Criteria for diagnosis of acute megakaryocytic leukemia.
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Bone Marrow↗
Biomedical subjects
J A Strauchen
Publications and source records attributed to J A Strauchen.
Concurrent chronic lymphocytic leukemia cutis and acute myelogenous leukemia cutis in a patient with untreated CLL.
Patients who have chronic lymphocytic leukemia (CLL) are known to have a high frequency of second malignant neoplasms. However, acute myelogenous leukemia (AML) occurring concurrent with or after a diagnosis of CLL is extremely rare. In this article we report a case of AML developing in a 55-year-old male with a 6-year history of untreated CLL. The diagnosis was facilitated by touch preparation of a skin punch biopsy specimen. The patient presented with a two-week history of fever, weakness, anasarca, and a skin rash. Physical examination revealed pink to skin-colored firm papules, which coalesced into indurated plaques on his trunk, upper extremities, and face. The lesions, in combination with generalized edema, produced a leonine facies. Touch prep of the biopsy showed medium to large blasts, large monocytoid cells, and numerous small mature lymphocytes, providing the preliminary diagnosis of a second, previously undiagnosed myelomonocytic malignancy in this patient. The initial diagnosis was subsequently confirmed by histologic, cytochemical, immunohistochemical and flow cytometry studies. This is the first reported case of CLL with concurrent AML in which rapid touch prep of a skin punch biopsy facilitated diagnosis.
Humans↗
Indolent T-lymphoblastic proliferation: report of a case with an 11-year history and association with myasthenia gravis.
T-lymphoblastic lymphoma is a high-grade malignant lymphoma with frequent occurrence in young males, mediastinal involvement, and systemic dissemination. Indolent T-lymphoblastic proliferations have rarely been recognized. In the present case, we report on an indolent T-lymphoblastic proliferation involving the oropharynx in a patient with myasthenia gravis with multiple local recurrences over an 11-year period without evidence of systemic dissemination. The T-lymphoblasts were consistently positive for terminal deoxynucleotidyl transferase (TdT), CD1, CD3, CD4, and CD8, corresponding to an intermediate thymocyte stage of differentiation. No cytokeratin-positive thymic epithelial cells were identified, ruling out an ectopic thymus or thymoma. T-receptor gene rearrangement studies by Southern blot revealed no monoclonal CT-beta rearrangement. Indolent T-lymphoblastic proliferations of undetermined clonality may rarely occur; predilection for involvement of oropharynx and possible association with myasthenia gravis are suggested.
Adult↗
Terminal deoxynucleotidyl transferase-positive cells in human tonsils.
To study the possible cellular origin of recently recognized indolent terminal deoxynucleotidyl transferase (TdT)-positive T-lymphoblastic proliferations of the tonsils and oropharynx, we studied normal human tonsils for the presence of TdT-positive cells. TdT-positive cells were readily demonstrated in the tonsils from 15 children and adults by immunohistochemical staining. TdT-positive cells were distributed in discrete foci at the periphery of lobules of lymphoid tissue and adjacent to fibrous septa and had the morphologic features of small to medium-sized lymphocytes. Double-antibody staining indicated the TdT-positive cells had the phenotype of uncommitted early lymphoid precursors (CD3-, CD79a-, CD10-). Foci of TdT-positive cells were not identified in 6 reactive lymph nodes studied as controls. These studies indicate that tonsils, like bone marrow and thymus, are sites of lymphopoiesis. The presence of TdT-positive precursor cells in human tonsils may be a factor in the pathogenesis of recently described indolent T-lymphoblastic proliferations involving the tonsils and oropharynx. The presence of TdT-positive cells in human tonsils should not be misinterpreted as evidence of lymphoblastic lymphoma or leukemia.
Adolescent↗
Renal cell carcinoma with intravascular lymphomatosis: a case report of unusual collision tumors with review of the literature.
We report the case of a 77-year-old white woman who presented with a left breast mass, lethargy, and weight loss. Pelvic computed tomographic scan revealed a 9.5-cm mass in the right kidney. Surgical pathology demonstrated a diffuse large B-cell lymphoma of the subcutaneous tissue of the breast and renal cell carcinoma with concurrent extensive intravascular lymphomatosis. Systemic dissemination of malignant lymphoma to a concurrent visceral primary neoplasm is rare. To the best of our knowledge, this is the first case illustrating a renal cell carcinoma collision with intravascular lymphomatosis.
Aged↗
Further "teleconsultation" called for.
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Adenocarcinoma↗
Posttransplantation plasmacytic proliferations related to Kaposi's sarcoma-associated herpesvirus.
