1/34. Haemophagocytosis in early congenital syphilis.A previously healthy male infant developed hepatosplenomegaly, severe anaemia and thrombocytopenia 5 weeks after birth. Marked haemophagocytosis was present in the bone marrow. A typical maculopapular rash suggested early congenital syphilis. The diagnosis was confirmed by serology and by the presence of untreated syphilis in both parents. CONCLUSION: syphilis needs to be excluded in infants suspected of haemophagocytic lymphohistiocytosis.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/34. Isoimmune haemolysis in pathogenesis of anaemia after cardiac surgery.A patient who had received multiple transfusions developed antiglobulin-positive haemolytic anaemia due to a delayed haemolytic transfusion reaction. Many cases of haemolytic anaemia after cardiac surgery could be explained on this basis.- - - - - - - - - - ranking = 6keywords = anaemia (Clic here for more details about this article) |
3/34. Hemoglobin Moabit: alpha 86 (F7) Leu leads to Arg: a new unstable abnormal hemoglobin.A new alpha chain abnormal hemoglobin variant was found in a Turkish patient with a mild Heinz body hemolytic anemia and splenomegaly. The substitution alpha 86 Leu leads to Arg, which is next to the heme binding proximal histidine, is responsible for a marked instability of the molecule. The oxygen affinity of the erythrocytes was found to be slightly decreased.- - - - - - - - - - ranking = 1.297247809676keywords = hemolytic (Clic here for more details about this article) |
4/34. mycobacterium genavense infection in a patient with long-standing chronic lymphocytic leukaemia.We describe the first case of disseminated infection with mycobacterium genavense in an hiv-seronegative patient with a chronic haematological disorder. Our patient, an 80-year-old woman, had been under long-term treatment with chlorambucil (partially in combination with prednisone) for B-cell chronic lymphocytic leukaemia (B-CLL). When she developed general fatigue and progressive anaemia, as well as progressive lymphadenopathy and splenomegaly, bone marrow biopsy revealed granulomas with acid-fast bacilli, and cultures of both bone marrow and blood grew M. genavense. The patient's CD4 cell count was approximately 100 microL(-1). Treatment with clarithromycin, ethambutol and rifabutin resulted in improvement of anaemia and general health as well as in regression of lymphadenopathy and splenomegaly.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
5/34. Fetal splenic rupture following transfusion.BACKGROUND: splenic rupture in the newborn is a rare complication in erythroblastosis fetalis. There are no reports of splenic rupture in the fetus affected by hemolytic disease of the newborn. CASE: A 41-year-old gravida 3, para 2-0-0-2 with severe rhesus alloimmunization was managed with serial intrauterine transfusions resulting in fetal death after the fourth procedure. autopsy findings revealed intra-abdominal clotted blood and splenic capsular defects consistent with splenic rupture. CONCLUSION: Fetal splenic rupture might occur in hemolytic disease of the newborn associated with splenomegaly. Acute hemodynamic changes with increased intra-abdominal pressure from intrauterine transfusion might precipitate splenic rupture. (Obstet Gynecol 2001;97:824-5.- - - - - - - - - - ranking = 2.5944956193521keywords = hemolytic (Clic here for more details about this article) |
6/34. splenosis in a port site after laparoscopic splenectomy.splenosis, the autotransplantation of splenic tissue, is most commonly seen after traumatic splenic rupture and splenectomy. It also can occur during embryonic development. Intraperitoneal, intrathoracic, and retroperitoneal sites have been reported. Although the presence of the splenic tissue often is asymptomatic and an incidental finding, it may present with pain or be confused with various pathologies including neoplasia. Because most pediatric splenectomies are performed for hemolytic disorders, parenchymal disruption must be contained to avoid recurrent disease. We present a case in which the devascularized spleen was contained in a bag and fragmented in situ. splenosis developed in the retrieval port site after laparoscopic splenectomy and cholecystectomy. Port-site splenosis needs to be considered in the differential diagnosis of port-site pain and a palpable nodule postsplenectomy.- - - - - - - - - - ranking = 1.297247809676keywords = hemolytic (Clic here for more details about this article) |
7/34. Gamna-Gandy bodies of the spleen detected with MR imaging: a case report.We report a case of a female patient with portal hypertension due to liver cirrhosis. In this case, MR imaging revealed small siderotic nodules of the spleen, called Gamna-Gandy bodies. These lesions are found in patients with portal vein or splenic vein thrombosis, hemolytic anemia, leukemia, or lymphoma, patients receiving blood transfusions, acquired hemochromatosis, or paroxysmal nocturnal hemoglobinuria. There are only few reports in the literature about these siderotic nodules which are not very familiar. MR imaging seems to be the superior imaging method for detection of these lesions. It is important to consider Gamna-Gandy bodies in the differential diagnosis of portal hypertension and the other diseases mentioned above.- - - - - - - - - - ranking = 1.297247809676keywords = hemolytic (Clic here for more details about this article) |
8/34. 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 = 5.1889912387042keywords = hemolytic (Clic here for more details about this article) |
9/34. splenectomy in a case of splenic vein thrombosis unmasks essential thrombocythemia.We report a patient with splenic vein thrombosis (SVT) in whom splenectomy resulted in the unmasking of essential thrombocythemia (ET). He had portal hypertension with haematemesis, resulting in anaemia requiring repeated blood transfusions. Investigations revealed SVT. Following splenectomy, he suffered a transient ischaemic attack episode, associated with persistent thrombocytosis (> 2000 x 10(9)/l). Other myeloproliferative disorders were excluded and a diagnosis of ET was established. He responded to hydroxyurea but, due to financial constraints, he discontinued treatment and subsequently relapsed. The association of ET with SVT is rare and the diagnosis of ET was missed initially as the platelet count was normal prior to splenectomy.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
10/34. 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 = 1.297247809676keywords = hemolytic (Clic here for more details about this article) |
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