ABSTRACT
B-acute lymphoblastic leukemia/lymphoma (B-ALL) is a neoplasm of precursor cells committed to the B-cell lineage. Extramedullary involvement is frequent, with particular predilection for the central nervous system, lymph nodes, spleen, liver, and testis. We report an unusual case of B-ALL relapsing as an isolated omental mass along with bone marrow involvement.
ABSTRACT
Histiocytic necrotizing lymphadenitis (HNL) is a rare benign disorder characterized histologically by nodal lesions composed of histiocytes, lymphoid cells, and so-called plasmacytoid T cells/plasmacytoid monocytes, with associated karyorrhexis. It has been proposed that plasmacytoid monocytes represent immature myeloid and lymphoid (plasmacytoid) early-committed dendritic cells (DCs). Monoclonal antibodies are now available for the detection of myeloid (CD1c [BDCA-1]+) and plasmacytoid (CD303 [BDCA-2]+) dendritic cells. With an extensive panel of antibodies to immature and mature DCs and interferon-alpha (IFN-alpha), cryostat section studies of 6 cases of HNL revealed that the morphologically distinctive mononuclear cells in lesional areas consisted of 2 populations of immature DCs: myeloid DCs immunoreactive for CD1c with coexpression of myeloid antigens CD13 and CD33 and plasmacytoid DCs immunoreactive for CD303 and CD123. These cells were CD68+, strongly expressed the IFN-alpha inducible protein MxA, and were nonreactive for fascin, a mature DC marker.
Subject(s)
Dendritic Cells/pathology , Histiocytic Necrotizing Lymphadenitis/diagnosis , Adolescent , Adult , Antigens, CD/metabolism , Biomarkers/metabolism , Cryoultramicrotomy , Dendritic Cells/metabolism , Female , Fluorescent Antibody Technique, Direct , GTP-Binding Proteins/metabolism , Histiocytic Necrotizing Lymphadenitis/metabolism , Humans , Immunoenzyme Techniques , Lymphatic Diseases , Male , Myxovirus Resistance Proteins , Phenotype , Young AdultABSTRACT
CD4+/CD56+ hematodermic neoplasm ("blastic natural killer [NK]-cell lymphoma") is a rare highly aggressive neoplasm associated with cutaneous manifestations followed by dissemination to blood, bone marrow, and other tissues. Neoplastic cells exhibit a lineage-negative CD4+/CD56+/CD43+/HLA-DR+ immunophenotype, initially suggesting an NK-cell derivation. The recent discovery of CD123 antigen expression by tumor cells has provided evidence for a relationship to immature dendritic cells (DCs), a group of myeloid and lymphoid early-committed progenitors capable of differentiating into antigen-presenting DCs. Based on flow cytometric analysis, myeloid DCs represent the majority of human peripheral blood DCs and are positive for blood dendritic cell antigen (BDCA)-1 (or CD1c), CD13, CD11c(high), CD33, and CD123(low). Plasmacytoid DCs are BDCA-2+/ CD123(high)+/CD13-/CD33- and produce interferon (IFN)-alpha when triggered by antigens. IFN-alpha production may be detected in tissue sections using antibodies for myxovirus A (MxA) protein, a surrogate marker. This report describes the clinical, histologic, immunophenotypic, cytogenetic, and molecular genetic findings for 3 cases of CD4+/CD56+ hematodermic neoplasms. In all cases, neoplastic cells were reactive for CD123, BDCA-2, and MxA protein, providing strong evidence for an immature plasmacytoid DC derivation for this rare neoplasm.
Subject(s)
CD4 Antigens/metabolism , CD56 Antigen/metabolism , Lectins, C-Type/metabolism , Lymphoma, Non-Hodgkin/diagnosis , Membrane Glycoproteins/metabolism , Receptors, Immunologic/metabolism , Adolescent , Aged , Dendritic Cells/metabolism , Female , GTP-Binding Proteins/analysis , Humans , Immunophenotyping , Lymphoma, Non-Hodgkin/pathology , Male , Middle Aged , Myxovirus Resistance ProteinsABSTRACT
Post-transplant lymphoproliferative disorder (PTLD) is a complication of transplantation resulting from impaired immune surveillance because of pharmacologic immunosuppression. We present two cases of central nervous system (CNS) PTLD in children on calcineurin-inhibitor free immunosuppression with dramatically different presentations and outcomes. One patient had brain and spinal cord lymphoma with a rapid and fatal course. The other patient had brain and ocular PTLD that responded to multimodal therapy with reduction of immunosuppression, high-dose steroids, and rituximab given in a dose-escalation protocol. This protocol may have enhanced the penetration of rituximab into the CNS. We review the literature on CNS and ocular PTLD and elaborate on the treatments available for both diseases.
Subject(s)
Central Nervous System Diseases/etiology , Kidney Transplantation/adverse effects , Lymphoproliferative Disorders/etiology , Review Literature as Topic , Adolescent , Antibodies, Monoclonal/therapeutic use , Antibodies, Monoclonal, Murine-Derived , Central Nervous System Diseases/diagnostic imaging , Central Nervous System Diseases/pathology , Central Nervous System Diseases/therapy , Child, Preschool , Dose-Response Relationship, Drug , Fatal Outcome , Female , Humans , Immunologic Factors/therapeutic use , Immunosuppressive Agents/therapeutic use , Lymphoma/etiology , Lymphoma/pathology , Lymphoproliferative Disorders/diagnostic imaging , Lymphoproliferative Disorders/pathology , Lymphoproliferative Disorders/therapy , Male , Radiography , Rituximab , Steroids/therapeutic use , Treatment OutcomeABSTRACT
We report a case of localized herpes simplex virus lymphadenitis in a patient with mantle cell lymphoma (MCL). A 43-year-old woman with a 2-month history of lymphadenitis and peripheral lymphocytosis received a diagnosis of stage IV MCL based on histologic, flow cytometric, and immunohistochemical findings. One week after completion of chemotherapy, she presented with rapidly enlarging bilateral cervical lymph nodes, a retropharyngeal mass, and incipient respiratory compromise. Multiple biopsies of the cervical nodes and oropharynx that were submitted for morphologic, flow cytometric, and immunohistochemical studies revealed involvement by MCL with superimposed herpes simplex virus lymphadenitis. The recognition of herpes simplex virus infection with MCL is important for treatment as well as to rule out transformation of MCL to a higher-grade lesion.
