Neoplasms of histiocytic and dendritic cells are rare disorders of the lymph node and soft tissues. Because of this rarity, the corresponding biology, prognosis and terminologies are still being better defined and hence historically, these disorders pose clinical and diagnostic challenges. These disorders include Langerhans cell histiocytosis (LCH), histiocytic sarcoma (HS), follicular dendritic cell sarcoma (FDCS), interdigtating cell sarcoma (IDCS), indeterminate cell sarcoma (INDCS), and fibroblastic reticular cell tumors (FRCT).
View Article and Find Full Text PDFBackground: Kikuchi-Fujimoto disease (KFD) is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. This disease is misdiagnosed as malignant lymphoma in up to one-third of cases and is associated with the development of systemic lupus erythematosus (SLE).
Methods: The medical literature between the years 1972 and 2014 was searched for KFD, and the data were collected and analyzed regarding the epidemiology, clinical presentations, diagnosis, management, and suggested diagnostic and treatment algorithms.
Background: Dendritic and histiocytic cell neoplasms are rare malignancies that make up less than 1% of all neoplasms arising in lymph nodes or soft tissues. These disorders have distinctive disease biology, clinical presentations, pathology, and unique treatment options. Morphology and immunohistochemistry evaluation by a hematopathologist remains key for differentiating between these neoplasms.
View Article and Find Full Text PDFThe objective of this study was to investigate and quantify the morphological and molecular changes in the thymus for common causes of human infant death. Thymic architecture and molecular changes apparent in human infant head trauma victims were assessed by microscopy and quantified by image analysis of digital whole slide images. Thymuses from victims of SIDS and suffocated infants displaying normal thymus architecture were used for comparison.
View Article and Find Full Text PDFAngioimmunoblastic T-cell lymphoma (AITL) is a systemic lymphoproliferative disease characterized by a polymorphous neoplastic infiltrate involving lymph nodes as well as extranodal locations, including the skin. Cutaneous involvement is seen in approximately 50 percent of cases and is usually secondary to systemic disease. Patients with cutaneous involvement classically present with a transient morbilliform eruption of the trunk; however, papules, nodules, urticarial plaques and erythroderma have also been reported.
View Article and Find Full Text PDFCastleman disease (CD) is a rare lymphoproliferative disorder that primarily affects mediastinal, retroperitoneal, and cervical lymph nodes. Clinically, these lesions occur as a localized (unicentric) or less frequently as a systemic (multicentric) disease. Two main distinct histologic variants are recognized, the more common hyaline-vascular (HV) type and the plasma cell (PC) type.
View Article and Find Full Text PDFA 31-year-old man with no significant medical history presented with a 5-day history of progressive left upper quadrant abdominal pain. Physical examination revealed a tender guarded abdomen, no icterus, and bilateral corneal "arcus senilis"-like changes. Laboratory workup showed a mild normocytic, normochromic anemia; and target cells were seen in the peripheral blood smear.
View Article and Find Full Text PDFPrimary cutaneous B-cell lymphoma is a B-cell lymphoma of the skin with no evidence of extracutaneous involvement at the time of diagnosis. In this report, we describe an 8-year-old boy who presented with a firm, alopecic, skin-colored, smooth nodule over the right frontal scalp. Histological examination revealed a mid-to deep-dermal mononuclear lesion.
View Article and Find Full Text PDFAcute basophilic leukemia (ABL) is an uncommon form of acute myelogenous leukemia recently recognized as a distinct entity in the World Health Organization classification of myeloid malignancies. A case is presented of ABL arising from chronic myelogenous leukemia with development of t(7;8)(q32;q13). Discussion includes a literature review.
View Article and Find Full Text PDFAutophagy is an evolutionarily conserved 'self-eating' process. Although the genes essential for autophagy (named Atg) have been identified in yeast, the molecular mechanism of how Atg proteins control autophagosome formation in mammalian cells remains to be elucidated. Here, we demonstrate that Bif-1 (also known as Endophilin B1) interacts with Beclin 1 through ultraviolet irradiation resistance-associated gene (UVRAG) and functions as a positive mediator of the class III PI(3) kinase (PI(3)KC3).
