Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical findings and prognosis in 15 patients with primary Ki-1 anaplastic large cell lymphoma (ALCL) were analyzed and compared with those of patients with T cell and B cell lymphoma and Hodgkin's disease. Clinical data revealed lymphadenopathy in 13 patients (87%) and skin lesions in eight (53%). Other organic involvements were hepatomegaly in two patients (13%), splenomegaly in five (33%), and bone marrow involvement in three (20%). The rate of skin involvement was significantly higher than that in B cell lymphoma and Hodgkin's disease. In laboratory findings the gamma-globulin concentration was significantly higher than that in T cell lymphoma, and the erythrocyte sedimentation rate (ESR) was significantly higher than that in B cell lymphoma. Complete remission was achieved in 11 patients (73%) and the five-year relapse-free survival was 27%. The overall survival was 4.0-69.8 months (mean 30.6 months). The mean survival was compatible with that of T cell lymphoma and was significantly shorter than that in Hodgkin's disease. Ki-1 ALCL can be distinguished from other lymphomas clinically as well as pathologically. Because Ki-1 ALCL is chemosensitive and the prognosis is as poor as that of T cell lymphoma, aggressive chemotherapy should be employed for the treatment of this disease.
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PMID:Clinicopathological characteristics of primary Ki-1 anaplastic large cell lymphoma. 871 76

A case is presented of CD30+ anaplastic large cell lymphoma of the spleen. The patient, a 61 year old woman with a history of chronic lymphocytic leukemia (CLL) was seen for the sudden development of splenomegaly with thrombocytopenia. A splenectomy was performed which showed massive replacement of the spleen by a population of large atypical lymphoid cells showing bizarre nuclear forms and multinucleated tumor cells reminiscent of Reed-Sternberg cells. Immunohistochemical studies showed strong membrane and dot-like paranuclear positivity in the majority of the atypical cells for CD30, with coexpression in many of the cells for CD15. Additionally, the cells also strongly reacted with CD3, UCHL-1, EMA and LCA. The present case illustrates an unusual variant of anaplastic (CD30+) large cell lymphoma sharing histologic and immunophenotypic features that overlap with those of Hodgkin's disease. The history in this patient of CLL with sudden development of splenomegaly raises the possibility of transformation of CLL into a high-grade lymphoma (Richter's syndrome). The possible pathogenetic implications of this phenomenon are discussed.
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PMID:Anaplastic large cell lymphoma of the spleen. 972 68

We report a retrospective immunohistochemical study on bone marrow biopsies of 43 patients with different types of lymphomas showing unusual intrasinusoidal infiltration. Most of these patients presented with splenomegaly (74.4%) and peripheral lymphocytosis (83%). In 20/43 patients, lymphoid infiltrates were not detectable on haematoxylin-eosin sections. After immunohistochemistry on bone marrow biopsies and blood and bone marrow smear examinations, the following diagnoses were made: splenic marginal zone lymphoma with villous lymphocytes (SLVL) in 24 patients, large granular lymphocyte (LGL) leukaemia in 14 patients, hepatosplenic T-cell lymphoma in two patients, anaplastic large cell lymphoma in two patients and intravascular large B-cell lymphoma in one patient. In the presence of intrasinusoidal infiltrates of small lymphocytes, a B-cell phenotype (CD20+, CD76/DBA44+/-) was associated with splenic marginal zone lymphoma whereas intrasinusoidal CD3/CD45RA-positive T-cell infiltrates were strongly suggestive of LGL leukaemia. Intrasinusoidal bone marrow infiltration appears to be a common feature of distinct lymphoma subtypes. Immunohistochemical analysis is essential to detect intrasinusoidal medullary infiltrates (which may be minimal) and should be systematically performed in patients with splenomegaly and peripheral lymphocytosis.
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PMID:Intrasinusoidal bone marrow infiltration: a common growth pattern for different lymphoma subtypes. 1247 68

A 33-years-old man was diagnosed as having undifferentiated carcinoma presenting with right neck lymphadenopathy in December 2000. He obtained complete remission (CR) following chemotherapy, radiation and lymphadenectomy on the right neck. He had multiple para-aorta lymphadenopathy and splenomegaly in December 2001. An open-abdominal lymph node biopsy was performed from which a diagnosis of anaplastic large cell lymphoma was made. CR was achieved with biweekly CHOP, however, the patient suffered from a relapse twice. He underwent allogeneic peripheral blood stem cell transplantation (PBSCT) from his HLA-matched sister while in non-CR in November 2002. Engraftment was achieved on day 14, and at the same time, complete chimerism was confirmed. Acute grade III graft-versus-host disease (GVHD) developed and was controlled with cyclosporine A and prednisolone. Extensive chronic GVHD was subsequently observed and required systemic immunosuppression. His condition returned to CR after the PBSCT and he sustained complete chimerism. He suddenly died of fulminant thrombotic microangiopathy seven months after the PBSCT. The tumor cells were ALK-positive, CD30-negative and JH rearrangement was detected, and were therefore classified as diffuse large B-cell lymphoma with expression of ALK according to the WHO classification, though they differed from this subtype in some points. Although this case was refractory for chemotherapy with a complex karyotype, the graft-versus-lymphoma effect might have contributed to the sustained CR following the PBSCT.
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PMID:[CD30-negative diffuse large B-cell lymphoma expressing ALK]. 1644 Jul 42

