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 present a case of acute monocytic leukemia presenting with extramedullary disease clinically resembling lymphoma. A 36 year-old man presented with arthralgia and was found to have skin eruption, nasopharyngeal mass, hepatosplenomegaly and superficial lymphadenopathy. The biopsies of those lesions as well as bone marrow revealed infiltration of CD45 positive large atypical cells and were interpreted as malignant lymphoma. At that time blood film showed leuko-erythroblastic picture, but no atypical cells were noted. He was treated with CHOP followed by VEPA chemotherapy and achieved partial remission. Four months later headache and double vision occurred with the same atypical cell in the cerebrospinal fluid. The cells were identified as monoblasts by cytochemistry and immunophenotype. The diagnosis of aleukemic acute monocytic leukemia was made and its CNS involvement was successfully treated with chemotherapy with DHAP and intrathecal MTX. However, two months later, blasts finally appeared in the peripheral blood and the patient died of multiorgan failure eight months after presentation. The blasts were positive for T cell markers (CD2, 4, 8) and NK marker (NKH-1) as well as monocytic markers. This finding, together with those by other investigators, may indicate the association between these immunophenotypes and extramedullary manifestations of acute monocytic leukemia.
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PMID:[Extramedullary diseases as presenting features of aleukemic acute monocytic leukemia]. 146 83

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 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

Within a few years of the introduction of effective systemic chemotherapy for the treatment of acute leukemia, central nervous system (CNS) leukemia emerged as a significant problem. The strategy for treatment of leukemia is that one achieves cure by destruction of all leukemic cells, through this cannot be verified with present technology. The incidence of CNS-leukemia and the factors influencing this complication have been studied in a group of 131 cases with acute childhood leukemia. The overall incidence of CNS-leukemia was 30% in acute lymphocytic leukemia (ALL), 8% in acute myeloblastic leukemia (AML). However, cumulative incidence of CNS-leukemia calculated by modified Life-Table Method showed 56.8% in children who survived over 48 months after diagnosis of ALL. It was considered that the chief cause of the increased incidence of this complication was prolongation of survival. The CNS-leukemia was significantly greater in patients with under 10 year of age (p greater than 0.05), high initial leukemic cell counts (p less than 0.025), high appearance rate of leukemic cells in peripheral blood (over 50%) (p less than 0.005), and hepatosplenomegaly, especially lymph node enlargement (p less than 0.05). Blood-borne metastasis was considered to be probably the chief route of leukemic infiltration to the CNS. Between 1972 and 1978, 153 children with ALL were treated with multiple methods of CNS-prophylaxis, and were analyzed in relation to treatment regimens, age, sex and initial hematologic status. Patients received CNS-prophylaxis; Group I: three doses of intrathecal methotrexate (MTX 12 mg/m2) and hydrocortisone (HDC 12 mg/m2), Group II: same as in Group I followed by cyclic MTX and HDC, Group III: same as in Group I plus 2,400 rads of cranial irradiation. CNS-leukemia terminated complete remission 25 of 153 patients (16.3%). The cumulative incidence of CNS-leukemia at 4 year calculated by the Kaplan-Meier Method was 40.5% in Group I, 26.9% in Group II, and 14.5% in Group III. Development of CNS-leukemia was more frequent in male than in female (p less than 0.05), and in patients with initial high leukocyte counts (WBC greater than 25,000/mm3). We conclude that the combination of cranial irradiation and intrathecal MTX and HDC was highly efficacious. However, more intensive CNS-prophylaxis should be needed for such a high risk patients in male with high initial WBC.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Acute leukemia]. 647 26

40 children (23 males, 17 females) have been diagnosed have ANLL during the period from february 1970 to september 1981. According to FAB classification, 24 cases were M1,-M2, 9 M3, 3 M4, 3 M5 and 1 M6. At diagnosis, 20 patients (50%) had leukocytes less than 10.000/mmc, 6 (15%) had leukocytes greater than 50.000mmc. Hb levels was 7 g% in 16 patients (40%); 10 children had hepatosplenomegaly (25%), 7 splenomegaly (18%) and 5 lymphoadenomegaly (13%). 4 patients had cutaneous or mucous infiltrates. None had meningeal involvement at diagnosis. According to the year of diagnosis, 3 groups can be identified. In the group I (1970-73), 11 patients have been treated with not codified combination chemotherapy as ARA-C, 6-TG, DNR, CTX, Metil-GAG. In the group II (1974-76) and in the group III (1977-81), the patients (respectively 12 and 17) have been treated according to the following protocols: LAM-5 (3), TRAP (5), COAP (1), LAM 80 (2), AIL 7402 (8), AIL 7604, AIL 7801 (6). Immunotherapy has been performed in 7 cases. CNS prophylaxis (MTX i.t. +/-ARA-C +/- RT) was given in 5 patients of group II and in 6 of group III. I patients of group I (45%), 6 of group II (50%) and 13 of group III (76%) achieved CR. Median duration of remission was 5 months in the group I and in 17 in group II and III.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Acute non-lymphatic leukemia in children]. 654 20

