地中海贫血儿童脾切除术后体液和细胞免疫功能研究进展

地中海贫血（地贫）为遗传性疾病，不但引起血液系统损害，还导致心脏受损。目前除了基因治疗和造血干细胞移植外，主要是综合及辅助治疗。脾脏为异常红细胞破坏的主要场所之一，所以脾切除可减轻贫血症状，缓解病情发展。但因脾脏为免疫器官，有报道脾切除后易出现各种感染性疾病，尤其是小儿。本文复习近20年来的相关文献，对地贫脾切除患儿术后体液免疫和细胞免疫功能改变情况及与年龄的关系综述如下。     

Splenic implant in the jejunum: Radiographic and pathologic findings

We report a patient in whom barium studies and computed tomography revealed an intramural mass in the jejunum that consisted pathologically of ectopic splenic tissue. Because the patient had a prior splenectomy, the lesion probably represented splenosis rather than an accessory spleen. When intramural lesions are detected on barium studies in patients who have had prior abdominal trauma or splenectomy,^99mTc-tagged, heated-red blood cell scintigraphy may be performed to confirm the diagnosis, thereby eliminating the need for surgery in these patients.     

Does Profile of Hemoglobin Eβ-thalassemia Patients Change After Splenectomy? Experience of a Tertiary Thalassemia Care Centre in Eastern India

Hemoglobin Eβ-thalassemia is by far the commonest form of thalassemia intermedia. Its phenotype ranges from mild anemia to severe transfusion-dependency necessitating splenectomy in many patients. The present study aimed to systematically analyze both clinical as well as laboratory parameters in profile of Eβ-thalassemia patients after splenectomy in terms of transfusion requirement, infections and other complications. Retrospective study conducted over a period of 3 years included 72 cases of splenectomized Eβ-thalassaemia patients, considering decrease in transfusion requirements, new complications, antibiotic, anti-malarial prophylaxis and iron chelation therapy. Out of 1380 registered Eβ-thalassemia patients, 618 (44.78 %) were regularly transfused and 72(5.22 %) underwent splenectomy. Mean age of diagnosis was 10.3 years. Nineteen patients (26.4 %) underwent splenectomy between 5 and 10 years, 38 cases (52.7 %) between 10 and 20 years. The leading cause (51.39 %) for splenectomy was mechanical discomfort. Mean steady state hemoglobin raised from pre-splenectomy level of 5.43–6.8 gm/dl after splenectomy. Mean transfusion requirement reduced from 18.1 to 7.8 units/year. Mean serum ferritin level increased from 907.58 to 1,091.6 ng/ml. Post-splenectomy; 21 (29.17 %) patients developed facial deformities, 17 (23.6 %) delayed pubertal growth, 11 (15.28 %) venous thromboembolism, five (6.94 %) pulmonary hypertension and four (5.5 %) had extramedullary hematopoiesis. Five (6.96 %) patients had documented bacterial infections and two (2.78 %) suffered from malaria. Forty eight patients (66.67 %) started with iron chelation therapy; but majority (52.7 %) stopped. Major advantage of splenectomy is reduced transfusion requirement, though it cannot prevent skeletal abnormalities and delayed pubertal growth. In resource constraint countries like India, routine anti-malarial and antibacterial prophylaxis is not desirable; iron chelation therapy should be encouraged and ensured.     

Understanding of the significance and health implications of asplenia in a cohort of patients with haemaglobinopathy: possible benefits of a spleen registry

Objectives: Asplenia and hyposplenism carry a significant risk of ongoing morbidity and mortality which can be reduced by education, vaccination and antibiotic use. We aimed to assess education and other methods of prevention in a cohort of patients with haemoglobinopathy in a tertiary referral centre, which also had access to a post-splenectomy registry created to reduce post-splenectomy infection risk.

Methods: A standardized questionnaire was used on patients who attended the service for regular therapy. Patients were also asked about standard post-splenectomy preventive therapies including antibiotics and vaccinations.

Results: There were 49 patients who had either had a splenectomy or knew their spleen to be non-functional. Of these, nearly half knew themselves to be on the Victorian Spleen Registry (51.0%). The median knowledge score was 12 (range 4–17) out of a possible 18. Most significantly the benefits of the registry were not seen in terms of knowledge but in delivery of recommended vaccines and the use of a medical alert card.

Conclusion: This study examined knowledge and attitudes about splenectomy in a cohort of haemoglobinopathy patients in an Australian tertiary referral centre. The majority had good or fair knowledge with a strong association of some elements of post-splenectomy care with being placed on a spleen registry and having received targeted education. Implementation of systematic approaches by medical staff is likely to be the main benefit of a clinical registry approach in this setting.