We describe a 47-year-old male admitted with fever and extreme neutrophil granulocytosis (up to 80 x 10(9)/L). All microbiology tests and test for autoimmune disease were negative. CT scan showed pulmonary infiltrates bilaterally, mediastinal lymphadenopathy and splenomegaly. Conventional pathological examination of bone marrow and lymph node biopsies did not demonstrate malignant cells and inflammatory disease was suspected. The patient died of multiorgan failure 23 days after admission. Autopsy showed neutrophil infiltration of several organs. Immunohistochemistry and cytogenetics postmortem led to a diagnosis of anaplastic large cell lymphoma (ALCL) of T-cell lineage. Involvement of peripheral blood with leukemoid reaction is a rare manifestation of ALCL. This case emphasizes the importance of immunophenotyping in unexplained extreme granulocytosis.
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PMID:Extreme neutrophil granulocytosis in a patient with anaplastic large cell lymphoma of T-cell lineage. 1755 Mar 90

Anaplastic large cell lymphoma (ALCL) is a well-known entity, but there are no data on prognosis according to the age of the patient, especially in infants. A 2-month-old girl was admitted with a 2-week history of coughing, fever, and lymphadenopathy. Physical examination revealed mild respiratory distress, an erythematous macular rash on her trunk, massive cervical lymphadenopathy, splenomegaly, and very mild ascites. Chest radiograph showed bilateral pulmonary infiltrates, pleural effusion, and a mediastinal mass. CBC count showed WBC: 172,000/microL (PMN 40%, lymphocytes 47%, monocytes 3%); hemoglobin concentration: 8.7 g/dL; platelets: 390,000/microL. Cervical lymph node biopsy revealed anaplastic lymphoma with positive staining to ALK 1 and TIA 1. Immunophenotypic analysis of peripheral and bone marrow lymphoid cells showed an aberrant T-cell immunophenotype, including expression of CD3, CD45R0+, CD43+, and CD30+. Cytogenetic analysis performed on blood and bone marrow samples demonstrated the translocation t(2;5) (p23;q35), and trisomy 47. After leucophoresis, the child received chemotherapy according to the ALCL-99-EICNHL protocol, and was started on corticosteroids and cyclophosphamide, which resulted in marked improvement. After the second course, WBC decreased to 6000/microL without tumor lysis syndrome, but the child developed bacterial and fungal disseminated infections and died of septic shock with multiorgan failure. This report is of a rare case of infant anaplastic lymphoma and excellent response to treatment. Unfortunately, she did succumb to overwhelming infection. More reports of similar cases may determine the cause and prognosis of such children, helping to tailor therapy according to the age of the child and other prognostic factors, especially bone marrow involvement.
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PMID:Infant anaplastic lymphoma: case report and review of the literature. 1761 84

We present a case of a 60-year-old male patient with primary bone marrow anaplastic large cell lymphoma. He was admitted to the hospital with the symptoms of anemia and fever. There was no evidence of lymphadenopathy or splenomegaly. Immunoelectrophoresis showed the presence of a triple M gradient (double IgM and an IgG), with the IgG and one of the IgM paraproteins functioning as a cryoglobulin. The patient had no hepatitis C virus infection. Bone marrow biopsy showed massive CD30-positive, ALK-negative large lymphoid cell infiltration of T-cell origin with anaplastic morphology. PCR analysis of lymphoid cells separated from the bone marrow demonstrated the presence of a B/T hybrid genotype disorder with no evidence of the t(2;5), nor t(1;2) translocations. The patient entered a period of remission following CHOP chemotherapy. The patient subsequently died of sepsis as a consequence of serious humoral immunodeficiency.
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PMID:Primary bone marrow T-cell anaplastic large cell lymphoma with triple M gradient. 1792 57

Hemophagocytic syndrome (HS) is a life-threatening condition of hyperinflammation. Main symptoms are: prolonged fever, cytopenia, hepatosplenomegaly, hemophagocytosis, hyperferritinemia, hypertriglyceridemia and hypofibrinogenemia. Primary genetic form and secondary HS associated with infections, malignancies or autoimmune disorders can be distinguished. Untreated HS in most cases leads to death. We analyzed retrospectively 7 cases of HS in children (3 girls, 4 boys; aged 10 days -14 years) treated in 3 different pediatric centers from 2004 to 2009. In 3 cases HS was associated with infections (EBV, CMV, Bacillus Calmette Guerin - BCG), in 1 child with non-Hodgkin anaplastic large cell lymphoma (ALCL), in 1 patients probably with side effect of antiepileptic drug. In 2 cases cause of HS remained unknown. Fever, hepatomegaly, pan- or bicytopenia and hyperferritinemia were present in all children. In addition, splenomegaly was noted in 6 cases, hemophagocytosis in 6 children, impaired function or decreased number of NK cells in 4 cases, hypofibrino-genemia in 5 and hypotriglyceridemia in 4 patients. Among other symptoms and signs we observed: lymphadenopathy, hepatic failure, oedema, rash, neurological symptoms, increased level of LDH and inflammatory markers. In one child acute pancreatitis occurred. Among others, antibiotics, antiviral and immunosuppressive drugs were used in therapy. HLH-2004 protocol was applied in 4 cases. Patient with ALCL was treated with chemotherapy and allogeneic stem cell transplantation. Four patients are alive, 2 died because of HS, child with ALCL died because of generalized infection in peritrans-plantation period. In case of prolonged fever, splenomegaly and cytopenia diagnosis of HS should be considered. Following tests are recommended: complete blood count, ferritin, triglycerides, fibrinogen, bone marrow aspiration and NK cell assessment. Patients should be also screened for infections and malignancies. Early diagnosis of HS and underlying condition is crucial to start lifesaving therapy.
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PMID:[Hemophagocytic syndrome in children with different underlying conditions]. 2134 76