1/21. Hepatosplenic T-cell lymphoma of alphabeta lineage in a 16-year-old boy presenting with hemolytic anemia and thrombocytopenia.The authors report an unusual case of peripheral T-cell lymphoma in a 16-year-old boy who presented initially with jaundice, splenomegaly, anemia, and thrombocytopenia. A lymphoma was found subsequently in the spleen, which was infiltrated extensively in the red pulp by medium-sized, blastic-appearing lymphoma cells. Immunologic characterization of these cells revealed positivity for CD3, CD5, CD45RO, CD56, and T-cell intracellular antigen (TIA), and negativity for CD2, CD3, CD4, CD8, CD57, CD34, and terminal deoxynucleotidyl transferase (TdT). Conventional cytogenetic studies revealed the presence of isochromosome 7q. On follow up, this patient deteriorated rapidly, with evidence of liver and bone marrow involvement. Although the overall clinical and pathologic features of this disease were characteristic of hepatosplenic gammadelta T-cell lymphoma, the T-cell receptor of this tumor showed an immunophenotype of alphabeta not gammadelta lineage. Using the Southern blot technique, the authors demonstrated monoclonal gene rearrangement of the T-cell receptor beta-chain. Thus, they confirmed the existence of hepatosplenic alphabeta T-cell lymphoma. In view of its overall similarity to hepatosplenic gammadelta T-cell lymphoma, this unusual entity probably represents a slight biologic variation of the same disease.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
2/21. Clostridial sepsis: is death avoidable?Massive intravascular hemolysis is a rare yet often fatal complication of clostridial sepsis. The only chance for survival is an early diagnosis and prompt initiation of treatment. We report a rapidly fatal case who developed electrocardiographic changes of acute myocardial injury. autopsy showed gas-filled bubbles and cysts in the myocardium partially filled with sporulating bacilli with the morphology of clostridia. Gas filled bubbles were also present in the lungs, liver, kidneys and spleen. The gastric mucosa showed hemorrhagic and necrotizing changes, the probable site of entry of the infection.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
3/21. Intravenous anti-D immune globulin-induced intravascular hemolysis in Epstein-Barr virus-related thrombocytopenia.RhoD immune globulin intravenous (anti-D IGIV) increases platelet counts in patients who have not undergone splenectomy and are positive for RhoD with idiopathic thrombocytopenic purpura. After treatment, in most patients, anemia develops as a result of immune-mediated red cell destruction in the spleen. Although intravascular hemolysis (IVH) is not expected, life-threatening IVH has been recently reported by the food and Drug Administration, and physicians are encouraged to report their experience with patients with idiopathic thrombocytopenic purpura in whom IVH develops after anti-D administration. Severe IVH was observed after treatment with anti-D IGIV in two adolescent girls with acute thrombocytopenia related to Epstein-Barr virus. They did not have hemolytic anemia before treatment. The authors believe that anti-D IGIV triggered an unusual virus-induced immune response causing hemolysis; therefore, anti-D IGIV should not be used in patients with Epstein-Barr virus-related thrombocytopenia, particularly during the acute phase of infection.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
4/21. 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 = spleen (Clic here for more details about this article) |
5/21. Sickle cell anemia and hematological neoplasias.Sickle cell disease (SCD) is a congenital hemolytic anemia with various clinical findings. In some cases hematological neoplasias and some solid tumors may accompany this disease but these have rarely been reported. Here we report five cases with SCD and accompanying hematological neoplasias including lymphoblastic lymphoma, multiple myeloma and hairy cell leukemia in four cases with sickle cell trait and one case of Hodgkin' disease in sickle cell anemia. All of the cases except one had no previously diagnosed congenital hemolytic anemia and/or family history. Peripheral blood findings suggestive for an underlying hemolytic anemia were the first step and the most important initial lead in the detection of SCD. Severe musculoskeletal signs during the first presentation was seen in the lymphoma case, residual renal dysfunction after remission of multiple myeloma, and areas of infarction in the spleen in CT scans in the patient with sickle cell anemia were the most interesting findings in these cases. A standard therapeutic approach without any additional toxicity was relevant in all cases. Detailed clinical presentation and outcome of these five cases are documented here and the literature has been reviewed.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