Kaposi's sarcoma-associated herpesvirus (KSHV), which was originally detected in Kaposi's sarcoma, also has been found in primary effusion lymphomas (PELs) and some cases of multicentric Castleman's disease. We describe two transplant recipients who developed Kaposi's sarcoma and a spectrum of non-neoplastic lymphoproliferative disorders that show pronounced plasmacytic and plasmacytoid features. The first patient had recurrent pleural effusions and Castleman's disease-like changes in lymph nodes. The second patient had systemic lymphadenopathy and hepatosplenomegaly secondary to diffuse infiltration by polyclonal plasma cells and plasmacytoid B lymphocytes that clinically mimicked Epstein-Barr virus (EBV)-associated posttransplant lymphoproliferative disease. In both cases, KSHV DNA was detected by polymerase chain reaction and Southern blotting, and KSHV vIL-6 protein expression was identified in affected tissues by immunohistochemical localization. In contrast, no evidence of KSHV coinfection was detected in any of 31 EBV-related posttransplant lymphoproliferative disorders or 112 non-PEL lymphomas tested. The pathologic findings in these two patients were not representative of malignancy by morphologic, immunophenotypic, or molecular criteria. This study underscores the marked propensity for hematolymphoid proliferations associated with KSHV infections to show plasmacytic features. Additionally, this study describes use of an antibody reactive against KSHV vIL-6 that can readily detect a subpopulation of KSHV-infected hematopoietic cells.
Adult↗
Foucar K: chronic lymphoid leukemias and lymphoproliferative disorders. Mod Pathol 12:141, 1999.
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Chronic Disease↗
Establishing a KSHV+ cell line (BCP-1) from peripheral blood and characterizing its growth in Nod/SCID mice.
Kaposi's sarcoma-associated herpesvirus (KSHV or HHV8) sequences are present in primary effusion lymphomas (PEL). KSHV+ cell lines have been established from such lymphomas. Here we report the first description of the establishment of a KSHV+, EBV- cell line (BCP-1) from the peripheral blood of a patient with PEL. Using this cell line and a KSHV+, EBV+ PEL cell line (HBL-6) previously established from ascitic fluid, we investigated whether in nonobese diabetic/severe combined immunodeficiency disease (Nod/SCID) mice tumors representing PEL can be established. When injected intravenously (IV) into Nod/SCID mice, BCP-1 and HBL-6 infiltrated organs, with only occasional macroscopic tumor formation. Intraperitoneal injections (ip) led to the development of ascites and diffuse infiltration of organs, without obviously solid lymphoma formation, resembling the diffuse nature of human PEL. To investigate a possible mechanism for the peculiar phenotype of PEL, we examine the presence of adhesion molecules and homing markers on PEL cells before and after growing in mice. Both BCP-1 and HBL-6 cells lack expression of important cytoadhesion molecules including CD11a and CD18 (LFA1 alpha and beta chains), CD29, CD31, CD44, CD54 (ICAM-1), and CD62L and E (L and E selectins).
Animals↗
Body cavity-based malignant lymphoma containing Kaposi sarcoma-associated herpesvirus in an HIV-negative man with previous Kaposi sarcoma.
BACKGROUND: The role of Kaposi sarcoma-associated herpesvirus in the development of malignant lymphomas in patients negative for the human immunodeficiency virus (HIV) has not been established. OBJECTIVE: To examine the possible role of Kaposi sarcoma-associated herpesvirus in a case of body cavity-based malignant lymphoma that occurred in an HIV-negative patient who had previously had Kaposi sarcoma. DESIGN: Case study. SETTING: Academic medical center. PATIENT: A 94-year-old man with lymphomatous ascites. MEASUREMENTS: Polymerase chain reaction (PCR) and Southern blot DNA analysis. RESULTS: The body cavity-based lymphoma cells were positive for Kaposi sarcoma-associated herpesvirus by PCR and were negative for other herpesviruses, including Epstein-Barr virus, cytomegalovirus, and human herpesviruses 6 and 7. Southern blot analysis of lymphoma DNA showed high levels of Kaposi sarcoma-associated herpesvirus (> 40 to 80 genomes/cell). Clonal rearrangement of the immunoglobulin JH and JK genes was present, confirming the presence of a clonal B-cell proliferation. CONCLUSIONS: Kaposi sarcoma-associated herpesvirus may be involved in the development of malignant lymphoma after Kaposi sarcoma in HIV-negative patients. This type of lymphoma, in contrast to body cavity-based lymphoma related to the acquired immunodeficiency syndrome, may have an indolent clinical course.