A 36-year-old woman was referred to our hospital because of leukocytosis in June 2000, and was admitted to our hospital and diagnosed as having adult T-cell leukemia/lymphoma (ATL; acute type). Complete remission was achieved with eight courses of CHOP therapy, but ATL relapsed and she was readmitted to our hospital in September 2001. Laboratory examination showed elevated levels of serum LDH and soluble IL-2 receptor, and hypercalcemia. CT examinations showed swelling of the abdominal lymph nodes and hepatosplenomegaly. CHOP therapy improved the symptoms, but recrudescence soon occurred. After two courses of salvage therapy which resulted in no remission, the patient received an allogeneic peripheral blood stem cell transplant (allo-PBSCT) from her HLA-matched sibling donor after preconditioning with BU + CY in January 31, 2002. Cyclosporin A (CsA) and short-term MTX were used to prevent GVHD. Bone marrow engraftment was prompt and acute GVHD was not found. Two months later, recurrence was seen in the form of subcutaneous tumors, but the tumors spontaneously disappeared following CsA withdrawal. At the time of writing, eight months after the transplant, remission has been maintained. A graft-versus-leukemia (GVL) effect may have been the curative action in this case.
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PMID:[Cyclosporin A withdrawal causes spontaneous remission of recurrent subcutaneous tumors after allogeneic peripheral blood stem cell transplantation for adult T-cell leukemia/lymphoma]. 1269 82

A 47-year-old woman was admitted to our hospital complaining of persistent fever and dry cough in June 2007. CT scan showed hepatosplenomegaly. Laboratory data revealed pancytopenia and increased levels of LDH and soluble interleukin-2 receptor. Malignant lymphoma was suspected, but histological diagnosis was difficult because superficial lymph nodes could not be palpated. Histological examination of the bone marrow biopsy specimen demonstrated the proliferation of large atypical lymphoid cells positive for CD20 and CD79a in the small capillaries, leading to the diagnosis of intravascular large B-cell lymphoma (IVLBCL). Although the results of neurological examination and CSF analysis were normal, head MRI showed a T2-hyperintense lesion in the pons. We chose R-hyper-CVAD/R-MTX-Ara-C alternating therapy with MTX intrathecal injection because CNS involvement in IVLBCL was highly suspected, and she responded well. Head MRI showed the disappearance of the abnormal signal in the pons after one cycle of R-hyper-CVAD. Five cycles of R-hyper-CVAD/R-MTX-Ara-C were performed and complete remission was obtained. R-hyper-CVAD/R-MTX-Ara-C alternating therapy was effective in an IVLBCL patient with CNS involvement.
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PMID:[Intravascular large B-cell lymphoma with pontine involvement successfully treated with R-hyper-CVAD/R-MTX-Ara-C regimen]. 2037 8

A Japanese man aged 30 years old contracted acute hepatitis B in October 2011, and was cured following conservative treatment. Mild hepatosplenomegaly was the only positive finding on computed tomography (CT) and ultrasonography at that time. In May 2012, slight impairment of the liver function was detected again in the patient; an abdominal CT at this time revealed a tumor mass in the right hepatic lobe, so subsegmentectomy of the right hepatic lobe was performed. On the basis of the findings of the resected specimen, primary hepatic circumscribed Burkitt's lymphoma (sporadic form), stage IA, was diagnosed. Multiple cycles of hyper-CVAD/MTX-Ara-C therapy with concomitant rituximab were administered, under which the patient was successfully maintained in complete remission. To date, at least 15 cases of primary hepatic Burkitt's lymphoma have been reported in the literature; all of the 11 patients without concurrent human immunodeficiency virus (HIV) infection had the sporadic form of the disease. Asians were relatively common (7 patients) among these patients, and patients in their childhood or adolescence accounted for a considerable proportion. Therefore, the present case may be regarded as rather typical. The presence of hepatitis virus infection as a background disorder other than HIV is considered to be of profound interest etiologically.
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PMID:Primary hepatic circumscribed Burkitt's lymphoma that developed after acute hepatitis B: report of a case with a review of the literature. 2399 15