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)

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

An unusual case of small cell variant of Ki-1 non-Hodgkin's lymphoma diagnosed one year after an original diagnosis of idiopathic myelofibrosis is reported. On the second occasion, the patient presented with fever, lymphadenopathy and hepatosplenomegaly. A lymph node biopsy specimen confirmed a diagnosis of small cell variant of Ki-1 lymphoma. A repeat bone marrow biopsy specimen showed myelofibrosis with no evidence of lymphomatous infiltration, but cytogenetic studies on blood, bone marrow and skin fibroblasts revealed a novel chromosomal translocation t(3,4)(q13;q12).
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PMID:Small cell variant of Ki-1 lymphoma associated with myelofibrosis and a novel constitutional chromosomal translocation t(3;4) (q13;q12). 867 43

We studied 14 adult patients presenting with fever and cytopenia of the peripheral blood and histiocytic hyperplasia with hemophagocytosis (HHH) in the bone marrow regarding an association of cytomegalovirus (CMV) and Epstein-Barr virus (EBV) by using in situ hybridization (ISH) and also evaluated the clinical and laboratory findings according to the encountered organisms. ISH using a CMV RNA probe demonstrated infected cells in 6 out of 14 cases (43%), and ISH using an EBV EBER RNA probe demonstrated infected nuclei in 5 out of the same 14 cases (36%) of HHH. No cases showed a positive reaction with both probes. Three cases showed a negative reaction with both probes. The mean age of all patients was 29 years; and that of the CMV-positive patients was 27 years and that of the EBV-positive patients was 36 years. Organomegaly was found in 3 out of 6 CMV-positive patients (1 hepatomegaly, 1 splenomegaly, 1 hepatosplenomegaly), and 4 out of 5 EBV-positive patients (lymphadenopathy in all 4 cases, hepatosplenomegaly in 2 cases). One of the CMV-positive case had acute myeloblastic leukemia, and 2 EBV-positive cases had underlying malignancy (1 Hodgkin's disease, 1 non-Hodgkin's lymphoma). Seven out of the 14 HHH cases (50%) died within several months after diagnosis. Nucleic acid hybridization methods can be used for the routine examination of the association of CMV or EBV.
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PMID:In situ hybridization studies of cytomegalovirus and Epstein-Barr virus in reactive histiocytic hyperplasia with hemophagocytosis. 887 10

Hepatosplenic T gamma/delta lymphoma is a rare entity of peripheral T cell lymphoma. Three of 386 patients with non-Hodgkin's lymphoma in our institute were found to have this subtype of lymphoma. All had chromosomal abnormalities of isochromosome 7q and trisomy 8. The clinical and hematological features of these three patients are reported. All were males with ages ranging from 23 to 29 years. Initial presentation comprised purpura and variable degree of hepatosplenomegaly. None had superficial lymphadenopathy. Hematologically, they showed pictures resembling immune related thrombocytopenia and/or hemolytic anemia. Examination of the bone marrows revealed hypercellularity with increased number of megakaryocytes and erythroid cells and various degrees of abnormal lymphoid cell infiltration. The histopathologic section of the spleen from one patient who underwent splenectomy revealed abnormal cell infiltration in the sinusoids of the red pulp. Lymphoma cells showed T gamma/delta lymphoid immunophenotype (CD3+ CD2+ CD4- CD8-, TCR delta-1+, and beta F1-). The platelet counts were elevated transiently after initial treatment with corticosteroids, but the condition soon deteriorated. All died of refractory lymphoma five to nine months after diagnosis. Review of the literature, showed that only four other cases have been reported until now and although no cytogenetic data were available for these patients, they had very similar clinical pictures as those in this series. It is suggested that hepatosplenic T gamma/delta lymphoma represents a rare, but distinct, clinicopathological and cytogenetic entity.
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PMID:Clinical and hematological characteristics of hepatosplenic T gamma/delta lymphoma with isochromosome for long arm of chromosome 7. 888 63

