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)

Intravascular large B-cell lymphoma (IVLBCL) is pathologically distinct with a broad clinical spectrum and immunophenotypic heterogeneity. A series of 96 patients with IVLBCL (median age, 67 years; range, 41-85 years; 50 men) was reviewed. Anemia/thrombocytopenia (84%), hepatosplenomegaly (77%), B symptoms (76%), bone marrow involvement (75%), and hemophagocytosis (61%) were frequently observed. The International Prognostic Index score was high or high-intermediate in 92%. For 62 patients receiving anthracycline-based chemotherapies, median survival was 13 months. CD5, CD10, Bcl-6, MUM1, and Bcl-2 were positive in 38%, 13%, 26%, 95%, and 91% of tumors, respectively. All 59 CD10- IVLBCL cases examined were nongerminal center B-cell type because they lacked the Bcl-6+MUM1- immunophenotype. CD5 positivity was associated with a higher prevalence of marrow/blood involvement and thrombocytopenia and a lower frequency of neurologic abnormalities among patients with CD10-IVLBCL. Compared with 97 cases of de novo CD5+CD10-diffuse LBCL, 31 cases of CD5+CD10-IVLBCL exhibited higher frequencies of poor prognostic parameters, except age. Multivariate analysis in IVLBCL revealed that a lack of anthracycline-based chemotherapies (P<.001, hazard ratio [HR]: 9.256), age older than 60 years (P=.012, HR: 2.459), and thrombocytopenia less than 100x10(9)/L (P=.012, HR: 2.427) were independently unfavorable prognostic factors; CD5 positivity was not. Beyond immunophenotypic diversity, IVLBCL constitutes a unique group with aggressive behavior.
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PMID:Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5. 1698 83

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma. Thirteen cases of IVLBCL with a median age of 56 years were analyzed retrospectively. Nonspecific symptoms such as fever and hepatosplenomegaly were the most common manifestations, and the bone marrow was usually involved in 8/13 (61.5%) cases. All tumors expressed CD20, and 12/13 (92.3%) of the tumors exhibited a nongerminal center phenotype by Hans algorithm. CD5 was expressed in 3/12 (25%) of the tumors. MYC was negative in all cases, and BCL2 was positive in 10/12 (83.3%) cases. Cytogenetic analysis revealed 5 cases that did not have rearrangements in either the MYC or the BCL2 gene. No association with Epstein-Barr virus was found. Seven of 11 patients received chemotherapy. The median survival time was 6 months. Patients with hemophagocytic syndrome had poor prognoses. Our study demonstrates that IVLBCL has a poor clinical outcome with a high frequency of bone marrow involvement and that the MYC gene may not play an important role in the poor prognosis of IVLBCL.
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PMID:High Frequency of Bone Marrow Involvement in Intravascular Large B-Cell Lymphoma. 2755 79

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma with an estimated incidence of less than one per million. Unlike other hematopoietic malignancies, lymphadenopathy and hepatosplenomegaly are uncommon, and patients typically present with nonspecific symptoms. IVLBCL presents a diagnostic challenge and patients are usually diagnosed late in the disease course, if at all, and the prognosis is poor. The differential diagnosis is broad, and physicians often pursue multiple diagnostic possibilities during patient workup. We present a case of IVLBCL discovered at autopsy in an 80-year-old male who presented with history and symptoms pointing to the tick-borne illness ehrlichiosis.
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PMID:Intravascular large B-cell lymphoma: The Great Imitator. 3077 28

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of extranodal non-Hodgkin's lymphoma, and the absence of specific findings makes ante-mortem diagnosis difficult. This study was conducted to identify the clinical findings useful for timely diagnosis of IVLBCL. Ten patients who were diagnosed with IVLBCL in our institute between 2005 and 2017 were retrospectively analyzed. Eight of the 10 cases had fever and 7 cases presented with respiratory symptoms, including cough, sputum, and dyspnea. Cytopenias were noted in all patients, and serum lactate dehydrogenase levels were elevated in 9 of the 10 patients. Arterial partial pressures of oxygen were <80 mmHg in 6 of the 7 patients examined. Computed tomography scanning detected hepatosplenomegaly and chest abnormalities in 7 and 9 cases, respectively. These results suggest that IVLBCL has a higher frequency of lung involvement than those reported previously. Physicians must therefore be vigilant in the identification of IVLBCL in patients who demonstrate respiratory symptoms or hypoxemia of uncertain origin, because early diagnosis can decrease the severity and prevent mortality.
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PMID:[Clinical features of intravascular large B-cell lymphoma: a single-institute retrospective analysis]. 3106 13