1/219. Altered erythropoiesis and increased hemolysis in hemoglobin m Akita (M Hyde Park beta92 His replaced by Tyr) disease.Hb M Akita disease is a cyanotic hemoglobinopathy found in Akita Prefecture, japan. The abnormal hemoglobin was found to be the same as Hb M Hyde Park (beta92 His replaced by Tyr) by chemical analysis in 1967. In this disease signs of accelerated hemolysis (serum bilirubin, 2.4 mg/dl; splenomegaly, 2 finger breadths; Hb, 10.7 g/dl; reticulocyte index, 2.7) were noted, but the causes of its slight anemia were revealed to be fairly complex by ferrokinetic study, RBC life-span measurement, and 99mTc myeloscintigram. The anemia in this disease is caused not only by shortened erythrocyte survival (T 1/2 = 11.5 days by 51Cr-tagging method) and sequestration of red cells in the spleen (spleen: liver ratio = 2.5 approximately 3.0 by 51Cr-surface counting), but also by slow supply of erythrocytes to the peripheral blood from the bone marrow, presumably, related to the existence of unstable Hb M Akita and its derivative (Hb Akita) in the erythroid cells. Both Carrell's isopropanol test and Heinz body formation test were positive. In spite of maximally increased total erythropoiesis (8 times as high as the normal level; M:E ratio = 0.22:1.0), supply of red cells from the bone marrow to the peripheral blood was significantly decreased. The distribution of hematopoietic sites throughout the body was reasonably uniform.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
2/219. Identification of new Fas mutations in a patient with autoimmune lymphoproliferative syndrome (ALPS) and eosinophilia.autoimmune lymphoproliferative syndrome (ALPS) is a rare, newly recognized, chronic lymphoproliferative disorder in children and is characterized by lymphadenopathy, splenomegaly, pancytopenia, autoimmune phenomena and expansion of double-negative (DN) T lymphocytes (TCR alpha beta , CD4-, CD8-). Defective lymphocyte apoptosis caused by mutations of the Fas (CD95) gene has been linked in the pathogenesis of ALPS, as binding of Fas-ligand to Fas can trigger apoptosis. Of the ALPS cases reported to date, point mutations, frameshifts and silent mutations in Fas all have been identified. We report two new point mutations in Fas in a child with ALPS and eosinophilia; studies on other family members established the pattern of inheritance for these mutations. Flow cytometric analysis of blood and tissues (spleen, lymph node, bone marrow) revealed abnormally expanded populations of DN T lymphocytes. Furthermore, activated lymphocytes and IFN gamma-activated eosinophils were resistant to Fas-mediated apoptosis. Eosinophil resistance to Fas-mediated apoptosis has not been previously described in ALPS. Sequencing of Fas revealed two separate mutations not previously reported. One mutation, a C to T change at base 836, was a silent mutation inherited from the mother, while the second mutation, a C to A change at base 916, caused a non-conservative amino acid substitution in the death domain of Fas, changing a threonine to a lysine. This mutation is associated with a predicted change in the structure of a part of the death domain from a beta-pleated sheet to an alpha-helix. We speculate that the mutation in the death domain prevents the interaction of Fas with intracellular mediators of apoptosis and is responsible for the autoimmune manifestations of ALPS and the abnormal lymphocytosis and eosinophilia in this patient.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
3/219. Splenic littoral cell angioma in an infant.A 1-year-old girl presented with fever, asthenia, and splenomegaly with hypersplenism. Abdominal ultrasound scan and magnetic resonance imaging showed multiple nodular cystic masses in an enlarged spleen. The histological examination of the resected spleen showed a novel type of vascular tumor called littoral cell angioma. The histopathologic and immunohistochemical features of this rare lesion are described. Distinction from other splenic vascular tumors is stressed because the clinical behavior of this new entity seems to be benign.- - - - - - - - - - ranking = 2keywords = spleen (Clic here for more details about this article) |
4/219. Partial splenic embolization in beta-thalassemia major. A case report.Partial splenic arterial embolization was performed in a thalassemic child for hypersplenism manifested by splenomegaly, leukopenia, thrombocytopenia, and anemia requiring frequent erythrocyte transfusion. During a follow-up period of 11 months, his hematological parameters improved significantly and the spleen size decreased. Partial splenic embolization could be an alternative therapy to surgical splenectomy for thalassemic children with hypersplenism.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
5/219. Laparoscopically assisted splenectomy following preoperative splenic artery embolization using contour emboli for myelofibrosis with massive splenomegaly.Laparoscopically assisted splenectomy with an 8- to 10-cm left upper paramedian laparotomy was performed following preoperative splenic artery embolization using painless contour emboli (super absorbent polymer microsphere) with early successful results in two men (46 and 37 years old) with myelofibrosis accompanied by massive splenomegaly. dissection around the lower part of the spleen and the hilum initially was performed intracorporeally with the usual laparoscopic view under 12 mm Hg pneumoperitoneum. The alternating changes of viewpoints between the direct view through an 8- to 10-cm incision and the usual laparoscopic view with or without application of a retraction method were effective for safe hilar devascularization. Preoperative splenic artery embolization at the distal site was effective for safe dissection around the enlarged spleen. The patients did not complain of pain before operation. Preoperative painless embolization and laparoscopically assisted splenectomy with small laparotomy promotes the feasibility and safety of minimally invasive splenectomy for myelofibrosis with massive splenomegaly.- - - - - - - - - - ranking = 2keywords = spleen (Clic here for more details about this article) |
