Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The surface molecule CD56 marks a category of malignant lymphoma of putative natural killer (NK) cell origin. We conducted a retrospective analysis of 24 cases of CD56+ NK lymphoma/leukaemia to define the clinicopathologic and prognostic features of this specific group of lymphomas. 56 cases of nasal lymphomas and 204 cases with an initial diagnosis of peripheral T-cell lymphoma were retrospectively analysed. To specifically examine lymphomas of putative NK origin, only those that were negative for surface expression of CD3 but positive for CD56 were analysed. 24 cases were identified. The initial predominant sites of involvement were nasal (n = 18), palate (n = 1), nodal (n = 1) and multi-organ (n = 4). Clinically, in patients with disease localized to one anatomical site (n = 20), most had symptoms confined to the nose, with a high percentage in early stage (I: 91%; IV: 9%). The marrow was not involved in any of these cases. However, patients with multi-organ involvement at presentation (n = 4) behaved differently. All presented acutely with pancytopenia, hepatosplenomegaly, and marrow infiltration with haemophagocytosis. A leukaemic phase was observed in one case. Anthracycline containing combination chemotherapy resulted in complete remission in 75% of patients with localized disease, but only in 25% with multi-organ involvement. The median survival of patients with localized disease was 12 months, compared with 2 months in the multi-organ group (P = 0.06); the disease-free survival was significantly better in the former (P < 0.01). The overall median survival of all patients was still poor at 11 months. We conclude that CD56+ NK lymphomas could be divided into two main patterns of disease presentations: localized (predominantly nasal), and multi-organ involvement. Each has different clinicopathologic and prognostic features. Conventional chemotherapy appeared ineffective for the majority of patients, and innovative treatment modalities are needed to improve outcome.
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PMID:CD56+ NK lymphomas: clinicopathological features and prognosis. 921 83

Cytotoxic cells include natural killer (NK) cells and cytotoxic alpha beta and gamma delta T lymphocytes (CTLs). These cells express cytotoxic molecules of T-cell restricted intracellular antigen (TIA-1), and activated cytotoxic molecules of perforin, granzyme B, and FasL. Recent studies suggest that most extranodal T-cell lymphomas are derived from CTLs, and that NK cell lymphomas are extranodal. However, only a few nodal NK and cytotoxic lymphomas have been described so far. We present here the clinicopathological features of seven cases of nodal cytotoxic T and NK cell lymphomas. The study excluded anaplastic large-cell lymphomas expressing cytotoxic molecules. The neoplastic cells of all cases contained activated cytotoxic molecules of TIA-1, granzyme B, Fas ligand, and/or perforin. Phenotypically and genotypically, four cases showed alpha beta T cell type [CD2+, CD3+, T-cell receptor (TCR)-delta-1-, beta F1+, and TCR gene rearrangement], two cases showed gamma delta cell type [CD2+, CD3+, T-cell receptor (TCR) delta-1+, beta F1-, and TCR gene rearrangement], and one case showed NK cell type [CD2+, CD3-, CD56+, T-cell receptor (TCR) delta-1-, beta F1-, and TCR gene germline]. Using Southern blot analysis, Epstein-Barr virus (EBV) sequences were detected in six cases, and monoclonal terminal repeat proliferation was confirmed. In addition, in situ hybridization (ISH) studies for EBV showed EBV infection in almost all neoplastic cells. Clinically, all patients presented with peripheral lymphadenopathy in high clinical stages and showed an aggressive course. Hepatosplenomegaly was detected in six cases. During the course of the disease, bone marrow and extranodal invasion were noted in five cases. The nodal type showed an aggressive clinical course in all cases but one, as did the extranodal type. The nodal type varied in phenotype, but was closely associated with EBV infection.
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PMID:Clinical, immunohistochemical and phenotypic features of aggressive nodal cytotoxic lymphomas, including alpha/beta, gamma/delta T-cell and natural killer cell types. 1059 6

