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.     

Clinical Investigation on Splenic Aneurysms and Gamna-Gandy Nodules

Coeliac angiography and histological studies of the spleen in 39 cases with portal hypertension revealed splenic artery aneurysm in 18%, Gamna-Gandy nodules in 43%, and infarction in 10%. No significant difference in the incidence of intrahepatic obstruction of the portal vein or intrahepatic obstruction of the hepatic vein was noted between the non-cirrhotic and cirrhotic groups.     

Memory switched B cell percentage and not serum immunoglobulin concentration is associated with clinical complications in children and adults with specific antibody deficiency and common variable immunodeficiency

Although idiopathic humoral immunodeficiencies are arbitrarily classified into specific antibody deficiency (SAD) or common variable immunodeficiency (CVID), this distinction does not accurately predict the risk of the bronchiectasis, one of the major long-term clinical complications in these patients. In this study, clinical complications were compared with laboratory markers of cellular and humoral immunity in fifty-five consecutive patients (27 children and 28 adults) attending regional immunology clinics in Manchester, United Kingdom. Reduced CD19(+)CD27(+)IgD(-) B cell percentage but not serum immunoglobulin levels or classification of patients into SAD and CVID was associated with a significantly higher prevalence of bronchiectasis (OR 0.4 (0.2-0.8), P = 0.001), splenomegaly (OR 0.2 (0.1-0.5), P = 0.001) and autoimmunity (OR 0.4 (0.2-0.7), P = 0.003). We conclude that in patients with idiopathic humoral immunodeficiencies assessment of B cell switching more accurately predicts clinical prognosis than either classification of patients into SAD and CVID or serum immunoglobulin concentrations.     

肝硬化脾大脾切除加自体脾移植分析

目的：分析脾切除加自体脾移植治疗肝硬化脾大的临床疗效。方法：检测我院采取脾切除加自体脾移植治疗的肝硬化脾大患者术前、术后肝功能及血常规,并进行对比,同时将术后2年回访的谷丙转氨酶（ALT）复常情况同术前进行对比。结果：术后2年ALT〉80 U/L的患者占比由术前的76.79%下降为19.64%;腹水患者占比由术前的87.50%下降为16.07%;肝萎缩患者占比由术前的60.71%下降为33.93%,术后患者病情得到显著改善（P〈0.01）。结论：脾切除加自体脾移植有效地保存了自体脾片组织,提高了患者术后机体免疫功能,使肝硬化致脾大患者术后的肝功能得到了良好改善。     

Isolated splenic peliosis presenting with giant splenomegaly and severe coagulopathy

We herein describe a case of successful surgical treatment of isolated splenic peliosis presenting with giant splenomegaly and severe coagulopathy. Pathological features of the spleen included multinodular, blood-filled, cyst-like lesions distributed throughout the whole organ. The pathogenesis of isolated splenic peliosis is controversial, and no definitive disease mechanism has been reported. To our knowledge, this is the first report in English of a case like this.     

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.     

Splenic irradiation for splenomegaly: A systematic review

Splenic irradiation (SI) is a palliative treatment option for symptomatic splenomegaly (i.e. for pain, early satiety, pancytopenia from sequestration) secondary to hematologic malignancies and disorders. The purpose of the current article is to review the literature on SI for hematologic malignancies and disorders, including: (1) patient selection and optimal technique; (2) efficacy of SI; and (3) toxicities of SI. PICOS/PRISMA methods are used to select 27 articles including 766 courses of SI for 486 patients from 1960 to 2016. The most common cancers treated included chronic lymphocytic leukemia and myeloproliferative disorders; the most common regimen was 10 Gy in 1 Gy fractions over two weeks, and 27% of patients received retreatment. A partial or complete response (for symptoms, lab abnormalities) was obtained in 85–90% of treated patients, and 30% were retreated within 6–12 months. There was no correlation between biologically equivalent dose of radiation therapy and response duration, pain relief, spleen reduction, or cytopenia improvement (r² all <0.4); therefore, lower doses (e.g. 5 Gy in 5 fractions) may be as effective as higher doses. Grade 3–4 toxicity (typically leukopenia, infection) was noted in 22% of courses, with grade 5 toxicity in 0.7% of courses. All grade 5 toxicities were due to either thrombocytopenia with hemorrhage or leukopenia with sepsis (or a combination of both); they were sequelae of cancer and not directly caused by SI. In summary, SI is generally a safe and efficacious method for treating patients with symptomatic splenomegaly.     

Déficit en sphingomyélinase acide (maladie de Niemann-Pick B) : une étude rétrospective multicentrique de 28 patients adultes

Introduction

Acid sphingomyelinase deficiency (ASMD) is an autosomal recessive disease with a clinical spectrum ranging from a neurovisceral infantile form (Niemann-Pick disease type A) to a chronic visceral form also encountered in adults (Niemann-Pick disease type B, NP-B).

临床护理路径在巨脾型晚期血吸虫病快速康复外科流程中的应用

目的 目的 评价快速康复外科 （FTS） 流程的临床护理路径 （FTS?CNP） 在围手术期巨脾型晚期血吸虫病 （晚血） 患者 中的应用效果。 方法 方法 将2012年1月-2014年12月在本院择期手术治疗的80例巨脾型晚血患者随机分为观察组和对 照组, 每组40例。观察组实施FTS?CNP,对照组实施常规护理。观察两组患者术后生理康复情况及心理健康水平。 结 结 果 果 观察组患者术后首次进食时间[ （14.67 ± 2.90）h]、 肛门排气时间[ （25.30 ± 3.46）h]、 下床活动时间[ （29.40 ± 5.57）h]、 住院时间[ （19.00 ± 2.82）d]均短于对照组[分别为 （44.50 ± 6.71）h、（52.80 ± 6.95）h、 （35.05 ± 10.37）h、（25.08 ± 2.39）d], 且术后并发症发生率 （7.50%） 低于对照组 （25.00%）, 差异均有统计学意义 （P均＜0.05）。术后观察组患者焦虑自评量表 评分[ （41.22 ± 5.60） 分]、 抑郁自评量表评分[ （40.28 ± 6.67） 分]均低于对照组[ （46.89 ± 8.92） 分、（47.06 ± 10.29） 分], 差异均 有统计学意义 （P均< 0. 01）。 结论 结论 FTS?CNP可促进围手术期巨脾型晚血患者术后快速康复, 减少住院时间及术后并 发症, 促进患者心理健康。