CD1d-restricted NKT cells contribute to malarial splenomegaly and enhance parasite-specific antibody responses

CD1d-restricted NKT cells are a novel T cell lineage with unusual features. They co-express some NK cell receptors and recognize glycolipid antigens through an invariant T cell receptor (TCR) in the context of CD1d molecules. Upon activation through the TCR, NKT cells produce large amounts of IFN-gamma and IL-4. It has been proposed that rapid cytokine output by activated NKT cells may induce bystander activation of other lymphoid lineages. The impact of CD1d-restricted NKT cell activation in the induction of B cell-mediated immune responses to infection is still unclear. We show here that CD1-restricted NKT cells contribute to malarial splenomegaly associated with expansion of the splenic B cell pool and enhance parasite-specific antibody formation in response to Plasmodium berghei infection. The increased B cell-mediated response correlates with the ability of NKT cells to promote Th2 immune responses. Additionally, antibody responses against the glycosylphosphatidylinositol (GPI)-anchored protein merozoite surface protein 1 (MSP-1) were found to be significantly lower in CD1(-/-) mice compared to wild-type animals. P. berghei-infected MHC class II (MHCII)(-/-) mice also generated antibodies against MSP-1, suggesting that antibody production against GPI-anchored antigens in response to malaria infection can arise from both MHCII-dependent and independent pathways.     

An unexpected cause of a febrile patient with huge splenomegaly

We report an unexpected cause of a febrile patient with huge splenomegaly. A 32-year-old patient with fever and huge splenomegaly was admitted to our hospital. Diagnostic splenectomy revealed that the enlarged spleen adhered strongly to the abdominal organs. Pathologically, the splenic parenchyma showed no malignant cells, and the soft tissue adjacent to the splenic hilum showed a proliferation of fibroblastic or myofibroblastic spindle cells with fibrosis and lymphoplasmacytic infiltration. These findings lead to a diagnosis of peritoneal fibrosis, and an administration of 50 mg/day of prednisolone alleviated all the symptoms. The differential diagnosis of huge splenomegaly with fever usually includes hematolymphoid malignancies and infectious diseases; however, our case was diagnosed as idiopathic retroperitoneal fibrosis. Our case suggests that when we see patients with fever and huge splenomegaly, differential diagnosis should include retroperitoneal fibrosis.     

Visceral Leishmaniasis mimicking a flare of systemic lupus erythematosus

Summary Fever in systemic lupus erythematosus (SLE) may be caused by exacerbation of the disease itself or by infection. We report on a patient with a long standing history of SLE that was complicated by fever and pancytopenia with no splenomegaly. SLE disease activity was suspected because of an elevated DNA-antibody titer. The early positive response to corticoid therapy may have masked the underlying infection. Visceral leishmaniasis was diagnosed by a repeated bone marrow biopsy and serological testing.     

门静脉高压巨脾大部切除后残脾神经分布与定量研究

目的：观察门静脉高压巨脾大部切除后残脾神经纤维分布与密度变化,评估残脾保留的价值。 方法：选取门静脉高压脾肿大行脾大部切除并残脾腹后固定术患者13例,收集患者术后切取的巨脾组织,以及术后8年穿刺获取的残脾组织,另取外伤性脾组织13例为正常对照。采用免疫组化法检测脾神经肽Y（NPY）和神经丝蛋白200（NF 200）阳性神经纤维分布及密度。 结果：3组脾组织NPY和NF200阳性神经纤维的分布部位大致相同,但两者在巨脾组织中的密度明显较高。红髓部分的定量分析显示,巨脾组织NPY与NF200阳性神经纤维密度均明显高于残脾组织和正常脾组织（均P<0.05）,而两种阳性神经纤维密度在残脾组织与正常脾组织间差异无统计学意义（均P>0.05）。 结论：巨脾大部切除术后残脾神经纤维分布及含量与正常脾大致相同,提示解除高压环境后,残脾神经功能能逐渐恢复正常。     

Splenomegaly, pancytopenia and pregnancy: a case report and review of the literature

We present a 35-year-old previously healthy primigravida who presented at 26^4/7 weeks of gestation with pancytopenia and hepatosplenomegaly. She received 10 transfusions and delivered at 34^4/7 weeks of gestation by cesarean section. Two months later following splenectomy, she was diagnosed with malaria. Physicians should have a high index of suspicion for malaria in the context of splenomegaly and pancytopenia in pregnancy even in the absence of fever.     

