病毒性肝炎患者血小板减少与脾脏肿大和血小板生成素的关系

目的研究病毒性肝炎患者血小板减少、脾脏肿大及血清血小板生成素(TPO)水平之间的关系,以探讨病毒性肝炎患者血小板减少的发病机制。方法应用腹部彩色B超测量58例病毒性肝炎并血小板减少症患者(A组)以及48例病毒性肝炎血小板正常患者(B组)和20例健康志愿者(C组)的脾脏大小,并采用酶联免疫吸附法测定其血清TPO水平。结果A组脾脏厚度(50.49±13.58mm)明显大于B组(38.45±8.14mm,P<0.01)和C组(32.25±3.73mm,P<0.01)。血小板数与脾脏大小呈负相关(r=-0.553,P<0.01)。血清TPO水平A组(88.05±17.09pg/mL)明显低于B组(100.20±17.63pg/mL,P<0.01)和C组(108.96±25.90pg/mL,P<0.01)。血小板数与血清TPO水平呈正相关(r=0.407,P<0.01)。结论病毒性肝炎血小板减少与脾脏肿大、血清TPO水平下降有关。     

Splenectomy for Torsion of a Wandering Spleen in a Patient with Myeloproliferative Disease

We herein report a rare case of torsion of a wandering spleen in a patient with myeloproliferative disease. A 66-year-old Japanese woman presented to our hospital with abdominal pain and a fever. She had a medical history of polycythemia and secondary myelofibrosis. Abdominal enhanced computed tomography showed an enlarged spleen without enhancement in the lower pelvic region. The clinical diagnosis was severe torsion of a wandering spleen in a patient with myeloproliferative disease, necessitating surgical intervention. Splenectomy was performed after de-rotating to revascularize the spleen. After the operation, the platelet count gradually increased, and aspirin was administered to prevent thrombosis.     

Use of splenic volume estimation to distinguish primary thrombocythaemia from reactive thrombocytosis

Splenic volume was measured by visual assessment of planar images of the spleen, and also by single photon emission computerised tomography (SPECT) using 99mtechnetium tin colloid, in a group of 33 patients with primary thrombocythaemia (PT) or reactive thrombocytosis. Volumes greater than 337 cm3 correlated strongly though not absolutely with PT, all patients with volumes greater than this figure being in the PT group. Simple visual assessment of planar images by an experienced observer matched measured splenic volumes very closely.     

Successful treatment with low-dose splenic irradiation for massive splenomegaly in an elderly patient with hairy-cell leukemia

The therapeutic approach to hairy-cell leukemia (HCL) is in some instances still debated. Although management with alpha-interferon and purine analogues is well established, there is an alternative role for therapeutic splenectomy in patients with massive splenomegaly who have failed to respond to systemic therapy. Most patients with HCL will not be suitable for treatment with splenectomy as their ages at diagnosis are high. Here, we report an elderly Japanese HCL patient whose refractory massive splenomegaly responded well to low-dose splenic irradiation.     

手助腹腔镜技术在巨脾切除中的应用

目的:探讨手助腹腔镜技术在巨脾切除术中的应用。方法:用手助腹腔镜技术实施1例巨脾切除术。结果:顺利完成手助腹腔镜巨脾切除,手术时间3h,术中失血30ml,切除脾脏约40cm×15cm×10cm大小,未中转开腹,无术中术后并发症发生,住院7d,治愈出院。结论:手助腹腔镜脾切除术对于巨脾是可行的、安全的,而且保留了微侵袭外科恢复快的优点,为组织学检查提供足够大的标本。     

Nonfunctioning islet cell carcinoma presenting bleeding gastric varices and splenomegaly

This is a report of a 63-year-old Japanese woman with a nonfunctioning islet cell carcinoma of the pancreas presenting bleeding gastric varices and splenomegaly. These manifestations are extremely rare in patients with nonfunctioning islet cell tumor. The tumor originated in the tail of the pancreas and grew mainly within the spleen. The gastric varices due to increased blood flow to the tumor and arteriovenous fistuals within the tumor were confirmed by angiography and operation. The tumor was resected and she is in a good health for 14-months after the operation.     

Haematological and biochemistry laboratory abnormalities associated with splenomegaly in asymptomatic adults in Masaka, Uganda: implications for HIV biomedical prevention trials

Objectives  To assess the degree of haematological and biochemistry abnormalities associated with splenomegaly in asymptomatic adults in order to determine whether they may be eligible for inclusion in HIV biomedical prevention trials.
Methods  Asymptomatic adults (50% women) aged 18–60  with splenomegaly (≥grade II by Hackett's classification) who agreed to provide blood and urine specimens for laboratory testing were invited to participate in a cross-sectional study. Volunteers who were menstruating, pregnant, infected with HIV, syphilis or Hepatitis B and C, or had significant clinical findings were excluded. Haematological and biochemistry laboratory evaluations were performed for enroled volunteers, and the results were compared to local reference ranges. The proportion of volunteers with out-of-range (OOR) values was estimated for each parameter. Linear regression models were fitted to investigate the association between grade of splenomegaly and laboratory values.
Results  The proportion of volunteers with OOR haematology values ranged from 4.5% (mean corpuscular volume) and 15% (CD4 cells) to 31% (basophils). Increasing spleen size was significantly associated with anaemia, thrombocytopenia and low CD4 count. OOR biochemistry values were found in about 10% of volunteers. Increasing spleen size was associated with reduced creatinine phosphokinase and creatinine (in men) and raised lactate dehydrogenase.
Conclusions  In areas with a high prevalence of splenomegaly, most asymptomatic individuals with this condition have haematology and biochemistry values that fall within the local reference ranges, and they could therefore be eligible for inclusion in HIV biomedical prevention trials. However, the effect of splenomegaly on certain parameters should be taken into account during interpretation of laboratory-based adverse events.     

Autoimmune lymphoproliferative syndrome: a cause of chronic splenomegaly, lymphadenopathy, and cytopenias in children-report on diagnosis and management of five patients

Autoimmune lymphoproliferative syndrome (ALPS) usually manifests in early childhood with splenomegaly, lymphadenopathy, and cytopenias. In most patients, it results from mutations in genes that regulate lymphocyte apoptosis via the Fas pathway. Here, we report five children with ALPS. All five children had splenomegaly, cytopenias, and hypertriglyceridemia at presentation; four had lymphadenopathy. Mutations in the Fas receptor gene were demonstrated in three children. Clinical picture is variable: in only one child manifestations are severe enough to require immunosuppressive therapy. Diagnosis of ALPS can be challenging and increased awareness of the disease can result in more directed diagnostic approaches as well as earlier initiation of treatment.