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)

To explore the clinical and pathological characteristics of hepatosplenic gammadelta T-cell lymphoma and its relationship with Epstein-Barr virus infection, the clinical features of a 9-year-old girl with hepatosplenic gammadelta T-cell lymphoma were investigated, the smears of bone marrow was stained with Wright' s stain, biopsies of bone marrow and liver specimen were embedded in plastic and sliced about 4 microm in thickness and routinely stained with HE staining, the immunohistochemical staining was used to mark the tumor cells, and EBER probes were used to detect Epstein-Barr virus RNA. The results showed that the girl presented with prolonged fever, anemia, thrombocytopenia, hepatosplenomegaly, chronic active Epstein-Barr virus infection, and elevated levels of serum ferritin and lactate dehydrogenase. Bone marrow aspirate revealed the infiltration of atypical lymphocytes in the bone marrow stroma. The liver biopsy specimen revealed the infiltration of lymphocytes in the sinusoids, which was positive for the T-cell associated marker CD3 and activated cytotoxicity-associated marker granzyme B. In-situ hybridization analysis with EBER probes revealed that the above-mentioned characteristics were negative in neoplastic cells. It is concluded that hepatosplenic gammadelta T-cell lymphoma is a disease with distinctive clinical, histopathologic, and phenotypic characteristics. Hepatic and/or splenic and/or bone marrow biopsy with combined phenotype is beneficial to diagnosis. Epstein-Barr virus infection is late event involving an already transformed gammadelta T-cell clone.
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PMID:[Hepatosplenic gammadelta T cell lymphoma and its relationship with Epstein-Barr virus infection]. 1720 79

A 63-year-old male presented with fever and general malaise in June 2004. On admission hepatosplenomegaly was apparent, but without lymphadenopathy. The laboratory examination revealed pancytopenia and increased levels of lactate dehydrogenase, direct bilirubin and soluble interleukin-2 receptor. Histological analysis of the bone marrow biopsy specimen demonstrated proliferation of atypical lymphoid cells positive for CD20 in the small capillaries, leading to the diagnosis of the Asian variant of intravascular large B-cell lymphoma (AIVL). The presence of rearrangement of the immunoglobulin gene confirmed the diagnosis. The patient responded well to CHOP therapy followed by seven courses of rituximab-combined CHOP therapy and has remained in complete remission up to the present. This case implies that bone marrow biopsy could be a useful examination for diagnosing AIVL and that rituximab-combinedchemotherapy could improve survival in patients with the disease.
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PMID:[Asian variant of intravascular large B-cell lymphoma diagnosed by bone marrow biopsy]. 1731 78

T-cell lymphoma-associated hemophagocytic syndrome (T-LAHS) has been frequently reported in Asian countries and is considered with extremely poor prognosis. To summarize its clinical characteristics and explore its early diagnosis and treatment, we retrospectively analyzed the records of 113 patients with aggressive T cell lymphoma, of which 28 were associated with LAHS. According to WHO classification (2001), 22 cases were classified into peripheral T-cell lymphoma (unspecified), 2 into extranodal NK/T-cell lymphoma, and 4 into systemic anaplastic large cell lymphoma. The median survivals of the LAHS and no-LAHS groups were 40 days and 8 months, respectively. The elevating rates of serum lactate dehydrogenase (LDH) (100% vs. 55%), ferritin (100% vs. 64%), fasting triglycerides (79% vs. 43%), and hypofibrinogen (43% vs. 14%) levels were higher in the LAHS group than in the no-LAHS group (P < 0.05), so were bone marrow involvement (57% vs. 32%, P < 0.05) and liver dysfunction (40% vs. 13%, P < 0.05). Eleven of the 28 LAHS patients did not receive any chemotherapy, and 14 received CHOP regimen as initial chemotherapy. Three patients in critical conditions were given plasma exchange and gained the chance of initial chemotherapy. We suggest that in patients presenting with fever, hepatosplenomegaly, cytopenia, and constantly increasing levels of serum LDH, CA125, ferritin, transglutaminase, and beta2-microglobulin, T-LAHS should be taken into account. Repeating biopsies of multiple parts of bone marrow may help diagnosis. The therapeutic result of chemotherapy alone or combined for T-LAHS was discouraging and the survival time of most cases was no more than 1 year. Plasmapheresis as initial therapy is worth considering in critical cases.
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PMID:Clinical characteristics of T-cell lymphoma associated with hemophagocytic syndrome: comparison of T-cell lymphoma with and without hemophagocytic syndrome. 1820 16

