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)

The anti-tumor therapy followed by tumor lysis syndrome may cause the metabolic disorders including hyperkalemia, hyperphosphatemia and hyperuricemia. It should be known that it occurs frequently in lymphoproliferative diseases, especially in Burkitt's lymphoma. Two cases of T-ALL accompanied by this syndrome, from which the patients were recovered, at the induction therapy of the first complete remission are reported here. Case 1. A 28-year-old man received VP therapy under the diagnosis of T-ALL with massive hepatosplenomegaly and bilateral enlarged kidneys. During the therapy, metabolic disorders with both renal failure and ventricular tachycardia happened. They were resolved by certain series of treatments. The patient was brought to a complete remission with normal size of liver, spleen and kidneys. Case 2. A boy aged 15 having received the intrasubarachnoidal infusion of MTX and 1-Ad-VP therapy under the diagnosis of T-ALL accompanied by this syndrome which was improved by an appropriate treatment, and the patient was lead to the remission. The risk factors of this syndrome, such as 1-high drug sensitivity of the tumor; 2-renal dysfunction; 3-rapid cytokinetics of the tumor cell; 4-bigger size of the tumor, as well as the preventive treatment of this syndrome are reviewed.
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PMID:[Tumor lysis syndrome at the induction therapy of the first remission in two cases of T-ALL]. 218 70

A case of abdominal Burkitt's lymphoma diagnosed through aspiration cytology is described. This 9-year-old boy presented with abdominal pain and distention for three months accompanied by fever and night sweat during the last month. An abdominal sonography and CT scan showed hepatosplenomegaly and an intrahepatic mass with celiac lymph node enlargement, ascites, and pleural fluid. A peripheral blood smear showed a few blast cells. Aspiration of the abdominal mass revealed very cellular aspirates consisting of diffusely scattered small monotonous round cells. The cells had little cytoplasm, along with round nuclei that showed clear-cut nuclear membrane, coarse chromatin pattern, and multiple small prominent nucleoli. Differential diagnoses considered were small round cell sarcomas such as malignant lymphoma, neuroblastoma, Ewing's sarcoma, and rhabdomyosarcoma. Of these, malignant lymphoma of the small noncleaved cell type was most consistent with the results of several studies including immunohistochemical staining, peripheral blood smear, and bone marrow biopsy. The cells were positive for leukocyte common antigen (LCA) and showed finely vacuolated basophilic cytoplasm in both the peripheral blood smear and bone marrow biopsy, characteristic of Burkitt's lymphoma cells.
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PMID:Abdominal Burkitt's lymphoma diagnosed by fine needle aspiration cytology--a case report. 227 68

Cancer is the second leading cause of death in children and, among cancers, lymphoma is the third most frequently diagnosed type. The majority of these are non-Hodgkin's lymphomas (NHL). Although NHL have been grouped in a variety of confusing ways, recent research in cytogenetics and immunology, coupled with a better understanding of the normal pathways of lymphoid differentiation, has greatly lessened this confusion. Burkitt's lymphoma (BL) is a type of B-cell NHL which is sub-classified as either endemic (African) or non-endemic (American). Kinetic studies have demonstrated a doubling time of only 24 h. As a result of this extremely rapid growth, symptomatic complaints occur early in the disease course. Structures contiguous to the primary become compressed and metabolic derangements occur. Signs of chronic debilitation such as cachexia, diarrhea, generalized lymphadenopathy, and weight loss usually are not found. Thirteen cases of American BL have been treated since 1980 at the Texas Children's Hospital and form the basis of this review. Ten patients had obstructive abdominal symptoms including constipation and pain, and 3 had masses in the neck, maxilla, or tonsil. Systemic manifestations included ascites, jaundice, fever, and hepatosplenomegaly. Two patients ultimately developed central facial nerve paralysis.
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PMID:Burkitt's lymphoma. 381 93

