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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The defects in lymphocyte apoptosis that underlie the autoimmune lymphoproliferative syndrome (ALPS) are usually attributable to inherited mutations of the CD95 (Fas) gene. In this report, we present the histopathological and immunophenotypic features seen in the lymph nodes (n = 16), peripheral blood (n = 10), bone marrow (n = 2), spleen (n = 3), and liver (n = 2) from 10 patients with ALPS. Lymph nodes showed marked paracortical hyperplasia. Interfollicular areas were expanded and populated by T cell receptor-alphabeta CD3+ CD4-CD8- (double-negative, DN) T cells that were negative for CD45RO. CD45RA+ T cells were increased in all cases studied. The paracortical infiltrate was a result of both reduced apoptosis and increased proliferation, as measured by in situ detection of DNA fragmentation and staining with MIB-1, respectively. The paracortical proliferation may be extensive enough to suggest a diagnosis of malignant lymphoma. Many of the paracortical lymphocytes expressed markers associated with cytotoxicity, such as perforin, TIA-1, and CD57. CD25 was negative. In addition, most lymph nodes exhibited florid follicular hyperplasia, often with focal progressive transformation of germinal centers; in some cases, follicular involution was seen. A polyclonal plasmacytosis also was present. The spleens were markedly enlarged, more than 10 times normal size. There was expansion of both white pulp and red pulp, with increased DN T cells. DN T cells also were observed in liver biopsies exhibiting portal triaditis. In the peripheral blood, the T cells showed increased expression of HLA-DR and CD57 but not CD25. CD45RA+ T cells were increased in the four cases studied. Polyclonal B cell lymphocytosis with expansion of CD5+ B cells was a characteristic finding. Taken together, the histopathological and immunophenotypic findings, particularly in lymph nodes and peripheral blood, are sufficiently distinctive to suggest a diagnosis of ALPS. Of note, two affected family members of one proband developed lymphoma (T-cell-rich B-cell lymphoma and nodular lymphocyte predominance Hodgkin's disease, respectively).
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PMID:Pathological findings in human autoimmune lymphoproliferative syndrome. 981 46

We report a case of Hodgkin's disease that developed 4 years after autologous peripheral blood stem cell transplantation (PBSCT). The patient, a 67-year-old Japanese male, underwent PBSCT for multiple myeloma (IgA-lambda type) under a conditioning regimen of carboplatin, etoposide and cyclophosphamide in June 1994 and obtained partial remission. In January 1995, he underwent a second PBSCT under a conditioning regimen of high-dose melphalan and obtained complete remission. In December 1998, he noticed swelling of the left cervical lymph nodes, and Hodgkin's disease of the mixed cellularity subtype was diagnosed from a lymph node biopsy sample. Immunohistochemistry studies showed that the Hodgkin's and Reed-Sternberg cells were CD30-positive. In situ hybridization using EBER probes showed that these cells expressed EBER RNA transcripts, indicating that the tumor was associated with Epstein-Barr virus. One noteworthy feature was the absence of both M-component in the serum and urine, as revealed by immunoelectrofixation, and plasmacytosis in the bone marrow, which would have indicated relapse of the multiple myeloma. After chemotherapy with MOPP and ABVD, the Hodgkin's disease was eradicated and the patient obtained complete remission in May 1999.
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PMID:[Development of Hodgkin's disease four years after autologous peripheral blood stem cell transplantation for multiple myeloma]. 1120 Nov 52

The association of Hodgkin disease with monoclonal gammopathy has rarely been reported. We present a case of a 48-year-old woman with a history of autoimmune hemolytic anemia and Graves disease who presented with hepatosplenomegaly and a gamma-heavy-chain paraprotein. Histopathology of lymph node and bone marrow revealed nodular lymphocyte-predominance Hodgkin disease, while examination of the spleen revealed plasmacytosis consistent with gamma-heavy-chain disease. Following splenectomy, the patient has remained in complete remission for both conditions with no further treatment. To our knowledge, this is the first report of a patient with both gamma-heavy-chain disease and nodular lymphocyte-predominance Hodgkin disease. Given recent data indicating the B-cell nature of this form of Hodgkin disease, the authors propose that in this unique case there may be a clonal relationship between these 2 concurrent B-cell lymphoproliferative processes.
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PMID:Composite nodular lymphocyte-predominance Hodgkin disease and gamma-heavy-chain disease: a case report and review of the literature. 1137 Dec 36

