艾滋病患者抗逆转录病毒治疗后的超声影像学研究

目的应用超声影像学技术评估艾滋病(AIDS)患者抗逆转录病毒治疗后左室结构、功能以及腹腔脏器形态学的变化。方法应用超声心动图技术于41例AIDS患者(AIDS组)用药前基线状态、抗病毒用药48周及96周分别测量其心脏形态结构参数,同时应用腹部探头测量AIDS组及38例健康体检者(对照组)的肝脏、脾脏大小及门静脉内径。结果 AIDS组用药96周与用药前、用药48周比较,除左室流出道血流速度、二尖瓣口E峰减速时间均有所提高(P<0.05)外,其余各参数比较差异均无统计学意义;AIDS组用药前的脾脏肿大阳性率较对照组显著增加(χ2=10.94,P<0.05),用药前、用药48周及用药96周3个时间点间的脾脏肿大阳性率比较差异均无统计学意义。结论 AIDS患者脾脏肿大者明显多于健康成人,短期抗病毒治疗不会影响心脏结构功能,且不会加重AIDS患者的脾肿大程度,超声可准确评估抗病毒治疗后AIDS患者心脏结构功能及腹腔脏器形态学变化。     

La maladie de Gaucher : quand y penser ?

Gaucher disease is a rare autosomal recessive genetic disease, caused by a deficiency of the lysosomal enzyme, glucocerebrosidase that leads to the accumulation of its substrate (glucosylceramide) in lysosomal macrophages. In the general population, its incidence varies between 0.4 and 5.8/100,000 inhabitants. Type 1 Gaucher disease is the most frequent and is characterized by its extreme heterogeneity including asymptomatic or more severe presentations. The most frequent symptoms are anemia, thrombocytopenia, splenomegaly, and/or hepatomegaly, and a potentially severe bone involvement. Type 2 and type 3 Gaucher diseases are associated with neurological involvement that can be severe. Diagnosis is confirmed by demonstrating a deficiency of glucocerebrosidase activity in leucocytes, and by the identification of biallelic pathogenic variants in GBA1 gene. Type 1 Gaucher disease is associated with a higher risk of Parkinson disease, some solid cancers, and hematologic diseases in particularly multiple myeloma. Specific treatment, such as enzyme replacement therapy or substrate reduction therapy is indicated in symptomatic type 1 Gaucher disease. Only enzyme replacement therapy is indicated in type 3 Gaucher disease. Treatment improves quality of life and prognosis. The rarity of Gaucher disease and its wide variability in clinical presentations lead to diagnosis delays. There is a strong need for a better knowledge of its symptoms among physicians, to reduce irreversible complications.     

射频消融治疗犬继发性脾脏肿大的影像学和病理学关系

目的：通过观察射频消融(RFA)脾脏治疗继发性脾肿大犬模型的影像学和病理学改变，探讨RFA治疗脾功能亢进的可行性。方法：16只健康杂种犬随机分为组Ⅰ(脾静脉结扎，4只)和组Ⅱ(脾静脉结扎 RFA，12只)，结扎脾静脉诱导瘀血性脾肿大，3周末组Ⅱ动物剖腹行RFA毁损脾脏。RFA后观察动物并发症，定期行CT扫描以及切取脾脏观察热毁损后脾脏病灶的影像学和组织病理学变化。结果：全组动物无死亡和并发症。CT扫描显示，脾静脉结扎后脾脏明显肿大并可持续2个月以上，脾脏RFA后病灶呈节段性毁损，包括高密度的坏死区和外周低密度的梗死区——后者称为“旁观者效应”；梗死区在RFA后4～6周内消失，残脾缩小。射频热能引起脾脏局部组织凝固性坏死和广泛的血栓性梗死，梗死区逐渐吸收、纤维化后最终消失，有活性脾脏组织内血管闭塞、脾窦消失、纤维蛋白沉积——“脾脏实性变”是脾脏缩小的病理基础。结论：RFA治疗实验性脾肿大和脾功能亢进是可行和安全的，RFA后脾脏独特的影像学和病理学变化可作为将来I临床实施RFA治疗脾功能亢进的疗效和随访观察的指标。     

Maladie de Gaucher de type 1 simulant une thrombopénie immunologique : intérêt de l’hyperferritinémie et de l’hypergammaglobulinémie dans le bilan d’une thrombopénie isolée

IntroductionGaucher disease type 1 is a rare genetic disease. It can cause thrombocytopenia. Current guidelines do not support bone marrow examination in front of isolated thrombocytopenia if no evidence suggests malignant hemopathy. This strategy aiming at sparing unnecessary investigations makes such rare diseases more difficult to diagnose.Case reportA 31-year-old woman was diagnosed with immune thrombocytopenia according to current guidelines. She presented later with mild splenomegaly. Bone marrow aspirate smears showed Gaucher cells. Gaucher disease was then confirmed. Looking backward, initial biological clues (hyperferritinemia, hypergammaglobulinemia) should have enabled to consider the diagnosis.ConclusionGaucher disease type 1 can be responsible for apparently isolated thrombocytopenia. The disease must be looked for if the thrombocytopenia is associated with unexplained hypergammaglobulinemia or hyperferritinemia. Diagnosing immune thrombocytopenia without bone marrow sample requires to systematically pay attention to any clinical or biological abnormality, not to ignore rare differential diagnoses.     