A 21-year-old Dutch crossbred mare was presented with a 1-month history of weight loss and weakness. Clinical evaluation revealed severe anemia and thrombocytopenia with evidence of hepatic disease and muscular damage. Necropsy findings included diaphragmatic rupture with an extensive retroperitoneal hematoma and severe hepatosplenomegaly. Microscopic findings were characterized by hepatic sinusoidal and splenic red pulp infiltration by atypical CD3-positive lymphocytes. No other nodal or extranodal sites were affected. Hepatosplenic lymphoma with a probable T-cell origin was diagnosed based on gross and histologic findings.
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PMID:Hepatosplenic T-cell lymphoma in a mare. 1212 57

Hepatosplenic T-cell lymphoma is an uncommon neoplasm characterized by a lymphoid infiltrate within the sinusoids of the liver, spleen, and bone marrow, without significant nodal involvement. The majority of cases express the gammadelta T-cell receptor and are associated with an isochromosome 7q cytogenetic abnormality. Recently, a small number of cases have been reported that express the alphabeta T-cell receptor. Here, we report our findings of a case of an S100-positive hepatosplenic alphabeta T-cell lymphoma in a 20-year-old woman who presented with pancytopenia and hepatosplenomegaly. The case adds to the growing literature of hepatosplenic alphabeta T-cell lymphomas.
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PMID:Hepatosplenic alpha beta T-cell lymphoma: a report of an S100-positive case. 1265 97

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a primary lymphoproliferative T-cell disorder, currently classified as a peripheral T-cell non-Hodgkin's lymphoma. AILD is characterized by generalized lymphadenopathy, hepatosplenomegaly, immunological abnormalities, polyclonal hypergammaglobulinemia and anemia. We report a case of AILD in an 80-year-old male who presented with a generalized pruritic maculopapular eruption and fever following doxycycline administration. The maculopapular rash progressed to formation of confluent nodules, plaques and finally erythroderma with lymphadenopathy and hepatosplenomegaly. Blood analysis revealed an elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. Lymph node biopsy showed almost complete effacement of the nodal architecture with diffuse proliferation of small vessels forming an arborizing network, surrounded by atypical lymphocytes, usually CD3+ CD4+ and occasionally CD3+ CD8+. There were also larger cells (immunoblastic shape) that displayed CD20 positively, some scattered plasma cells, and eosinophils. Histology of a cutaneous lesion showed spongiosis and infiltration of the epidermis by atypical lymphocytes with large hyperchromatic nuclei, perivascular dermal lymphocytic infiltrate (CD3+) mixed with plasma cells and occasional large immunoblasts (CD20+). During hospitalization the patient developed hemolytic anemia (Coombs positive) and lung metastases. The prognosis of AILD is generally poor, with a median survival of less than 20 months. Our patient died two and a half months after the diagnosis was made due to sepsis caused by Staphylococcus aureus isolated in hemoculture.
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PMID:Angioimmunoblastic lymphadenopathy with dysproteinemia following doxycycline administration. 1272 71

The occurrence of abnormal Hodgkin's and Reed-Sternberg cells in the peripheral blood in a patient suffering from Hodgkin's disease has been noticed exceptionally rare in a previous period, and especially rare in last ten years primarily due to successful treatment of this disease. The presence of atypical mononuclear cells in peripheral blood which cytomorphologically resembled Reed-Sternberg cells was registered in 8 patients till 1966. During the last decade, the presence of atypical mononuclear cells in the peripheral blood was used for their isolation, cultivation, and detailed immunophenotypic and genetic analysis. The analysis of mononuclear cells in rare patients with Hodgkin's disease was established that they belong to the B-lymphoid cells with expression of CD30 and CD15 antigens. The examination of presence of Hodgkin's cells in the peripheral blood of patients with Hodgkin's disease is important for patients with advanced stage of the disease in which autologous stem cell transplantation and high dose chemotherapy is planned. The authors present a 33-year-old patient, who noticed enlarged neck lymph nodes in September 2000, high temperature and loss in weight. On physical examination enlarged neck lymph nodes 5 x 8 cm and hepatosplenomegaly were found. There was anemia and thrombocytopenia, and normal WBC count with 24% of lymphoid elements in differential formula. On histologic examination of lymph nodes, Hodgkin's disease, type nodular sclerosis with mixed cellularity was found. Histology of bone marrow showed nodal lymphomatous infiltration. Immunohistochemistry with monoclonal antibodies of concentrate of peripheral blood cells showed expression of CD30+ and CD15+, immunophenotypically and morphologically matching Reed-Sternberg cells. Cytogenetic analysis of mononuclear cells of the bone marrow showed normal karyotype. The patient was in clinical stage IV/V of the disease and chemotherapy with 9 cycles of ABVD + Mp protocol was applied. He is still in remission.
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PMID:[Hodgkin's and Reed-Sternberg cells in the peripheral blood of a patient with advanced stage Hodgkin's disease]. 1505 20