Unlike monoclonal gammopathy of undetermined significance (MGUS) or non-Hodgkin's lymphomas (NHLs) with plasmacytoid differentiation, multiple myeloma (MM) is commonly associated with lytic bone lesions. Although the mechanisms of increased osteoclast activity are partially understood, comparatively little is known about the mechanisms that lead to the observed decrease in osteoblast function. Studies have shown neural cell adhesion molecule (NCAM) homophilic binding between MM cell lines and osteosarcoma cell lines, and that binding results in decreased osteoid production in vitro. Thus, we postulated that the expression of NCAM by MM cells contributes to lytic lesion formation by causing decreased osteoid production in vivo. We used immunohistochemistry in bone marrow core biopsies to assess NCAM expression in osteoblasts and plasma cells (PCs) in vitro. We found consistent, strong, uniform NCAM expression by the osteoblasts in all bone marrow core biopsies (352 of 352, 100%). Strong expression of NCAM by PCs correlated with the presence of lytic bone lesions (chi-square, 33.39: P <0.000; odds ratio, 16.9). There was also a strong correlation between NCAM expression and the diagnosis of MM in comparison to reactive PCs, MGUS, or NHLs with plasmacytoid differentiation (all P values <0.000). In conclusion, using immunohistochemistry, we found strong expression of NCAM by osteoblasts and that when equal to the intensity of osteoblast expression, NCAM expression by PCs correlates with the presence of lytic bone lesions and distinguishes MM from reactive plasmacytosis, NHLs with plasmacytoid differentiation, and most cases of MGUS.
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PMID:Expression of CD56/neural cell adhesion molecule correlates with the presence of lytic bone lesions in multiple myeloma and distinguishes myeloma from monoclonal gammopathy of undetermined significance and lymphomas with plasmacytoid differentiation. 1194 14

Primary Sjogren's syndrome (pSS) is a chronic autoimmune disease with well-documented association of lymphoid malignancies during the progress of the disease. Although several types of malignancy and pseudomalignancy have been reported in pSS, low-grade non-Hodgkin's lymphomas are the most frequently observed. Reactive plasmacytosis mimicking myeloma is a very rare condition in association with pSS. We describe a 72-yr-old woman with pSS who presented with hypergammaglobulinemia, and extensive bone marrow and lymph node plasmacytosis, which mimicked multiple myeloma. In this patient, there was an abnormal differentiation of memory B cells to plasma cells in the peripheral blood suggesting underlying pathogenetic mechanism for this condition.
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PMID:A case of reactive plasmacytosis mimicking multiple myeloma in a patient with primary Sjogren's syndrome. 1595 79

Lymphadenopathy, which may be associated with systemic symptoms, is frequently associated with rheumatoid arthritis (RA). Reactive non-neoplastic tissue comprises the majority of the lymph node lesions. However, several cohort studies have demonstrated that RA has an increased risk of non-Hodgkin's lymphomas (NHLs). Since the early 1990s, an atypical or malignant lymphoproliferative disorders (LPD) in patients immunosupressed with methtorexate (MTX) therapy for RA has been emphasized, namely MTX-associated LPDs. Epstein-Barr virus (EBV) has received attention in connection with the etiology of RA. The present review describes the clinicopathologic and immunohistochemical findings of reactive, atypical and malignant LPDs associated with RA along with the presence or absence of EBV in LPDs using the in situ hybridization (ISH) method. The majority of reactive lymph node lesions exhibit reactive follicular hyperplasia with interfollicular polyclonal plasmacytosis. Atypical LPDs rarely appears in RA patients. However, these cases occasionally pose difficult problems in the differential diagnosis from malignant lymphomas associated with RA or atypical and malignant LPDs showing RA-like clinicopathological findings. Clinicopathologically, three types of atypical LPDs have delineated, i.e. (i) resembling multicentric Castleman's disease (MCD); (ii) atypical paracortical hyperplasia with lymphoid follicles (APHLF) and; (iii) atypical lymphoplasmacytic immunoblastic proliferation. Malignant lymphoma associated with RA is characterized by; (i) predominance of elderly cases; (ii) usually female predominance, reflecting the sex ratio of RA; (iii) longstanding history of RA; (iv) relatively frequent advanced stage of disease; (v) majority of the patients had the B-cell phenotype; and (vi) an increased frequency of diffuse large B-cell lymphoma (DLBCL) in RA. It is unlikely that EBV is the causative agent of either reactive or atypical LPD. Among malignant lymphomas, EBV-associated lymphoma comprised only a small fraction of all NHLs in the general RA patient population.
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PMID:Benign, atypical and malignant lymphoproliferative disorders in rheumatoid arthritis patients. 1706 72