View Article and Find Full Text PDFContext: Although the early post-myelosuppressive chemotherapy pathologic changes of the marrow have been described, the rate and the histologic definition of recovery are not defined.
Objective: To study the rate of recovery of bone marrow in patients given myelosuppressive therapy for acute myelogenous leukemia, establish the histologic criteria of recovered marrow, and correlate the recovery pattern with those patients who received a bone marrow transplant by using histology, peripheral blood, immunophenotyping, and computerized morphometry and mathematical slope equation.
Design: We studied the post-myelosuppression recovery of the bone marrow to determine patterns and rate of recovery in 135 serial bone marrow biopsies of 51 patients.
Background: Peripheral T-cell neoplasms (PTCNs) comprise a group of uncommon and heterogeneous lymphoid malignancies. They are more difficult to diagnose and treat and have a worse prognosis than B-cell lymphomas. Although PTCNs initially show a significant degree of chemosensitivity, the outcome of treatment with conventional dose chemotherapy remains poor.
View Article and Find Full Text PDFBackground: The spectrum of diseases that constitute the CD30+ lymphomas, with lymphomatoid papulosis (LyP) at one end, and anaplastic large-cell lymphoma (ALCL) at the other end, shows variable morphology, immunophenotype, and clinical behavior. The border between these diseases is sometimes difficult to establish and there are many grey zones in their classification.
Methods: We reviewed the clinical and research literature and guided by our experiences attempted to discern molecular and phenotypic criteria to improve the classification and identify molecular targets for therapy of CD30-positive cutaneous lymphomas.
Background: The classification of cutaneous lymphomas has been contentious. Two major competing classifications were the World Health Organization (WHO) and the European Organization for Research and Treatment of Cancer (EORTC). The principal authors met for a consensus meeting resulted in a combined classification called WHO-EORTC Classification of Cutaneous Lymphoma.
View Article and Find Full Text PDFCytometry B Clin Cytom
January 2007
Background: A method and approach is developed for fully automated measurements of immunostained lymphocytes in tissue sections by means of digital color microscopy and patent pending advanced cell analysis. The validation data for population statistic measurements of immunostained lymphocytes in tissue sections using tissue cytometry (TC) is presented. The report is the first to describe the conversion of immunohistochemistry (IHC) data to a flow cytometry-like two parameter dot-plot display, hence the technique is also a virtual flow cytometry.
View Article and Find Full Text PDFA 50-year-old woman presented with a 2-year history of facial lesions that were resistant to rosacea therapy. Evaluation of histology, immunohistochemistry, gene rearrangement study, bone-marrow biopsy specimen, and systemic workup revealed the findings of lymphoplasmacytoid lymphoma (immunocytoma) in both the skin lesions and bone marrow, and IgM kappa paraprotein. Lesions cleared after chemotherapy.
View Article and Find Full Text PDFBackground: Lymph node (LN) histopathologic class has been shown to be a significant determinant of survival in patients with mycosis fungoides. Often, histopathologic evaluation of just 1 node is used in staging patients with cutaneous T-cell lymphoma.
Objective: We examined whether sampling multiple nodes alters the staging and prognostic group placement of patients with mycosis fungoides as compared with sampling just 1 node.
Background: Debates regarding nosology and clonality surround the entity known as cutaneous pseudolymphoma and its questionable transformation to frank cutaneous lymphoma. The relevance of these arguments is important, not only from a diagnostic standpoint, but also for making inferences based upon behavior, prognosis, and treatment.
Objective: Our goal was to demonstrate further evidence of progression from cutaneous pseudolymphoma to malignant lymphoma while at the same time advocating a comprehensive plan for evaluation, treatment, and followup of these patients.