Hematological complications have been frequently associated with acute brucellosis, but pancytopenia is less frequently seen. Also, capillary leak syndrome has been rarely reported in the literature. In this report, we present a case of brucellosis with pancytopenia leading to capillary leak syndrome. A 21-year-old man was admitted to hospital with complaints of a one-month history of weakness, sweats, and fever and he had hepatosplenomegaly and edema over the pretibial areas. Hemogram revealed pancytopenia and biochemical tests revealed moderate hypoalbuminemia, elevations of lactate dehydrogenase and aspartate aminotransferase. He was diagnosed as brucellosis and capillary leak syndrome. He was given doxycycline and rifampicin. The patient's symptoms were resolved after treatment.
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PMID:The first documented case of brucellosis manifested with pancytopenia and capillary leak syndrome. 1845 81

This study was to investigate the predictive factors influencing prognosis of non-Hodgkin's lymphoma (NHL). The clinical data on 125 cases of NHL were analyzed retrospectively. The results indicated that in 125 cases, the incidence of B cell NHL (B-NHL) was 68%, T cell NHL (T-NHL) was 28%, and uncertained cases were 4%. B-NHL was with more bone marrow involvement, while T-NHL was associated with more presence of B symptom, increased lactate dehydrogenase (LDH), advanced clinical stage and higher International Prognostic Index (IPI) scores. For T-NHL and B-NHL, the 3-year overall survival (OS) rate was 41.07% and 71.64% respectively. Age, B symptom, LDH level, and clinical stage were associated with OS. Immunophenotyping was not identified as a significant prognostic factor. The incidence of born marrow involvement was 31.2%, mainly in B-NHL. The involvement manner had no relationships with age, B symptom, LDH level and T/B immunophenotyping. Diffuse bone marrow involvement was often linked with hepatosplenomegaly, and its OS was shorter than that focal manner. In conclusion, age, B symptom, LDH level and clinical stage affect NHL survival, while immunophenotyping was not an independent prognostic factor for NHL. The manner of bone marrow involvement helped to predict prognosis.
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PMID:Clinical and prognostic analysis of 125 cases of non-Hodgkin's lymphoma. 1854 26

We analysed the long-term outcome of the L86 protocol using L-asparaginase (L-asp), vincristine (VCR) and prednisolone (PSL), collectively known as LVP or L97 protocol using LVP along with pirarubicin hydrochloride (THP-ADR) for 97 patients with acute lymphoblastic leukemia (ALL) diagnosed between 1986 and 2002. No significant differences were seen in the two protocols regarding the complete remission (CR) rate or survival. Seventy-five of the 97 patients (77%) achieved a CR. The overall survival (OS) and disease-free survival (DFS) rates were 32.1% and 30.4% at 10 years, respectively. By univariate analysis, we identified seven adverse factors for DFS which included the L2 subtype by French-American-British classification, hepatosplenomegaly, a white blood cell count of more than 30 x 10(9)/L, a blast cell count of more than 10 x 10(9)/L in the peripheral blood, hemoglobin concentration greater than 10 g/dL, a serum lactate dehydrogenase value greater than twice the upper limit of normal and the presence of the Philadelphia chromosome (Ph). According to multivariate analysis, only the presence of Ph was a significant unfavourable factor for DFS and OS. In the 30 patients under 35 years of age without Ph, the OS in the 20 patients treated with L86 and in the 10 patients treated with L97 were 48 and 86%, respectively (P = 0.011). These results indicate that intensified chemotherapy, such as the L97 protocol that includes an anthracycline, might be beneficial for younger patients who are Ph-negative.
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PMID:Long-term outcome of L86 and L97 protocols for adult acute lymphoblastic leukemia. 1902 Oct 56