Aged↗
Epstein-Barr virus and primary lung lymphoma: a study utilizing the polymerase chain reaction.
Seven cases of primary lung lymphoma were analyzed for the presence of Epstein-Barr virus DNA sequences by the polymerase chain reaction. The series included: four cases of diffuse, small lymphocytic lymphoma; one case of diffuse, intermediate lymphocytic lymphoma; one case of diffuse, small cleaved cell lymphoma; and one case of large cell, immunoblastic lymphoma. The latter occurred in a patient with acquired immunodeficiency syndrome. A 200-bp sequence of the Epstein-Barr nuclear antigen 1 gene was used as a template for PCR amplification. The only tumor that contained Epstein-Barr virus sequences was the immunoblastic lymphoma. These findings support previous observations that small lymphocytic lymphomas of the lung are not related to Epstein-Barr virus infection. In contrast, some large cell lymphomas may represent Epstein-Barr-virus--associated lymphoproliferative disorders.
Adult↗
Lymphoma in inflammatory bowel disease.
Nine patients with lymphoma occurring in association with inflammatory bowel disease were admitted to The Mount Sinai Hospital between 1960 and 1983. Five (two men and three women) occurred among 1156 patients (0.43%) with ulcerative colitis (UC) and four (men), among 1480 patients (0.27%) with Crohn's disease (CD), a strong male preponderance in the latter group. In all four of the patients with CD and in four of the five patients with UC, the lymphomas were extraintestinal. The mean age of onset of UC in these patients was late (46 years, 19 years older than in our overall series), with lymphomas occurring a mean of only 12 years later. By contrast, patients with CD had bowel disease much younger (mean age, 26 years), and their lymphomas appeared after a longer disease duration (mean, 24 years). The risk factors for the one patient with colonic lymphoma were similar to those with colitis-associated colorectal carcinoma: extensive and long-standing colitis and relatively young age when malignant disease developed. Four of the patients with lymphoma had associated colonic carcinoma; in three of them, the carcinoma appeared within the first decade of colitis, an unusual occurrence. A second malignant lesion also occurred in three patients with UC.
Adult↗
Histiocytic cytophagic panniculitis: molecular evidence for a clonal T-cell disorder.
Histiocytic cytophagic panniculitis is a systemic disease of unknown etiopathogenesis that invariably involves the subcutaneous fat and is histologically characterized by phagocytosis of blood elements by histiocytes that appear to be benign. Immunophenotypic and genotypic studies of biopsy specimens of the lesions of a 58-year-old woman showed that the lymphocytic infiltrates accompanying the histiocytes in the subcutis were composed of clonal T-cells with rearrangement of the surface receptor gene. Our findings suggest that the primary abnormality in histiocytic cytophagic panniculitis may be a clonal T-cell proliferation.
Biopsy↗
Pleural effusion in non-Hodgkin's lymphoma.
Intrathoracic non-Hodgkin's lymphoma (NHL) usually presents with roentgenographic evidence of mediastinal lymph node enlargement, pulmonary masses, pleural effusion, and a clinical picture of a systemic disease with lymphadenopathy. The presentation of NHL with pleural effusion as the major roentgenographic abnormality and no clinical peripheral lymphadenopathy or organomegaly is unusual. During a seven-year period, we encountered 19 patients with NHL in whom pleural effusion was the major roentgenographic and clinical finding. Pleural fluid cytologic results were diagnostic in only two patients. Closed pleural biopsy was positive in three. Eight of 11 patients had diagnostic immunophenotypic lymphocyte cell marker studies. Seven of nine patients had diagnostic thoracoscopy and one thoracotomy. The CT scan identified biopsy sites when pleural fluid and tissue studies were nondiagnostic. Lymphomatous tissue was obtained from the pleura in 17 of the 19 patients supporting the contention that pleural effusion in patients with NHL is usually due to pleural lymphoma rather than obstruction to mediastinal lymphatics.
Adult↗
Human immunodeficiency virus-associated Hodgkin's disease. Clinicopathologic studies of 24 cases and preponderance of mixed cellularity type characterized by the occurrence of fibrohistiocytoid stromal cells.
