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)

A case of peripheral T-cell lymphoma presenting with secondary myelofibrosis and meningeal involvement is described. A 65-year-old female was admitted because of remarkable weight loss and pancytopenia. On admission, she was confused and showed tiny cervical lymph nodes but no hepatosplenomegaly. Bone marrow aspiration resulted in dry tap and its biopsy showed remarkable myelofibrosis with marked decrease of hematopoiesis and increase of lymphoid cells. Lymph node biopsy revealed diffuse medium sized cell lymphoma, which was diagnosed as CD3+4+8-peripheral T-cell lymphoma with immunohistochemistry (anti-HTLV-1 antibody negative). The lymphoid cells of bone marrow expressed the markers of T-cell lineage (LCA+ UCHL1+ MT1+ L26- MB1-). The cerebrospinal fluid examination revealed many lymphoma cells. She was treated with CHOP regimen and intrathecal injection of MTX. After three months, bone marrow biopsy showed recovery of hematopoiesis and disappearance of lymphoma cells and reticulin fibers. Immunohistochemical analysis of bone marrow specimen was useful for the diagnosis of atypical myelofibrosis.
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PMID:[Peripheral T-cell lymphoma initially presenting as secondary myelofibrosis]. 269 63

We report a rare case of idiopathic myelofibrosis transformed to acute myelomonocytic leukemia associated with non-Hodgkin's lymphoma. A 64-year-old woman was admitted to our department because of anemia and leukocytosis. On admission, anemia and hepatosplenomegaly were noted. The hemoglobin content was 6.8 g/dl, and WBC count was 26,200/microliters with an increased number of immature neutrophils. Bone marrow biopsy revealed an increased amount of reticulin fiber. Because she had no disease which causes secondary myelofibrosis, idiopathic myelofibrosis was diagnosed, and she was treated with prednisolone, anabolic steroid and blood transfusion. Fifteen months after the diagnosis of myelofibrosis, blast increased in her peripheral blood and her spleen and liver enlarged remarkably. A tumor of right parotid region was recognized at the same time. The pathological diagnosis of biopsied tumor was non-Hodgkin's lymphoma. The cytochemical study of blasts in her peripheral blood showed that she had acute myelomonocytic leukemia. In spite of intensive chemotherapy, she died from heart failure, respiratory failure and renal insufficiency.
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PMID:[Idiopathic myelofibrosis transformed to acute myelomonocytic leukemia associated with non-Hodgkin's lymphoma]. 807 93

Ankle oedema and abdominal swelling suddenly developed in a 55-year-old woman who also had lymphadenopathy in the neck, axillae and groin. Ultrasonography demonstrated hepatosplenomegaly, ascites and pleural effusions. Histological examination of some lymph-nodes from the axilla and groin revealed angioimmunoblastic lymphadenopathy (low-malignant peripheral T cell lymphoma). Bone-marrow biopsy was undertaken because of a normocytic anaemia (haemoglobin 4.9 g/dl) requiring blood transfusion, thrombocytopenia (5000/microliters) and monoclonal IgG gammopathy. This showed lymphoma-associated secondary myelofibrosis. Treatment with prednisone (2 mg/kg daily for 8 weeks) and vincristine (1 mg/m2 once weekly for 4 weeks) brought about partial remission of the angioimmunoblastic lymphadenopathy with normalization of the clinical and laboratory findings, the splenohepatomegaly regressed, and there was only a small amount of ascites. Four months after onset of the illness bone-marrow biopsy also showed regression of the myelofibrosis.
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PMID:[Reversible myelofibrosis in angioimmunoblastic lymphadenopathy]. 818 22

Anemia and hepatosplenomegaly are common reasons for referring a child to a pediatric hematologist or oncologist. Among the many causes for these findings is severe rickets, which has been shown to be associated with secondary myelofibrosis and myeloid metaplasia. The authors present the case of an infant with severe rickets and secondary myelofibrosis and review the differential diagnosis of hepatosplenomegaly from the viewpoint of the pediatric hematologist/oncologist.
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PMID:Anemia and hepatosplenomegaly as presenting features in a child with rickets and secondary myelofibrosis. 1452 7

A 68-year-old man was referred to our hospital in August 2003 with a high fever, a prominent inflammatory reaction in blood test and also bicytopenia (anemia and thrombocytopenia) with marked hepatosplenomegaly. He was temporarily diagnosed as having malignant lymphoma considering the elevated levels of LDH and soluble interleukin-2 receptor, following which treatment with the CHOP regimen was started. Thereafter, based on the pathological findings from a bone marrow biopsy and a quite high viral load revealed by real-time PCR analysis, the diagnosis was changed to Epstein-Barr virus (EBV) related B-lymphoproliferative disorder (B-LPD) complicated with reticulin fibrosis. A total of 2 courses of the CHOP regimen together with anti-viral reagents almost resolved the clinical symptoms and abnormal findings of laboratory tests. This unique case was considered to be "a senile EBV positive B-LPD" complicated with a secondary myelofibrosis, a category of the disorder which has recently been proposed by Shigeo Nakamura at the Aichi Cancer Center.
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PMID:[Epstein-Barr virus related B-cell lymphoproliferative disorder complicated with bone marrow fibrosis, which was successfully treated with 2 courses of CHOP regimen]. 1644 Jul 76

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

The authors describe 10 cases of myelofibrosis diagnosed and managed at their center over 16 years. There were 2 and 8 cases, respectively, of primary and secondary myelofibrosis. All patients presented with fever, pallor, hepatosplenomegaly, and/or lymphadenopathy. Hodgkin's lymphoma (n = 4), neuroblastoma (n = 1), thrombasthenic thrombopathy (n = 1), and retroperitoneal-mass (n = 1) were causal in 7 patients, whereas the diagnosis could not be established in a sole case of secondary myelofibrosis. Patients were managed with chemotherapy and appropriate care. However, outcome was poor. The authors emphasize variable clinical-laboratory spectrum of myelofibrosis, highlight management concerns, and demonstrate that prognosis/outcome depends upon appropriate management of the underlying condition.
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PMID:Myelofibrosis in children: experience at a single tertiary care center in India. 2067 Jan 65

A 75-year old man had been diagnosed at 42 years of age as having polycythemia vera and had been monitored at another hospital. Progression of anemia had been recognized at about age 70, and the patient was thus referred to our center in 2008 where secondary myelofibrosis was diagnosed based on bone marrow biopsy findings. Hematemesis due to rupture of esophageal varices occurred in January and February of 2011. The bleeding was stopped by endoscopic variceal ligation. Furthermore, in March of the same year, hematemesis recurred and the patient was transported to our center. He was in irreversible hemorrhagic shock and died. The autopsy showed severe bone marrow fibrosis with mainly argyrophilic fibers, an observation consistent with myelofibrosis. The liver weighed 1856 g the spleen 1572 g, indicating marked hepatosplenomegaly. The liver and spleen both showed extramedullary hemopoiesis. Myelofibrosis is often complicated by portal hypertension and is occasionally associated with gastrointestinal hemorrhage due to esophageal varices. A patient diagnosed as having myelofibrosis needs to be screened for esophageal/gastric varices. Myelofibrosis has a poor prognosis. Therefore, it is necessary to carefully decide the therapeutic strategy in consideration of the patient's concomitant conditions, treatment invasiveness and quality of life.
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PMID:Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension. 2285 73