晚期血吸虫病巨脾切除对免疫功能的影响

目的 探讨晚期血吸虫病巨脾切除对免疫功能的影响。方法 对20例晚期血吸虫病巨脾病人切除前、术后两周、术后半年、术后一年分别抽取周围静脉血测IgG，IgA,IgM,C3,C4,TNF,sIL-2R,CD3^ ,CD4^ ,CD8^ ，用F检验作出显著性测定。结果 IgA,IgM,C3,C4,TNF,sIL-2R,CD8^ 切脾前后差异不显著（P＞0．05），IgG,CD3^ ,CD4^ ,CD4^ /CD8^ 切脾后升高，差异显著（P＜0．01－0．05）。结论 晚期血吸虫病巨脾切除后对体液免疫和细胞免疫均无不良影响，因而治疗性脾切除是合理的。     

Enzymhistochemische,histometrische und ultrastrukturelle Untersuchungen von Milzen bei der Vinylchlorid-Krankheit

Zusammenfassung Durch histometrische, enzymhistochemische und elektronenmikroskopische Untersuchungen konnte nachgewiesen werden, da\ die pathologischen MilzverÄnderungen bei der Vinylchlorid-Krankheit nicht durch hepatisch bedingte Pfortaderdruckerhöhung verursacht werden, sondern auf einer Gewebsalteration durch das Vinylchlorid oder dessen Metabolite beruhen. Dabei sind vor allem die faserbildenden Zellen der Milz beteiligt. Faserassoziierte Reticulumzellen in der roten Pulpa und fibroblastische Reticulumzellen in der wei\en Pulpa werden zu übermÄ\iger Bindegewebsneubildung stimuliert. Hierdurch kommt es in der roten Pulpa zur bindegewebigen Verödung der FiltrationsrÄume mit einer Reduzierung der Pulpastrangmakrophagen und in der wei\en Pulpa zu einer Vernarbung der periarteriellen Lymphscheiden. Die Folgen dieser Fibrosierungsprozesse sind charakteristische VerÄnderungen in der quantitativen Zusammensetzung der Gewebskomponenten, die sich deutlich von denjenigen in Milzen bei Lebercirrhose und Milzvenenthrombose unterscheiden.

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Enzyme histochemical, histometrical and ultrastructural studies of spleens in vinylchloride-disease

Summary By means of histometric, enzyme histochemical, and electron microscopic investigations it was demonstrated that the pathological changes in the spleen in vinylchloride-disease are primary. Fibroblastic cells are the only specific splenic cells involved. Fibre-associated reticulum cells of the red pulp and fibroblastic reticulum cells in white pulp are stimulated to produce excessive amounts of the extracellular elements of connective tissue, especially collagen fibrils. The newly formed connective tissue causes obliteration of extracellular blood channels in the red pulp and thus a reduction in the number of pulp-cord macrophages, and scarring of the periarterial lymphatic sheaths. The results of this fibrosing process are characteristic quantitative changes in the splenic histologic structures. These changes are different from those structural alterations occuring in spleens following extrasplenic hemodynamic changes, such as thrombosis of the splenic veins or cirrhosis of the liver.

     

TNF-dependent overexpression of CCL21 is an underlying cause of progressive lymphoaccumulation in generalized lymphoproliferative disorder

The human condition autoimmune lymphoproliferative syndrome and the murine mutation generalized lymphoproliferative disorder (gld/gld) are both caused by mutations of Fas or Fas ligand and are characterized by severe splenomegaly and lymphadenopathy. In the mouse, the additional absence of TNF attenuates the gld/gld syndrome through an unknown mechanism. We hypothesized that this unexpected outcome was not mediated by increased apoptosis but changes of T cell localization. We demonstrated that the homeostatic chemokine CCL21 is strongly up-regulated in the spleen of C57BL/6 (B6).gld/gld and B6.gld/gld.TRAIL-/- mice. In contrast, a distinct consequence of TNF deficiency in B6.gld/gld mice was the substantially reduced splenic production of CCL21. An analysis of the cognate chemokine receptor CCR7 showed a complete, age-dependent down-regulation of this receptor on B6.gld/gld conventional peripheral T cells that are therefore unable to react to this chemokine. These results demonstrate a new role for the pro-inflammatory cytokine TNF and the TNF-regulated chemokine CCL21 in the complex etiology of the autoimmune syndrome in B6.gld/gld mice.     

肝硬化充血性脾大伴脾功能力进行脾次全切除术后免疫功能观察

24例肝硬化充血性脾大伴脾功能亢进患者分脾次全切除和全脾切除组，对其手术前后的T淋巴细胞转化率、IgA、IgG、IgM进行了对比观察，发现脾次全切除，保留原脾组织6cm×5cm×4cm大小，能较好地维持各项免疫指标在正常范围。而全脾切除术后T淋巴细胞转化率、IgM、IgA呈持续下降，直至术后3个月仍处于最低值。认为脾次全切除，保留适当的残脾能较好地维持其免疫功能．     

Case report of rare chronic myelogenous leukemia related multibacterial splenic abscess presenting with scrotal swelling

INTRODUCTION

Splenic abscesses associated with leukemia are rare. Most reported cases of splenic abscesses occur after chemotherapy and are related to the immunosuppressive effects of the chemotherapy. Their etiology is most frequently fungal.

PRESENTATION OF CASE

A 58-year-old male presented with splenomegaly and scrotal swelling secondary to a multibacterial splenic abscess which required a splenectomy. Upon investigation he was found to suffer from chronic myeloid leukemia (CML) and epididymitis.

DISCUSSION

Splenic abscesses are rarely found in leukemic patients. Reported cases are fungal and commonly occur after chemotherapy due to immunosuppression. Scrotal swelling with concurrent splenomegaly can be found in other pathologies including brucellosis, Lyme disease and even non-Hodgkin primary testicular lymphoma. Scrotal swelling in our case was likely secondary to epididymitis and exacerbated by the effects of splenomegaly upon the systemic circulation promoting venous congestion.

CONCLUSION

This case illustrated an unusual presentation of CML because the patient presented with splenomegaly, a multibacterial splenic abscess, and scrotal swelling.