Primary effusion lymphoma with central nervous system involvement in an HIV-negative homosexual male.

Primary effusion lymphoma (PEL) is a rare form of non-Hodgkin lymphoma that presents with body cavity effusions. It occurs chiefly in immunodeficient HIV-positive patients. The tumor cells generally express gene sequences of human herpesvirus-8 (HHV-8) and Epstein-Barr virus (EBV).

Postchemotherapy histiocyte-rich pseudotumor involving the spleen.

We report 2 cases of splenic postchemotherapy histiocyte-rich pseudotumor. Each patient had a history of diffuse large B-cell lymphoma, treated with multiagent chemotherapy. Computed tomography scans performed on both patients showed splenic masses.

Transient atypical monocytosis mimic acute myelomonocytic leukemia in post-chemotherapy patients receiving G-CSF: report of two cases.

Granulocyte colony-stimulating factor (G-CSF) is now widely used in patients with malignant disorders receiving intensive chemotherapy to increase leukocyte count and to upregulate phagocyte function during neutropenia. Monocytosis associated with G-CSF has been reported in anecdotal literature. We report two cases of pseudoleukemia secondary to G-CSF administration.

High-grade T-cell lymphoma complicating B-cell chronic lymphocytic leukemia: an unusual manifestation of "Richter's syndrome".

Approximately 3% of patients with B-cell chronic lymphocytic leukemia (CLL) develop a high-grade large-cell lymphoma consistent with Richter's Syndrome. In most cases, these lymphomas are of B-cell origin and are believed to arise by clonal evolution from the CLL cells. We present a case of a patient with a 10-year history of B-CLL who developed an aggressive large-cell lymphoma, confirmed by immunophenotype to be of T-cell origin.

MEIS1 and HOXA7 genes in human acute myeloid leukemia.

Co-activation of Meisl with Hoxa7 or Hoxa9 homeobox genes by retroviral gene insertion has recently been reported to be leukemogenic in murine myeloid leukemia. In this study we determined their expression in human leukemia. Most human myeloid leukemia cell lines co-expressed MEIS1 with HOXA7 and HOXA9.

Apoptotic index from fine needle aspiration cytology as a criterion to predict histologic grade of non-Hodgkin's lymphoma.

Objective: To investigate whether the assessment of apoptotic index (AI) from fine needle aspiration (FNA) smears of non-Hodgkin's lymphomas (NHL) is reliable and has potential utility as a criterion to predict histologic grade.

Study Design: AI was independently determined by four cytopathologists as a percentage from routine FNA smears in 96 NHLs and 15 lymphoid hyperplasias. Working formulation (WF) grades from corresponding surgical biopsies were modified to include mantle zone-derived NHLs as intermediate grade and to make diffuse large cell NHL a separate category called "high" grade, whereas WF high grade NHLs were called "very high" grade.

Peripheral T cell lymphoma in a patient with common variable immunodeficiency disease: case report and literature review.

This report documents the occurrence of a peripheral T cell lymphoma arising in the bone marrow and liver of a patient with common variable immunodeficiency disease. The T cell origin of this lymphoma was demonstrated by immunohistochemical phenotyping and gene rearrangement studies and was not associated with EBV infection of the lymphoma cells. The frequency and characteristics of lymphomas complicating CVID are reviewed.

Multilineage involvement and erythropoietin-"independent" erythroid progenitor cells in a patient with systemic mastocytosis.

We report on a patient with systemic mastocytosis with an activating point mutation of the c-kit gene. This mutation was identical to the c-kit mutation recently described by other groups. Additionally, we found that in this patient the mutation was also present in myeloid and erythroid lineages, indicating a multilineage involvement and suggesting a clonal origin of the disease similar to that described in other myeloproliferative disorders.

Large-cell variants of mantle cell lymphoma: cytologic characteristics and p53 anomalies may predict poor outcome.

Large-cell variants are uncommon in mantle cell lymphoma (MCL). Here we describe the pathologic and clinical findings in five patients with large-cell lymphoma related to MCL (L-MCL), and compare them to a group of classic small-cell MCL (s-MCL) cases. Histologically, the MC origin of the large cells was evinced by their association with a small mantle cell component in the same tissue, or their distribution in a classic mantle zone pattern, or their development in a patient with previous s-MCL.

Morphological, ultrastructural, and genetic characterization of an unusual T-cell lymphoma in a patient with sinus histiocytosis with massive lymphadenopathy.

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare benign disease of unknown etiology. It is rarely associated with malignant lymphoma. This report documents the first case of a T-cell lymphoma, which developed in a patient with a 10-year history of SHML.

A paraneoplastic mixed bullous skin disease associated with anti-skin antibodies and a B-cell lymphoma.

Background: The full spectrum of bullous diseases associated with underlying cancers remains to be fully defined.