The hematologic, histologic and morphologic bone marrow findings of 18 patients with human immunodeficiency virus (HIV) infection were reviewed. The mean age of the patients studied was 27 years; age range was six to 63 years. The main bone marrow morphologic finding was hypercellularity (72%), which was mainly due to megakaryocytic hyperplasia with or without granulocytic or erythrocytic hyperplasia. Naked (denuded) megakaryocytic nuclei, which are considered an indicator of HIV infection, were present in 72% of the bone marrows examined. Reticuloendothelial iron blockade was identified in 78% of cases. Other less frequent findings included erythrocytic dysplasia (44%), plasmacytosis (28%), nonspecific granulomas (17%), Hodgkin's and non-Hodgkin's lymphoma (17%), lymphocytic aggregates (11%) and histiocytosis (6%) . The bone marrow findings in this series of HIV patients appear to be similar to what has been previously reported from other countries.
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PMID:Bone marrow morphologic findings in patients with human immunodeficiency virus (HIV) infection. 1737 95

The diagnoses of Hodgkin lymphoma and multiple myeloma have rarely been made simultaneously in the same patient. We present a case of an 82-year-old man who rapidly developed pancytopenia and liver failure with coagulopathy. Serum protein electrophoresis and immunofixation revealed an unequivocal immunoglobulin Gkappa and immunoglobulin Glambda biclonal gammopathy. Bone marrow biopsy showed involvement by classic Hodgkin lymphoma with an inflammatory background including 49% mature plasma cells. Unfortunately, the patient died 14 days after admission. To our knowledge, a case of concurrent Hodgkin lymphoma and biclonal multiple myeloma has not previously been reported. Detection of severe bone marrow plasmacytosis in the background of Hodgkin lymphoma should alert the pathologist to the possibility of collision with a plasma cell neoplasm, warranting a complete diagnostic workup.
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PMID:Concurrent diagnoses of Hodgkin lymphoma and biclonal myeloma in the bone marrow. 2063 33

Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of non-Hodgkin lymphoma characterized by aggressive symptoms and various abnormal laboratory test results. One of the rare immunologic abnormalities in AITL is exuberant polyclonal plasmacytosis, but its clinical significance has not been evaluated. This report concerns three AITL cases with exuberant polyclonal plasmacytosis and investigates its clinical impact by comparison with 12 patients without plasmacytosis. Our study found that the performance status (PS) of the former was significantly worse and their serum immunoglobulin levels were significantly higher. All other parameters, including B symptoms, various prognostic scores, blood cell counts other than plasmacyte, and serum levels of lactate dehydrogenase, C-reactive protein and soluble interleukin-2 receptor, showed no significant differences. More importantly, although the diagnosis of AITL with plasmacytosis was not straightforward in our series, outcomes of treatment with conventional chemotherapy or immunosuppressive therapy with cyclosporine A were favorable. To conclude, AITL should be considered a candidate underlying disease of exuberant polyclonal plasmacytosis. Provided a correct diagnosis is made early and is followed by adequate treatment, the prognosis for AITL with plasmacytosis may not be worse than that for those without plasmacytosis despite the severe exhaustion at first presentation.
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PMID:Clinical manifestation of angioimmunoblastic T-cell lymphoma with exuberant plasmacytosis. 2394 15

Castleman disease (CD) is a benign heterogenous lymphoproliferative disorder presenting with varied clinical manifestations. Histomorphologically, two distinct variants are known, hyaline vascular type and plasma cell type. CD is reported to be associated with lymphomas. Though Non Hodgkin lymphoma is more commonly associated with CD, its association with Hodgkin lymphoma (HL) is also well known and documented. Many a times, CD obscures or masks the underlying lymphomas thus delaying the diagnosis. There are also reports of HL showing Castleman like features. We report a case of HL with Castleman like features which posed diagnostic difficulty owing to the fragmented nature and tiny size of the initial lymph node biopsy. Striking peripheral blood rouleaux formation which was seen in our case along with bone marrow plasmacytosis has not been described in the previously diagnosed cases.
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PMID:Diagnostic issues in a case of hodgkin lymphoma with castleman like features. 2533 27


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