We describe a case of peripheral T cell lymphoma that is remarkable for its fulminate course and selective targeting of both kidneys. The patient was a 6-year-old girl who was in her usual state of good health until the onset of abdominal pain and fever. She was treated for acute oliguric renal failure and visual disturbances. A renal biopsy was performed. Biopsy findings were interpreted as suggestive of a vasculitic process, and treatment was initiated for a presumptive diagnosis of Wegener's granulomatosis. The patient died 3 days following admission, and autopsy revealed extensive bilateral kidney infiltration by a peripheral T cell lymphoma. The remainder of the body was spared with the exception of mild infiltration of the pulmonary parenchyma and choroid plexus by neoplastic lymphocytes. The neoplastic nature of the disease was confirmed utilizing immunoperoxidase stains and T cell receptor gene rearrangement. Primary renal lymphoma and renal failure attributable to involvement by lymphoma are rare findings that should be considered when other more common causes of renal insufficiency have been excluded. The presenting clinical complaints are generally of short duration, nonspecific, and atypical. Most patients exhibit oliguria. Physical examination may reveal hepatosplenomegaly, lymphadenopathy, and flank and/or abdominal mass(es). Laboratory findings frequently include an elevated serum creatinine, blood urea nitrogen, lactate dehydrogenase, and a mild proteinuria. Electrolyte abnormalities are variably present. Possible radiographic findings include hypodense or hypoechoic renal lesions and diffuse bilateral renal enlargement. Although the prognosis is dismal, survival may be prolonged utilizing current treatment modalities, and rare patients may be "cured" of disease. The clinical presentation, radiological findings, and prognosis of patients with clinically evident renal involvement by non-Hodgkin's lymphoma are discussed.
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PMID:Rapidly progressive T cell lymphoma presenting as acute renal failure: case report and review of the literature. 918 23

A previously well 70 year old woman was admitted to hospital following a three day history of vomiting and confusion. Her serum calcium was 6.58 mmol/l, phosphate 1.09 mmol/l, and alkaline phosphatase 91 iu/l. The mechanism of this hypercalcaemia was not obvious as there was no evidence of a primary malignancy, lymphadenopathy or hepatosplenomegaly. The calculation of indices of urinary excretion of calcium and phosphate suggested the presence of excessive parathyroid hormone (PTH) activity as the mechanism of hypercalcaemia. Plasma intact PTH, 25-hydroxycholecalciferol, and 1,25-dihydroxycholecalciferol were not raised suggesting the presence of PTH related peptide (rP). This led to a systematic search for a malignancy, which revealed the presence of a high grade B cell non-Hodgkin's lymphoma confined to the bone marrow. Plasma PTH-rP was subsequently shown to be raised confirming the interpretation of the initial urinary and calcium excretion indices. This case highlights the value of standard laboratory measurements such as urinary calcium and phosphate excretion in cases of hypercalcaemia of obscure aetiology, which can complement measurements of PTH and other calcitropic hormones.
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PMID:Value of assessing parathyroid hormone-like activity in a case of extreme hypercalcaemia. 965 76

Ki-1 anaplastic large cell lymphoma is a well-described subtype of non-Hodgkin's lymphoma with distinctive characteristics from the cytological, immunohistochemical and clinical points of view. One of the clinical behavior characteristic is that it rarely evolves into a leukaemic phase. We report the case of a 72-year-old man in which the appearance of tumor cells in peripheral blood was one of the most revealing information. The patient showed B-symptoms, bicytopenia and bone marrow involvement, together with hepatosplenomegaly and right axilar adenopathy, which after biopsied lead to Ki-1 anaplastic large cell lymphoma's diagnosis (stage IV-B). As far as the treatment and evolution are concerned, we choose a polychemotherapy (ACOP-B) because of the patient's age. Up to now clinical and analitical course is excellent and the patient is now in remission.
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PMID:[Ki-1+ large-cell anaplastic lymphoma with a leukemic appearance. Study of a case]. 974 Dec 34