Observation: We describe a patient with a mixed bullous disease exhibiting combined features of cicatricial pemphigoid and pemphigus and associated with a B-cell lymphoma producing an IgM paraprotein to intercellular antigens in human skin. The patient had the clinical features of cicatricial pemphigoid and the histologic and immunofluorescence abnormalities of both cicatricial pemphigoid and pemphigus.

Immunophenotypic evaluation of the bone marrow in non-Hodgkin's lymphoma.

Immunophenotypic evaluations of the bone marrow (BM) are reported on 69 aspirates from 58 patients who had non-Hodgkin's lymphoma or chronic lymphocytic leukemia involving the BM. Using flow cytometry and immunofluorescence microscopy on density gradient isolated BM mononuclear cells, the neoplasm could be identified and characterized in 59 aspirates (86%) from 49 patients (84%). Using International Working Formulation guidelines the neoplasms were classified on the basis of prior or subsequent histopathology of lymph node, spleen, skin, or other soft tissue site, or by evaluation of peripheral blood in chronic lymphocytic leukemia.

IgM monoclonal gammopathy/Waldenström's macroglobulinemia: a morphological and immunophenotypic study of the bone marrow.

The presence of a monoclonal paraprotein in the serum or urine raises the possibility of myeloma or lymphoma/leukemia. Yet, in a significant proportion of individuals with serum paraproteins, particularly those with low levels of paraprotein, clinical and routine bone marrow evaluation is not diagnostic of an underlying neoplasm. The purpose of this study was to define the pathologic basis for macroglobulinemia in patients whose routine bone marrow biopsies were not diagnostic of a lymphoplasmacytic neoplasm.

The association of progressive, atrophying, chronic, granulomatous dermohypodermitis with Hodgkin's disease.

The case of a patient with an unusual skin disorder--progressive, atrophying, chronic, granulomatous dermohypodermitis (PACGD)--who developed Hodgkin's disease is reported. A review of the literature revealed only two other cases of PACGD, one of which affected a patient who also was found to have Hodgkin's disease. In an additional report, the diagnosis of Hodgkin's disease was made in a patient who may have had the same dermatologic disorder.

The karyotype of Philadelphia chromosome-negative, bcr rearrangement-positive chronic myeloid leukemia.

Philadelphia (Ph) chromosome negative chronic myeloid leukemia (CML) can be distinguished from clinically similar disorders on the basis of the presence of rearrangement of the breakpoint cluster region (bcr) of chromosome 22. We have identified six patients with Ph-negative CML, each with bcr rearrangement. Apparently normal karyotypes were observed in two cases, and a third contained a rearrangement that did not appear to involve chromosomes 9 or 22.

Immunohistological analysis of Rosai-Dorfman histiocytosis. A disease of S-100 + CD1-histiocytes.

Five cases of Rosai-Dorfman histiocytosis (RDH) (also called Sinus Histiocytosis with Massive Lymphoadenopathy; SHML) have been studied by immunohistochemical methods with heteroantisera and monoclonal antibodies. One case was also studied by Southern blot hybridization analysis with DNA probes specific for T cell receptor beta chain and immunoglobulin heavy chain. Immunophenotyping of large histiocytes, characteristic of RDH, evidenced the presence of S-100 protein and the absence of CD1 and other markers usually found in histiocytes and macrophages.

Simultaneous occurrence of mycosis fungoides and Hodgkin disease: clinical and histologic correlations in three cases with ultrastructural studies in two.

We present three patients who manifested both Hodgkin disease and mycosis fungoides. Ages ranged from 39 to 66 and two were male. Skin lesions were present from 3 to 40 years before the diagnosis of Hodgkin disease.

Evolution of Sézary syndrome in the course of hairy cell leukemia.

A patient with a history of "leukemia" for 19 yr and documented hairy cell (HC) leukemia for 10 yr developed mycosis fungoides and the Sézary syndrome. The manifestations of both diseases were diagnostic on clinical and pathologic grounds. Ultrastructural, immunohistochemical, and surface marker techniques proved the HC to have phenotypic characteristics of the T-helper subset of lymphocytes to which the Sézary cells (SC) also belonged.

Autoimmune thrombocytopenic purpura in homosexual men.

Since November 1980 we have diagnosed 11 cases of severe autoimmune thrombocytopenic purpura in homosexual men; their mean platelet count (+/- SE) was 16 000 +/- 3000/mm3. All patients have been sexually active with multiple partners and exposed to numerous viruses and drugs. During this period, we also have diagnosed 20 cases of classic autoimmune thrombocytopenic purpura in heterosexual persons, with a normal women to men ratio of 3:1.

Procainamide-induced agranulocytosis and thrombocytopenia.

Procainamide therapy has frequently been reported as a cause of agranulocytosis, but severe thrombocytopenia associated with the use of this drug has been noted only once. We report a case of simultaneously occurring agranulocytosis and profound thrombocytopenia in a patient receiving procainamide hydrochloride. Different mechanisms appeared to be responsible for the two cytopenias.