1/12. Hepatosplenic gammadelta T-cell lymphoma presenting with immune-mediated thrombocytopenia and hemolytic anemia (Evans' syndrome).We describe an unusual case of hepatosplenic T-cell lymphoma in a 61-year-old man who presented with fever, hepatosplenomegaly, anemia, and thrombocytopenia. A spleen biopsy was consistent with T-cell lymphoma. Cytogenetic studies did not reveal chromosome abnormalities. Using the polymerase chain reaction approach, clonality of the T-cell receptor gamma-chain gene rearrangement could be demonstrated, while Southern blot analysis disclosed only a germline configuration of the T-cell receptor beta chain genes. Of interest, an immune-mediated mechanism was demonstrated and was most likely responsible for erythrocyte and platelet destruction; this is, therefore, the first report of gamma T-cell lymphoma in association with Evans' syndrome. Initial steroid treatment was efficacious in limiting autoimmunity but constitutional symptoms did not subside. Chemotherapy (MACOP-B) was successful in obtaining complete clinical remission. Finally, thrombocytopenia in gammadelta T-cell lymphoma patients should be routinely evaluated for platelet autoantibodies.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
2/12. Hyporegenerative anemia associated with Rh hemolytic disease: treatment failure of recombinant erythropoietin.A postnatal hyporegenerative anemia may complicate Rh hemolytic disease. Intramedullary hemolysis, bone marrow suppression, and erythropoietin deficiency have been implicated etiologically. Treatment with recombinant erythropoietin (r-EPO) has yielded encouraging preliminary results. The authors describe an infant with rh isoimmunization who developed severe hyporegenerative anemia unresponsive to a 5-week course of r-EPO. Two additional doses at 12 weeks resulted in brisk reticulocytosis, coinciding with a 16-fold decline in the anti-Rh(D) antibody titer. Thus, treatment with r-EPO may be ineffective when anti-Rh(D) antibody titers are high. The authors also show that erythropoietin deficiency in hyporegenerative anemia is not as frequent and severe as originally thought.- - - - - - - - - - ranking = 1.25keywords = hemolytic (Clic here for more details about this article) |
3/12. autoimmune lymphoproliferative syndrome: report of two cases and review of the literature.autoimmune lymphoproliferative syndrome (ALPS) is a rare disease occurring in childhood. Recently, it has been shown that heritable mutations in Fas or Fas ligand genes, which regulate lymphocyte survival by triggering apoptosis of lymphocytes, are the most frequent cause of ALPS. patients with ALPS frequently have lymphadenopathy, splenomegaly and hepatomegaly, especially at young ages. A positive result of the Direct Coomb's test, autoimmune hemolytic anemia, and idiopathic thrombocytopenic purpura are the most common features of autoimmunity in patients with ALPS. Elevated numbers and percentages (>1%) of double-negative (CD4-CD8-) T cells, and characteristic pathologic findings in lymph nodes or spleen are other important diagnostic features. In this report, we present the clinical, immunologic, and pathologic features of two children who were diagnosed with ALPS. The early recognition of ALPS in children with enlarged lymph nodes, hepatosplenomegaly, and autoimmune hematologic features has important diagnostic and prognostic value in avoiding expensive and time-consuming studies and unnecessary treatments. The ratio of CD4-CD8- T cells, immunoglobulin levels and the histopathologic features of lymph nodes should be rapidly determined in these patients in order to establish an early diagnosis and treatment.- - - - - - - - - - ranking = 0.25keywords = hemolytic (Clic here for more details about this article) |
4/12. Unusual and severe disease course in a child with ataxia-telangiectasia.Ataxia-telangiectasia (AT) is an autosomal recessive syndrome of combined immunodeficiency. Hallmarks of the disease comprise progressive cerebellar ataxia, oculocutaneous telangiectasia, cancer susceptibility and variable humoral and cellular immunodeficiency. We describe a patient with AT presenting with autoimmune haemolytic anaemia, neutropenia, hepatosplenomegaly, lymphadenopathy and hyper-IgM at the age of 6 months. At the age of 26 months she developed persistent fever, progressive lymphadenopathy and pulmonary nodular infiltrates, which were responsive to steroid therapy.- - - - - - - - - - ranking = 0.36561539609938keywords = anaemia (Clic here for more details about this article) |