Hodgkin's disease (HD) was diagnosed in 24 patients who were either seropositive for human immunodeficiency virus (HIV) (21) or members of a high-risk group (three), but had not developed acquired immune deficiency syndrome (AIDS). Clinical presentation of the disease was characterized by constitutional symptoms in all, especially fever (23/24) and disseminated disease (22/24) at diagnosis. Mediastinal adenopathy was rare. Bone marrow involvement was particularly frequent (12/24), and a positive bone marrow biopsy preceded lymph node biopsy in 5 of the 12. Histopathologic features of these tumors included an increased number of nonlymphoid stromal cells, i.e., histiocytic and/or fibroblastoid. In some tumors these fibrohistiocytoid stromal cells were arranged in bundles, but distinct nodule with birefringent collagen band formation was not observed. Twenty-two patients were treated, most with combination chemotherapy; one was untreated; one, unknown. Sixteen, including the one untreated, died with disease at 3 to 25 months; one died of an unrelated cause; four were alive at 3 to 24 months; three were lost to follow-up. Frequent bone marrow involvement at presentation suggests the usefulness of the bone marrow biopsy for diagnosis in subjects at risk, especially when they present with spiking fever of unknown origin. Contrary to most previous series, virtually all of our cases were of mixed cellularity type, characterized by increased fibrohistiocytoid stromal cells in place of depleting lymphocytes. The classic nodular sclerosing feature with birefringent collagen band formation was not observed. In conclusion, HIV-associated HD was characterized by advanced stage with fever at presentation, preponderance of mixed cellularity histologic type with increased fibrohistiocytoid stromal cells, and poor outcome. Hodgkin's disease in AIDS patients presents an intriguing biological model to study the role of stromal histiocytes in immunodeficient patients.
Adult↗
Immunoglobulin expression in B-cell lymphoma. Immunohistochemical study of 345 cases.
Immunoglobulin expression was studied in a series of 345 cases of B-cell lymphoma by immunohistochemical studies and correlated with the histopathologic classification with the use of the Working Formulation. Immunoglobulin expression was present in 254 cases (74%) of B-cell lymphomas (IgM kappa, 122; IgM lambda, 82; light chain only, 40; mu heavy chain only, 10). Immunoglobulin expression occurred with the greatest frequency in lymphomas of small lymphocytic, small cleaved cell, and small noncleaved cell histologic types (93%, 100%, 100%, respectively) and occurred with the least frequency in lymphomas of large cell (cleaved and noncleaved) and immunoblastic histologic types (59% each). Other lymphomas demonstrated intermediate frequencies of immunoglobulin expression. An excess of cases expressing lambda light chain was noted overall (kappa-lambda ratio of 1.3; expected, 2.0) and was particularly evident for intermediate lymphocytic and follicular mixed histologic types (kappa-lambda ratios of 0.8 and 0.9, respectively). Immunoglobulin expression in B-cell lymphomas varies as a function of cellular differentiation as reflected in the histologic type and grade of the Working Formulation. An excess of cases expressing lambda light chain in specific histologic categories suggests the possibility that lymphocytes bearing the lambda light chain rearrangement may be more susceptible to certain types of lymphomatous transformation.
Antibodies, Monoclonal↗
Sarcomatoid neoplasm of monocytic lineage.
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Aged↗
Prediction of prognosis in primary breast cancer by detection of a high molecular weight mucin-like antigen using monoclonal antibodies DF3, F36/22, and CU18: a Cancer and Leukemia Group B study.
Three monoclonal antibodies (MAbs) (DF3, F36/22, CU18) were used to monitor expression of distinct epitopes present within a family of mucin-like, breast carcinoma-associated molecules. Primary tumor specimens from more than 190 stage II breast cancer patients were evaluated for expression of the high molecular weight antigens. With a median follow-up of 6 years, patients whose tumors exhibited high immunoperoxidase staining scores (greater than 50% positive cells) with MAb DF3 had a superior disease-free survival ([DFS] 56% +/- 6% v 37% +/- 5% at 6 years; P = .0088) and overall survival ([OS] 72% +/- 5% v 59% +/- 5% at 6 years; P = .025). Staining scores with the other two antibodies did not correlate with improved prognosis. For MAbs DF3 and CU18, patients whose tumors exhibited predominantly apical cellular reactivity patterns had improved DFS, although differences reached conventional levels of statistical significance only with MAb CU18. In multivariate analyses, the prognostic value of MAb DF3 staining was independent of other identified prognostic factors. Furthermore, the concordance between primary and axillary lymph node metastases staining with each MAb was 73%, 80%, and 85% for MAbs DF3, F36/22, and CU18, respectively. These results suggest that staining with MAb DF3 identifies a group of node-positive women with a relatively favorable prognosis. Expression of the DF3 mucin-like glycoprotein is related to better differentiation, and staining with MAb DF3 provides an accurate and objective estimate of clinical outcome independent of histopathologic evaluation.
Adult↗