A clinico-pathological study of malignant lymphoma in Jamaica was undertaken to examine the disease pattern in a predominantly negro population of West African origin. During a 9-year period (1958-66) 260 histologically verified cases of malignant lymphoma were encountered. The distribution of the different histological types was as follows: Hodgkin's disease 50.9%, lymphosarcoma 33%, reticulum cell sarcoma 14.2%, giant follicular lymphoma 1.9%. No cases of Burkitt's tumour were encountered.This study indicates that malignant lymphoma is not uncommon in Jamaica, and that its distribution pattern is similar to that observed in Europe and North America, except for the paucity of giant follicular lymphoma, and is different from the pattern observed in parts of Africa populated by Negroes, where Burkitt's tumour is the most common type, and where Hodgkin's disease is relatively uncommon. The age and sex incidence was in general similar to other reported series, but the duration of symptoms was short. The majority of patients presented with generalised peripheral lymphadenopathy. Hepatosplenomegaly and anaemia were common on admission. The prognosis was generally poor in comparison with European and North American series due to advanced stage of disease on presentation.
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PMID:Clinico-pathological study of malignant lymphoma in Jamaica. 491 67

A monoclonal malignant lymphoma was diagnosed in a 30-month-old Japanese boy, who, since the age of 12 months, had had chronic, recurrent infectious mononucleosis manifested by repeated episodes of severe cough and high fever accompanied by marked lymphadenopathy and hepatosplenomegaly and high serum Epstein-Barr virus (EBV) antibody titres. The diagnosis of Burkitt's lymphoma was made when a cervical lymph-node biopsy specimen revealed massive proliferation of immature B-cells with starry-sky histiocytes. These lymph-node cells were characterised by a translocation between chromosomes 10 and 17. There were approximately 9 EBV genome-equivalents per cell. Most cells were positive for nuclear antigen (EBNA); early antigen (EA) and viral capsid antigen (VCA) were also detected. The presence of EBV was supported by finding that the cell-free lymph-node extract transformed cord-blood lymphocytes into EBNA-positive blast cells. The proportion of EA-positive and VCA-positive cells increased rapidly in culture for 24 h, then the positive cells degenerated rapidly and completely. The cells also contained numerous herpes-type virus particles. The child improved considerably with cytostatic treatment and has been in remission for 2 years.
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PMID:Evolution of infectious mononucleosis into Epstein-Barr virus carrying monoclonal malignant lymphoma. 612 20

Four Venezuelan patients with the autosomal recessive Chediak-Higashi syndrome (CHS) were studied. The results confirm the severe reduction in natural killer (NK) cell activity, as previously described and showed also a decline in the activity of cells involved in antibody-dependent cellular cytotoxicity (ADCC). No defect was found in the production of immunoglobulins and of specific antibodies to measles, varicella, herpes simplex, and cytomegalo viruses. Two of the patients had extremely high antibody titers to the Epstein-Barr virus (EBV) specific viral capsid antigen (VCA), to the restricted (R) component of the EBV-induced early antigen complex, and to the EBV-associated nuclear antigen (EBNA). These two patients had enlarged livers, spleens, and lymph nodes indicative of the lymphoproliferative phase. The other two patients were initially negative for all EBV-associated antibodies but seroconverted subsequently and, in the course of a year, also developed high antibody titers to VCA and R. In one of these patients the primary infection was accompanied by moderate signs of infectious mononucleosis (IM) followed after more than 6 months by persistent hepatosplenomegaly. The other patient also developed signs of a lymphoproliferative syndrome with hepatosplenomegaly and jaundice and died 8 months later. Such high anti-R titers are seen frequently in Burkitt's lymphoma, but rarely in other conditions. It is likely that the high antibody titers reflect an increased production of VCA and R due to defective NK and ADCC cell activities so that productively infected B lymphocytes are no longer eliminated before they have synthesized maximal amounts of antigens. The high anti-EBNA titers suggest normal T lymphocyte function. The possibility that the accelerated, lymphoma-like phase of the CHS involves EBV-transformed cells is discussed.
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PMID:Elevated antibody titers to Epstein-Barr virus and low natural killer cell activity in patients with Chediak-Higashi syndrome. 630 71

