and BOB.1 and negative for CD15 and bcl-2. In case 2, the HRS-like cells were positive for CD30, fascin, CD20, OCT2, and BOB.1 and negative for CD45RB, CD10, CD15, and bcl-2. CCCB and single HRS-like cells were isolated by laser capture microdissection followed by polymerase chain reaction amplification and sequencing of immunoglobulin heavy chain gene rearrangements. In both cases, identical sequences were obtained from CCCB and HRS-like cells. These findings confirm that although the HRS cells and CCCB cells in these cases demonstrate morphologic and immunophenotypic divergence, they share a common cell of origin. These cases further highlight the potential diagnostic pitfall presented by FL with HRS-like cells.

Long term follow‐up of allogeneic stem cell transplantation in patients with myelodysplastic syndromes using busulfan, cytosine arabinoside, and cyclophosphamide

We report here the 10‐year follow‐up of 86 patients who underwent allogeneic stem cell transplantation (ASCT) for myelodysplastic syndrome (MDS). All patients received the busulfan, cytosine arabinoside, and cyclophosphamide (BAC) preparative regimen which consisted of busulfan 16 mg/kg, cytosine arabinoside 8 g/m2 IV, and cyclophosphamide 120 mg/kg IV. Fifty‐nine patients (69%) had de novo MDS; 26 (30%) had secondary MDS (treatment related), and one had a preceding aplastic anemia which progressed to MDS before transplant. Cytogenetics (80 patients) was classified as good (34%), intermediate (17%), or poor (42%). With a median follow‐up for survivors of 124 months, the 10‐year Kaplan‐Meier estimates for overall survival (OS) was 43% (95% confidence interval [CI]: 31–53%). Cumulative nonrelapse mortality (NRM) and relapse was 43% (95% CI: 32–54%) and 19% (95% CI: 11–27%), respectively. No patient relapsed after 2 years. In patients with RAEB‐T/AML, 10‐year relapse‐free survival (RFS), relapse, and NRM was 36%, 36%, and 27%, respectively. Younger age (P = 0.05), human leukocyte antigen (HLA) match (P = 0.002), good risk cytogenetics (P = 0.008), and having a related donor (P = 0.03) significantly improved overall and RFS in the multivariable analysis. The long‐term follow‐up of patients receiving the BAC regimen with ASCT in this study indicated durable relapse‐free and OS with acceptable toxicity in this group of patients with high‐risk features. Am. J. Hematol., 2010.

Retroperitoneal extramedullary anaplastic plasmacytoma masquerading as sarcoma: Report of a case with an unusual presentation and imprint smears.

BACKGROUND Extramedullary plasmacytoma of the retroperitoneum is rare. Furthermore, plasmacytoma with anaplastic features can be confused with high grade sarcoma clinically and histologically, particularly when the initial immunohistochemical tumor markers are negative. However, paying attention to cytologic imprint smears can give valuable clues to the correct diagnosis. CASE A 73-year-old male was admitted to our hospital with a recent history of back pain. Abdominal computed tomography revealed a large retroperitoneal mass (6.8 x 5.1 cm). The initial pathologic evaluation revealed a high grade pleomorphic neoplasm that failed to express multiple epithelial, mesenchymal, lymphoid and melanoma immunohistochemical markers. Subsequent fresh tissue evaluation with touch imprints and immunophenotypic characterization confirmed the plasma cell origin of the tumor. Thorough retrospective review of the touch imprint smears clearly showed the plasmacytic cytologic features. Features of multiple myeloma were essentially absent. CONCLUSION Performing cytologic imprint smears on fresh tissue material may help in making the correct diagnosis and is highly recommended.

Chronic vulvar fissure--a rare manifestation of mycosis fungoides.

Background. Vulvar fissures are a common cause of vulvar pain and discomfort. The differential diagnosis of the underlying process is broad, and some cases remain undiagnosed. Mycosis fungoides, the dominant component of cutaneous T-cell lymphoma, rarely present as fissures. We report a case of a chronic vulvar fissure due to mycosis fungoides. Case. A 55-year-old woman was referred to the vaginitis clinic for evaluation of a chronic vulvar fissure, 6 cm in length, located at the left interlabial sulcus. A detailed history and examination for other skin lesions revealed an erythematous pruritic patch on left breast that had been present for years. Repeat biopsies from both sites showed a dense dermal lymphocytic infiltrate composed predominantly of CD3- and CD4-positive T cell with minimal epidermotropism. A T-gamma polymerase chain reaction analysis demonstrated a clonal T-cell rearrangement. Based on a diagnostic algorithm that combines clinical features, histopathology, and molecular biology, a diagnosis of mycosis fungoides was confirmed. Conclusions. Patients presenting with vulvar lesions should always be suspected of having an underlying dermatosis, and a detailed examination for other skin lesions should be performed. In the presented case, once both skin lesions were linked clinically, repeat biopsies of both sites led to a confirmed diagnosis of mycosis fungoides.

Positive pregnancy tests in a postmenopausal woman due to beta-human chorionic gonadotropin production by multiple myeloma.

Extensive immunochemical characterization has shown that the free beta subunit of hCG (betahCG) can be produced by common epithelial tumors, including lung, colon, and bladder. However, the expression of beta-human chorionic gonadotropin (hCG) in hematologic malignancies and its significance is largely unknown. We present an extremely rare case of positive serum betahCG in a postmenopausal woman with relapsed, refractory multiple myeloma with myeloma cells expressing betahCG on immunohistochemical stain.

Adult T-Cell Leukemia/Lymphoma with CLL-Like Morphology—A Case Report

Adult T-cell Leukemia/Lymphoma (ATL) is rarely seen in the U.S. and Europe, usually limited to African Americans from the southeastern U.S. and immigrants from HTLV-1 endemic areas. Reaching an accurate and timely diagnosis of ATL in such nonendemic areas can be challenging, owing to limited exposure, diverse manifestations, and varying cell morphology. We present a case of chronic adult T-cell leukemia (ATL) with Chronic Lymphocytic Leukemia- (CLL-) like morphology that remained untreated for ten years and then developed treatment refractory acute ATL crisis.