5/12. Recurrent ascites in an infant with perinatally acquired cytomegalovirus infection.We describe an infant with symptomatic perinatally-acquired cytomegalovirus (CMV) infection manifested by fever, anaemia, thrombocytopenia and hepatosplenomegaly. This infant developed recurrent episodes of severe ascites during which the virus was isolated from his urine. This rare hepatic manifestation of neonatal CMV infection has, to the best of our knowledge, only been reported twice in aborted fetuses with intrauterine systemic CMV infection.- - - - - - - - - - ranking = 0.36561539609938keywords = anaemia (Clic here for more details about this article) |
6/12. Clinical features of hepatic sequestration in sickle cell anaemia.Episodes of hepatic sequestration were seen in four patients with sickle cell anaemia (SCA). One case presented a severe and fatal sequestration crisis whereas the others showed mild episodes associated with less striking haematological and clinical changes. These clinical pictures are similar to those seen in the splenic sequestration crises of children with SCA. Different causes of liver enlargement in SCA suggest that the clinical spectrum representing intrahepatic trapping of blood could range from the acute sequestration crisis to chronic sequestration events which very probably should play any role in the pathogenesis of the hepatomegaly frequently found in these patients.- - - - - - - - - - ranking = 1.8280769804969keywords = anaemia (Clic here for more details about this article) |
7/12. An unusual case of pycnodysostosis.A 6 year old boy with clinical and radiological features of pycnodysostosis is described. In addition to pycnodysostosis he had a myelophthisic type of anaemia suggesting an overlap with osteopetrosis.- - - - - - - - - - ranking = 0.36561539609938keywords = anaemia (Clic here for more details about this article) |
8/12. liver damage in the hemolytic uremic syndrome.During the recovery stage of the hemolytic uremic syndrome in 2 cases an increase of serum levels of GOT, GPT, LDH, gammaGT, 5'ND and AP was noticed, without signs of a recurrence of the disease. In one patient also jaundice and hepatomegaly were found. The observations suggest a parenchymal damage of the liver.- - - - - - - - - - ranking = 1.25keywords = hemolytic (Clic here for more details about this article) |
9/12. Hepatic sequestration in sickle cell anaemia.Several episodes of acute hepatic enlargement associated with a dramatic fall in haemoglobin concentration were observed in two patients with sickle cell anaemia. No appreciable disturbances of liver function or signs of cardiac failure were evident. The most likely mechanism was sequestration of sickled erythrocytes in the liver. This complication, which may have a basis similar to that of splenic sequestration and the sickle lung syndrome, may be easily overlooked unless the size of the liver is regularly monitored in patients with sickle cell crisis.- - - - - - - - - - ranking = 1.8280769804969keywords = anaemia (Clic here for more details about this article) |
10/12. Hb Leiden-beta (0) thalassemia in a Chinese with severe hemolytic anemia.The first case of Hb Leiden (alpha2beta2 6 or 7 Glu |
O)-beta (0) thalassemia in a young patient with chronic severe hemolytic anemia, which improved after splenectomy, is described. His parents were Chinese. The patient's blood showed no Hb A or normal beta chains when no blood transfusion was given. His mother was heterozygous for beta(0) thalassemia, and his father and brother had a trait for the unstable Hb Leiden. The Hb Leiden level of the father was 22.6% and that of the brother was 19.3%. It is probable that the abnormal hemoglobin in this Chinese family resulted from an independent gene mutation, unrelated to the one found in 2 Caucasian families reported earlier. |
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