Co hematolog powinien wiedzieć o chorobie Gauchera

Hematological symptoms can be helpful for the diagnosis of inherited metabolic disorders, including Gaucher disease. Gaucher disease is a progressive, multisystem lysosomal storage disorder caused by the deficient activity of the lysosomal enzyme, glucocerebrosidase, arising from autosomal recessive mutations in the GBA1 gene (1q21). Because of constant presence of hematological symptoms in Gaucher disease, hematologists have always been at the forefront of specialists, who performed initial diagnostics of Gaucher disease. Gaucher cells, the lipid-laden storage macrophages, are the pathologic hallmark of Gaucher disease. The clinical presentation of Gaucher disease is highly variable, giving a complex phenotype of multiorgan disease. Classically, three clinical types of Gaucher disease are distinguished according to the absence (type 1) or presence (types 2 and 3) of neurological symptoms and the dynamics of developing clinical signs. Thrombocytopenia, anemia, hepatosplenomegaly and bone manifestations are the most typical signs of type 1, the most prevalent form of Gaucher disease. This paper presents the most important, from the point of view of a hematologist, issues related to symptomatology, diagnosis, and treatment of Gaucher disease.     

Impact of hematological diagnosis on early and late outcome after laparoscopic splenectomyrid=""

Background: Laparoscopic splenectomy (LS) is now regarded as the treatment of choice for autoimmune thrombopenia (ITP). However, there have been few reports describing the application of LS to other splenic diseases, such as malignant entities and conditions associated with splenomegaly. Hematological diseases have specific clinical features that can influence immediate outcome after LS. Although the long-term effects of LS are unknown, a risk of splenosis has been suggested. Therefore, we designed a study to analyze the impact of primary hematological disease on immediate and late outcome in a prospective series of LS patients. Methods: We performed a prospective analysis of 111 LS done between February 1993 and March 1999. The patients were classified by hematological indications into the following four groups: (a) group 1, low platelet count. This group was further subdivided into group 1A, idiopathic thrombocytopenic purpura (ITP) (n= 48) and group 1B, HIV-related ITP (n= 8); (b) group 2, anemia. This group was further subdivided into group 2A, autoimmune hemolytic anemia (n= 8), and group 2B, spherocytosis (n= 11); (c) group 3, malignancy (n= 28); and (d) group 4, others (n= 8). Immediate outcomes were recorded prospectively. Hematological status and late complications were reviewed after a mean follow-up of 24 ± 18 months. Results: There were no significant differences between the groups in terms of conversion, transfusion requirements, and morbidity, although transfusion and morbidity were slightly higher in group 3. However, hospital stay was significantly longer in groups 3 and 4 than in groups 1 and 2. Long-term follow-up showed satisfactory hematological results in ≥75% of patients (group 1A, 82%; group 1B, 88%; group 2A, 88%; group 2B, 100%; group 3, 75%; group 4, 88%). Overall, late morbidity was 8.3% and mortality was 6.2%, mainly due to deaths in group 4 (six of 22 patients). Conclusion: LS is a safe and reproducible procedure for most hematological indications, with a similar immediate outcome for benign diseases and a long-term hematological response comparable to the standard results that have been observed in open series. Received: 1 April 1999/Accepted: 22 November 1999/Online publication: 8 May 2000     

Epidemiology and morbidity of Schistosoma mansoni infection in a fishing community along Lake Albert in Uganda

Schistosoma mansoni infection, associated morbidity and symptoms were studied in Piida fishing community at Butiaba, along Lake Albert, Uganda, from November 1996 to January 1997. The study revealed that S. mansoni is highly endemic with an overall prevalence of 72%, a mean intensity of 419.4 eggs per gram (epg) faeces (geometric mean for positives only), with 37.8% of males and 33.0% of females excreting over 1000 epg. Prevalence and intensity peaked in the 10-14 year old age group and decreased with increasing age. Females were less heavily infected than males. Differences were also shown between tribes. Diarrhoea and abdominal pain were commonly reported in Piida. However, no clear-cut correlation between intensity of S. mansoni infection and these conditions could be demonstrated, indicating that retrospective questionnaires concerning S. mansoni related-symptomatology are of limited value. Organomegaly, as assessed by ultrasonography, was frequent and hepatomegaly was associated with heavy S. mansoni infection. No correlation was demonstrated between splenomegaly and infection. This study emphasizes that schistosomiasis mansoni is a major public health problem in Piida fishing community and presumably also in many similar fishing communities. These observations call for immediate intervention and can help in planning long-term strategies for sustainable morbidity control.