6/219. Laparoscopic splenectomy for splenic sequestration crisis.Medical and surgical advances have improved the treatment of splenic sequestration crisis in pediatric patients with sickle cell disease (SCD). Rapid enlargement of the spleen can result from sickled blood cels being trapped in the spleen, which can be life threatening. The laparoscopic splenectomy procedure using the lateral approach has been adapted successfully for the pediatric patient since 1993. Children with SCD who have a history of a splenic sequestration crisis usually are scheduled for an elective splenectomy procedure after the first documented crisis to reduce the risk of death. Some key benefits of this new surgical approach include a shortened hospital stay, decreased postoperative pain, and faster recovery and return to normal activities for the child with SCD.- - - - - - - - - - ranking = 2keywords = spleen (Clic here for more details about this article) |
7/219. splenectomy may improve the glomerulopathy of type II mixed cryoglobulinemia.Many patients with type II mixed cryoglobulinemia have been shown to be infected with hapatitis C virus (HCV). Therefore, interferon-alfa has become the first choice of treatment for patients with HCV-associated cryoglobulinemia. However, the disease often relapses after the discontinuation of interferon therapy. The long-term effect of interferon therapy is controversial. Therefore, a more effective therapy needs to be developed. A 62-year-old Japanese woman was admitted to our hospital for the examination of abnormal liver function tests, severe edema, and purpura in her lower extremities. Glomerulopathy secondary to HCV-related cryoglobulinemia was suspected. Her serum creatinine was increased to 2.1 mg/dL. Interferon therapy was considered initially. However, because of pancytopenia caused by liver cirrhosis and splenomegaly, splenectomy was performed in February 1997, before the start of interferon therapy. Renal biopsy specimen taken at the time of the splenectomy showed typical cryoglobulinemic glomerulonephritis. Gradually, after surgery, the patient's thrombocytopenia and anemia improved, her proteinuria and hematuria were decreased, her cryocrit dropped from 15% to 5%, the Ccr increased from 21.1 mL/min to 48.8 mL/min, and the purpura in her lower extremities disappeared. A repeat renal biopsy performed in May 1998 showed marked histological improvement. splenectomy is not widely accepted as a treatment for cryoglobulinemia. Our case suggests the possibility that the monoclonal-IgM component of the type II cryoglobulin may be formed in the spleen. In conclusion, splenectomy may be an effective therapy for cryoglobulinemia in patients with HCV-positive liver cirrhosis and pancytopenia secondary to splenomegaly.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
8/219. Isolated splenic lymphoma: an elusive preoperative diagnosis.Four patients underwent splenectomy for various clinical and radiological diagnoses and were found to have primary splenic lymphoma at surgery and histology. The diagnosis was classical Hodgkin's lymphoma, mixed cellularity type (one case); marginal zone B-cell non-Hodgkin's lymphoma (one case); and large B cell type non-Hodgkin's lymphoma (two cases). The first two patients had multiple nodules in the spleen measuring 0.1-0.5 cm while large cell lymphomas had large nodules (largest measuring 11 cm x 7 cm x 4 cm). The diagnoses were confirmed by immunohistochemical analysis. Mean follow up of these patients was 11 months; all patients received chemotherapy. One patient died, of causes not related to the disease process.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
9/219. splenomegaly and chronic disseminated intravascular coagulation in Osler-Weber-Rendu disease: a case report.A 45-year-old Japanese woman with splenomegaly and thrombocytopenia was referred to our hospital. The diagnosis of Osler-Weber-Rendu disease (Osler's disease) was made because of spotty telangiectasia on her tongue, recurrent epistaxis since childhood, and a diathesis indicated by her family history. The patient's laboratory examination revealed anemia, thrombocytopenia, and other data consistent with chronic disseminated intravascular coagulation (DIC). bone marrow examination was normal. Abdominal computed tomography showed marked enlargement of the spleen with deformity and calcified plaque, not homogeneously enhancing. hypersplenism was not observed. Platelet scintigraphy indicated a remarkable uptake in the spleen. She was diagnosed as having chronic DIC associated with vascular lesions of Osler's disease in the spleen. splenectomy was performed and the subsequent pathological findings indicated that fragility of the fine vascular architecture of the splenic red pulp might have been responsible for pathogenesis. The large pooling of blood with coagulation was thought to be secondary.- - - - - - - - - - ranking = 3keywords = spleen (Clic here for more details about this article) |
10/219. Disseminated histoplasmosis with reactive hemophagocytosis: aspiration cytology findings in two cases.Two cases of disseminated histoplasmosis associated with reactive hemophagocytic syndrome are described. The clinical presentation was with prolonged unexplained fever and hepatosplenomegaly. On a strong clinical possibility of tuberculosis, antitubercular treatment was initiated in both patients. Lymph node (case 1), splenic (case 2), and bone marrow aspiration, however, showed sheets of proliferating histiocytes, and intracellular and extracellular histoplasma organisms. Aspiration cytology was thus valuable in establishing the final diagnosis. The patients had a fulminant clinical course and died of hemorrhagic shock within 48 hr of hospital admission before specific therapy could be initiated. histoplasmosis can mimic tuberculosis clinically. There is a need for an increased awareness of the clinicopathological spectrum of histoplasmosis, especially its rarer manifestations as hemophagocytic syndrome. In suspected cases, aspirations from the lymph node, liver, and spleen can be performed safely and should be utilized for early diagnosis.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
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