6/21. An ultrastructural study of the red pulp of the spleen and the liver in unstable hemoglobin hemolytic anemia.Electron microscopic observations on the process of red cell destruction in the spleen and liver of a patient with congenital Heinz body hemolytic anemia, associated with a new variant of unstable hemoglobin, are reported. Two major mechanisms of destruction of Heinz body-containing red cells were noted. One was phagocytosis of these cells in toto by cordal macrophages. The other mechanism though less significant quantitatively, was intravascular hemolysis of injured red cells in the splenic microvasculature. In the liver, phagocytosis of damaged red cells by kupffer cells was rate and there was no evidence of intravascular hemolysis in this organ. These morphological findings, together with almost complete recovery from hemolysis following splenectomy, indicated that Heinz body-containing red cells were removed from the circulation predominantly by the spleen. In contrast to experimental Heinz body anemia in animals, heinz bodies were present even in the nucleated cells.- - - - - - - - - - ranking = 6keywords = spleen (Clic here for more details about this article) |
7/21. Dominant beta-thalassemia with hemoglobin Hradec Kralove: enhanced hemolysis in the spleen.We describe a 6-year-old girl and her mother with dominant beta-thalassemia due to hemoglobin Hradec Kralove (Hb HK). Both patients presented microcytic anemia, jaundice, splenomegaly, cholelithiasis, and recurrent hemolytic bouts. Osmotic resistance tests using saline and coiled planet centrifugation revealed the increased fragility of the red cell membrane. On the other hand, the glycerol lysing time was prolonged, and results of the isopropanol test were weakly positive. Despite mimicking the features of hereditary spherocytosis, the results of the genetic analyses verified the second reported family with Hb HK (codon 115, GCC [Ala] --> GAC [Asp]). splenectomy was effective for the amelioration of hemolysis. Of 7 reported patients with Hb variants at beta-globin codon 115 (Hb Madrid and Hb HK), 5 underwent splenectomy. Because of the variable augmentation of extramedullary hemolysis in dominant beta-thalassemias, genotyping is necessary for determining the clinical indication of splenectomy.- - - - - - - - - - ranking = 4keywords = spleen (Clic here for more details about this article) |
8/21. Fine needle aspiration diagnosis of extramedullary hematopoiesis resembling mediastinal and paravesical tumors. A report of 2 cases.BACKGROUND: Extramedullary hematopoiesis is a compensatory phenomenon that occurs when normal function of the bone marrow is disturbed. It is most often seen in patients with hematologic disorders. Although the sites most frequently involved are the spleen, liver and lymph nodes, other organs may be involved. We report on 2 cases of extra-medullary hematopoiesis mimicking posterior mediastinum and paravesical tumors and diagnosed by fine needle aspiration cytology. CASES: Two men, aged 72 and 82 years, with hemolytic anemia (thalassemia intermedia and idiopathic) presented with solid masses involving the posterior mediastinum and paravesical region. The patients underwent computed tomography-guided fine needle aspiration. The smears were composed of normal bone marrow elements. Both cases were diagnosed as extramedullary hematopoiesis. CONCLUSION: Fine needle aspiration cytology is an useful method of diagnosing extramedullary hematopoiesis and aids in planning treatment.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
9/21. Report of an unusual case of persistent bacteremia by bartonella bacilliformis in a splenectomized patient.We report a case of a 56-year-old man with a history of splenectomy for idiopathic thrombocytopenic purpura who developed persistent bacteremia in the acute phase of human bartonellosis. This patient did not develop hemolytic anemia. Only after several courses of antibiotic treatment was the infection eradicated. This is an unusual case of overwhelming post-splenectomy infection by bartonella bacilliformis, which provides clinical evidence that the spleen is a critical effector organ of clearance of this infection as well as the effector organ of bartonellosis-associated hemolytic anemia.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
10/21. Kala-azar associated with coombs-positive autoimmune hemolytic anemia in the patients coming from the endemic area of this disease and successful treatment of these patients with liposomal amphotericin b.Kala-azar is an intracellular parasitic infection that infects and multiplies in the macrophages of the liver, the spleen, and the bone marrow. It is characterized by intermittent fever, hepatosplenomegaly, pancytopenia, and hypergammaglobulinemia. Although anemia is a usual finding, Coombs-positive autoimmune hemolytic anemia (AIHA) has rarely been reported with this disease. Pentavalent antimonial compounds remain the mainstay of treatment worldwide. Liposomal amphotericin b (L-AmB) is currently preferred in the treatment of kala-azar because of the resistance to pentavalent antimonals. The authors diagnosed kala-azar associated with Coombs-positive AIHA in 3 patients and treated them with L-AmB (1-5 mg/kg/day) for 30-36 days. Now, all of these patients are healthy following up at the outpatient base for 18-34 months. Kala-azar must be considered in patients with Coombs-positive AIHA and living in and coming from the endemic region for this disease, and it can be successfully treated with L-AmB.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
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