Twenty five cases of Burkitt's lymphoma in Thai children were diagnosed at the Department of Pediatrics, Siriraj Hospital during the period of 13 years (January 1969 to April 1982). Males were more affected than females with the ratio of 1.7:1. The age ranged from 2 to 11 years with the median age of 4-5 years. The most common clinical manifestations were abdominal mass associated with nausea vomiting, abdominal pain, anorexia, weight loss and generalized lymphadenopathy which occurred in 50-60% of cases. Additional symptoms and signs included anemia, hepatosplenomegaly, edema and pleural effusion. Jaw tumor was found in only 37.5% of the patients. Definite diagnosis depended on the characteristic starry sky appearance of the lymph node biopsy or section of abdominal mass. In advance cases, the tumor cells could be discovered in bone marrow aspiration, ascitic fluid pleural fluid and cerebrospinal fluid. The typical blast cells were detected in the peripheral blood in 4 cases. Antibody to Epstein-Barr virus could be detected in almost all cases with high titers in some cases. Most patients responded very well to local irradiation and chemotherapy with prednisolone plus cyclophosphamide and vincristine or methotrexate. However, relapse occurred rapidly and 80% of the patients died within 3 months after diagnosis with the median survival of only 1 month. Five cases expired early before any specific treatment. The main causes of death were disease, sepsis, excessive bleeding and hyperkalemia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Burkitt's lymphoma in Thai children: an analysis of 25 cases. 631 66

An eight-year-old boy was admitted with a three-week history of painless masses on the left upper eyelid, in front of the left ear and on the left side of his jaw. On examination, there was a 3x2 cm tumor on the left upper eyelid, and lymphadenopathies in front of the tragus and in the left submandibular area were observed in the absence of hepatosplenomegaly. Complete blood count, peripheral smear and bone marrow aspiration were normal. After the left submandibular lymphadenopathy was removed, he was diagnosed with Burkitt's lymphoma by pathological examinations. Thorax and abdomen computed tomography showed pulmonary and renal involvement. On the seventh day of treatment, eyelid involvement disappeared. After induction therapy, no renal or pulmonary lesions were observed. An unusual clinical presentation of Burkitt's lymphoma with eyelid and pulmonary involvement is reported.
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PMID:A Burkitt's lymphoma case with eyelid, renal and pulmonary involvement. 1684 25

Hemophagocytic syndrome is a clinical condition characterized by the infiltration of the bone marrow and reticuloendothelial system by macrophages and activated histiocytes, leading to uncontrolled phagocytosis of platelets, erythrocytes, lymphocytes and precursor cells. It is a severe inflammatory and aggressive condition, characterized by high fever, hepatosplenomegaly, lymphadenopathy and cytopenia, and it may lead to organ dysfunction. This syndrome is classified as familial or acquired; the latter is more frequent and is associated with diverse conditions, such as infections, malignancies and rheumatic diseases. We report a case of HLH associated with cytomegalovirus infection in a patient with acquired-immunodeficiency syndrome and Burkitt's lymphoma.
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PMID:Hemophagocytic syndrome associated with cytomegalovirus infection in a severely immunocompromised AIDS patient: case report. 1957 35

Infectious mononucleosis (IM) is one of the representative, usually benign, acute diseases associated with primary Epstein-Barr virus (EBV) infection. IM is generally self-limiting and is characterized mostly by transient fever, lymphadenopathy and hepatosplenomegaly. However, very rarely primary EBV infection results in severe or fatal conditions such as hemophagocytic lymphohistiocytosis together with fulminant hepatitis designated as severe or fatal IM or EBV-associated hemophagocytic lymphohistiocytosis alone. In addition, chronic EBV-associated diseases include Burkitt's lymphoma, undifferentiated nasopharyngeal carcinoma, Hodgkin lymphoma, T-cell lymphoproliferative disorder (LPD)/lymphoma, natural killer-cell LPD including leukemia or lymphoma, gastric carcinoma, pyothorax-associated lymphoma and senile B-cell LPD as well as chronic active EBV infection and LPD/lymphoma in patients with immunodeficiency. The number of chronic life-threatening diseases linked to the EBV infection is increasingly reported and many of these diseases have a poor prognosis. This review will focus on the historical, pathogenetic, diagnostic, therapeutic and prophylactic issues of EBV-associated life-threatening diseases.
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PMID:Acute or chronic life-threatening diseases associated with Epstein-Barr virus infection. 2210 26


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