We report a 75-year-old Japanese man infected with hepatitis C virus (HCV) who died of acute hepatic failure due to the hepatic infiltration of B-cell non-Hodgkin's lymphoma (NHL) cells. He suddenly developed jaundice, fatigue, fever, and hepatosplenomegaly during the course of chronic infection with HCV. Postmortem liver necropsy revealed extensive infiltration of lymphoma cells into the liver. Although the association between HCV infection and NHL has recently become a matter of concern, we believe this to be the first reported case of acute hepatic failure caused by hepatic involvement of non-Hodgkin's lymphoma in an HCV-infected patient.
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PMID:Acute hepatic failure due to hepatosplenic B-cell non-Hodgkin's lymphoma in a patient infected with hepatitis C virus. 985 65

A 26-year-old man presented with a 38 degrees C fever of undetermined origin in November 1977. His fever subsided in 1 month, but recurred concomitantly with dysphagia on December 10. For this reason, the patient visited our out patient clinic on December 15, and was hospitalized because a mass was found radiographically in the left lower lung field. A contrast-enhanced computed tomographic scan of the chest revealed an irregularly-shaped mass encircling the esophagus. Based on open-lung biopsy findings, the mass was diagnosed as non-Hodgkin's lymphoma, diffuse pleomorphic type. Neither swelling of the superficial lymph nodes nor hepatosplenomegaly was detected, and a bone-marrow aspiration specimen disclosed no atypical cells. Ga scintigraphy found accumulation only in this region, suggesting the posterior mediastinum as the origin of the lymphoma. Reports of non-Hodgkin's primary lymphoma in the posterior mediastinum are rare. Further, to our knowledge, there have been no reports at all on the diffuse pleomorphic type to date. This case was accordingly considered to be of highly informative value.
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PMID:[Posterior mediastinal diffuse pleomorphic lymphoma]. 1021 48

The clinical presentation and surgical and pathological findings of 46 children with unilateral tonsillar enlargement (UTE; age range 2 to 13 years, mean age 6.5) who underwent tonsillectomy for biopsy purposes between 1975 and 1995 were compared with those of 7 children who received treatment for tonsillar lymphoma (TL; age range 2 to 9 years, mean age 4.8) during the same period. There was no history of rapid tonsillar enlargement in children in the UTE group, and only 20 (43%) were symptomatic. Symptoms included recurrent sore throats in 10 patients (22%), snoring in 5 (11%), nasal obstruction in 4 (9%), and dysphagia in 1 (2%). No children had systemic symptoms or significant cervical lymphadenopathy. In contrast, tonsillar enlargement was observed to occur within a 6-week period in all children with TL, and 6 (86%) children had symptoms at presentation that included dysphagia in 5 (71%), snoring in 3 (43%), night sweats in 2 (29%), and fever and rigors in 2 (29%). Cervical lymphadenopathy greater than 3 cm was present in 6 (86%) children, while 1 child (14%) had hepatosplenomegaly. There was no histopathologic evidence of neoplasia in the UTE group, and a true discrepancy in size between the two tonsils was confirmed in only 21 of 44 (48%) cases. All 7 patients in the TL group had non-Hodgkin's lymphoma. All received chemotherapy, with 5 of the 7 cured and 2 dying of disease. The data suggest that tonsillectomy should be performed for biopsy purposes in UTE where there is a history of progressive enlargement, significant upper aerodigestive tract symptoms, systemic symptoms, suspicious appearance of the tonsil, cervical lymphadenopathy, or hepatosplenomegaly. The diagnosis of TL should also be considered when UTE is present in an immunocompromised child or one with a previous malignancy, when acute tonsillitis is asymmetric and unresponsive to medical treatment, or when rapid bilateral tonsil enlargement occurs. Observation is appropriate management for other cases of UTE.
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PMID:Unilateral tonsillar enlargement and tonsillar lymphoma in children. 1052 79


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