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

Based on the rewiew of the literature and own clinical observations, we presented examples of the most common pediatric onco-hematologic malignancies, that are manifested early by osteoarticular complaints. When these complaints predominate in the clinical presentation, they lead the diagnosis towards nonmalignant conditions, that are most common cause of such symptoms in children, like injuries, nonspecific reactive arthritis or inflammatory connective tissue diseases. However, in acute lymphoblastic leukaemia, the most prevalent childhood malignancy, bone and joint pains are present early in 40-60% of cases and they frequently anticipate any abnormalities in complete blood counts. Findings reported in the literature and own observations indicate that these complaints correlate with: lower white cell counts, lower percentage of blast cells in the peripheral blood and lower incidence of organomegaly - that may delay the decision of bone marrow aspiration. In our study we have also presented 4 cases of other malignancies in children, who were complaining of the osteoarticular pains, that limited they activity, long time before the beginning of treatment in Department of Oncology and Pediatric Hematology of University's Pediatric Hospital in Cracow. Long-lasting and intensive osteoarticular complains, that restraint normal activity and do not resolve during rest, spinal compression symptoms, coexisting adenopathy, hepatosplenomegaly, weight loss, change of behaviour, unexplicained fever must be recognized as specific "red flags". Oncologic vigilance must be inspired by discreet hematological abnormalites (like increased anemia, lower white cell counts with lymphocytosis, mild thrombocytopenia) -that indicate bone marrow infiltration, as well as high erythrocyte sedimentation rate, accompanied by moderately elevated C-reactive protein - characteristic for malignancy. Basic and commonly accessible radiological imaging may provide valuable information, because it can reveal tumors, osteolytic lesions or destruction of bone architecture. Laboratory tests of lactate dehydrogenase (LDH) and uric acid level, often raised in malignancies are also helpful. The aim of this study was to focus the attention of pediatricians to the necessity of including malignancy in the differential diagnosis of intensive or unexplained osteoarticular complaints.
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PMID:[Osteoarticular pains as early manifestation of malignancies in children]. 1948 54

Hemophagocytic syndrome (HS) may be primary, or secondary, to malignancy, or to metabolic, collagen vascular, and infectious diseases such as brucellosis, miliary tuberculosis and some viral and fungal infections. The diagnostic findings of HS are high fever, hepatosplenomegaly, cytopenia, high serum ferritin and triglycerides, and low serum fibrinogen levels. Brucellosis is a zoonotic disease, with fever, fatigue, sweating, arthritis, hepatosplenomegaly, lymphadenopathy, and cytopenia being the most common symptoms and findings. Hematological manifestations of the disease may include anemia, leucopenia, leukocytosis, thrombocytopenia, and thrombocytosis. Brucellosis may occur in association with HS. Here, we describe brucellosis associated HS in an 8 year-old male patient. The patient was admitted to our clinic with weight loss, arthralgia, prolonged fever, sweating, and fatigue. Physical and laboratory findings revealed hepatosplenomegaly, pancytopenia, elevated serum transaminases, triglycerides, lactate dehydrogenase, and ferritin, and with erythrocytes, leukocytes, and thrombocytes phagocytosed by macrophages indicating hemophagocytosis. The Brucella agglutination test was positive. The patient improved after treatment with Rifampin (15 mg/kg/day) and trimethoprim-sulfamethoxazole (10 mg/kg/day).
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PMID:A rare hematological manifestation of brucellosis: reactive hemophagocytic syndrome. 2045 34

A 14-year-old girl, who had been suffering from intermittent fevers for 2 months, developed painful erythematous plaques on the lower extremities. Laboratory data revealed elevated C-reactive protein, lactate dehydrogenase, and aspartate aminotransferase/alanine aminotransferase (AST/ALT). Blood and urine cultures were negative. CT showed hepatosplenomegaly. F-18 FDG PET revealed multiple patchy uptakes on the subcutaneous surfaces residing mainly at the lower trunk and extremities. The PET images and clinical manifestations appeared indistinguishable from those due to panniculitis while the pathology from skin biopsy demonstrated panniculitis-like T-cell lymphoma. She received chemotherapy and the follow-up PET showed significant resolution of previous abnormal uptakes from the subcutaneous lesions.
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PMID:F-18 FDG PET images for subcutaneous panniculitis-like T-cell lymphoma. 2115 18


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