Inflammatory pseudotumor (IPT) is an uncommon benign disorder characterized by proliferation of spindle cells, inflammatory cells, and small vessels. The IPT of lymph nodes is a rare cause of lymphadenopathy that usually affects one or two nodal groups. We describe a 27-year-old male presenting with generalized lymphadenopathy, hepatosplenomegaly and fever for 1 year. Histologic examination of lymph node revealed few remnant lymphoid aggregates with marked sclerosis and numerous anastomosing blood vessels in lymph node parenchyma. Interspersed in between the fibrotic bands was a polymorphic infiltrate composed of lymphocytes, plasma cells, eosinophils, and immunoblasts. Also, many histiocytes, multinucleated giant cells some being Langhan's type and at places forming ill-defined granulomas were observed. The presence of granulomas and Langhan's type of giant cells can lead to a misdiagnosis of tuberculosis as was also done in the present case. It is thus not only important to be aware of this rare benign pathologic entity while dealing with a case of lymphadenopathy, but also consider it in the differential diagnosis of granulomatous disease.
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PMID:Inflammatory pseudotumor of lymph nodes presenting as pyrexia of unknown origin. 1841 62

Hepatosplenic T-Cell lymphoma (HSTCL) is a rare form of extra-nodal post-thymic T-cell non-Hodgkin's lymphoma that primarily involves liver and spleen with B symptoms, with a characteristic absence of lymphadenopathy. We report such an entity in a 65-year-old man who was diagnosed to have multiple myeloma and treated for the same for two years. A clinical diagnosis of secondary myelofibrosis was suspected and was investigated, when he developed pancytopenia and massive hepatosplenomegaly at one of his follow-up visits. The patient underwent therapeutic splenectomy with a simultaneous wedge biopsy of the liver and with their corresponding histopathological and immunohistochemical features, the diagnosis of HSTCL was clinched.
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PMID:Malignant lymphoma without lymphadenopathy. 1870 Jun 47

We retrospectively reviewed the clinical and laboratory data and bone marrow (BM) histomorphologic features in 33 angioimmunoblastic T-cell lymphoma (AITL) cases. Paraffin-embedded BM core biopsy specimens were reacted immunohistochemically with antibodies to pan-T-cell markers, CD20, CD10, CD21, and bcl-6. The frequency of BM involvement was 70% (23/33). The following features were more significant in patients with than without BM involvement: fever, hepatosplenomegaly, pleural effusion, elevated lactate dehydrogenase level, hypoalbuminemia, hyponatremia, hypocalcemia, anemia, circulating atypical cells, hypercellular marrow, and plasmacytosis in the BM. Morphologic features included nodular or interstitial infiltration in a paratrabecular distribution, periodic acid-Schiff-positive intercellular materials, mixed infiltrates of T and B cells, presence of clear cells, and blood vessel proliferation. Immunohistochemical assays revealed that CD5, bcl-6, and CD10 were useful markers of BM infiltration. Seven cases with BM involvement were interpreted as negative for lymphoma initially, mainly owing to insufficient information in nodal biopsy specimens. Several clinical and laboratory features indicate BM involvement of AITL at diagnosis. Laboratory physicians should be more familiar with BM histomorphologic and immunophenotypic characteristics suggesting AITL infiltration to make accurate diagnoses even without prior nodal biopsies.
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PMID:Distinct features of angioimmunoblastic T-cell lymphoma with bone marrow involvement. 1936 22

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive non-Hodgkin's nodal peripheral T-cell lymphoma characterized by general lymphadenopathy, night sweats, fever, hepatosplenomegaly, polyclonal hypergammaglobulinemia, and cutaneous involvement. We present a rare case of AITL cutaneous involvement mimicking toxic erythema recurring with AITL relapse and suggesting a precursor of disease progression.
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PMID:Cutaneous involvement in angioimmunoblastic T-cell lymphoma. 2106 26


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