Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 48-year-old woman was hospitalized because of severe thrombocytopenia, leg edema, and fever. Intravenous immunoglobulin therapy was administered, but no efficacy was obtained. Her bone marrow was dry-tap, and fibrosis was found in the biopsy specimens. A positron emission tomographic study showed FDG-avid lymphadenopathy and hepatomegaly. Biopsy specimens of axillary lymph nodes showed Castleman's disease-like findings. Since she then developed severe proteinuria and massive pleural effusion, steroid therapy was started, providing temporary relief of symptoms other than the thrombocytopenia. However, rapid worsening of her general condition prompted us to attempt rituximab as salvage therapy. The pleural effusion, edema, and proteinuria disappeared soon after starting rituximab administration. Platelet counts also normalized and fibrosis of the bone marrow showed amelioration. Recently, a variant of multicentric Castleman's disease, termed the TAFRO syndrome, has been proposed, and our patient's features fit the diagnosis of this syndrome. Rituximab might be considered as a therapeutic option in such cases.
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PMID:[Efficacy of rituximab for TAFRO syndrome, a variant type of multicentric Castleman's disease]. 2468 40

Multicentric Castleman disease (MCD) is a lymphoproliferative disorder characterized by systemic symptoms like recurrent lymphadenopathy, fever and hepatosplenomegaly. Human herpes virus 8 (HHV-8) can be associated with MCD whether the patient is infected with human immunodeficiency virus (HIV) or not. A 59-year-old male patient presented with fatigue, drowsiness and enlarged lymph nodes. Thoracic and abdominal computed tomography showed enlarged mediastinal, axillary, paracardiac, paraaortic, celiac, mesenteric, obturator and inguinal lymph nodes concomitant with enlarged liver and spleen. Cervical lymph node biopsy revealed HHV-8 positive plasma cell MCD. The patient's tests were negative for HIV. R-CEOP (rituximab, cyclophosphamide, etoposide, vincristin, prednisolone) and valganciclovir treatments were started simultaneously. After sixth cycle of R-CEOP, the patient achieved unconfirmed complete remission. Rituximab combined with CEOP protocol and antiviral therapy against HHV-8 might be an effective therapeutic approach without a considerable side effect for HHV-8-positive HIV-negative MCD patients.
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PMID:A HHV-8 positive, HIV negative multicentric Castleman disease treated with R-CEOP chemotherapy and valganciclovir combination. 2694 31

Primary hepatic lymphoma (PHL) is a rare clinical entity comprising 0.016% of all cases of non-Hodgkin's lymphoma and 0.4% of extranodal non-Hodgkin's lymphoma and can be missed easily. Here, we report a case of PHL treated with primary hepatic resection followed by an Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisone (R-CHOP) chemotherapy regimen, diagnosed after postoperative biopsy report. The patient presented with complaints of pain abdomen, vomiting, anorexia, and weight loss. She had hepatomegaly and no other significant finding. Blood investigations were unremarkable. Biopsy or fine needle aspiration cytology (FNAC) was not taken before surgery. Contrast-enhanced computed tomography of the abdomen demonstrated well-defined solid mass with central hypodense fluid attenuating area in the liver with a thin pseudocapsule. The differential diagnoses considered were secondary to the liver, hepatocellular carcinoma, and hemangioma. Left hepatectomy with the removal of the middle hepatic vein was performed. The postoperative biopsy was reported as diffuse large B cell lymphoma of the liver.
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PMID:Primary hepatic lymphoma: A rare case report. 3127 45