Lymphocyte Blastogenic Responses in Sickle Cell Disease

Cell mediated immunity (CMI) has not been extensively studied in sickle cell disease (SCD). In this study, an assessment of lymphocyte blastogenic responses was done in 62 SCD patients (62 steady state and 25 in crisis with or without documented infection), along with 30 normal controls in good health and 10 with various infections. The mitogen phytohemagglutinin (PHA) and two antigens (Candida albicans and Tetanus Toxoid) were used as stimuli. An evaluation of these results by stimulation index revealed 53/62 (86%) of SCD steady state patients, 30/30 (100%) of healthy controls and 7/10 (70%) of normal control patients with infection positively responded to all three stimuli. Conversely, only 5/25 (20%) of SCD crisis state patients had totally normal blastogenic responses. Analysis of the data from SCD crisis patients revealed 14/20 (70%) with abnormally depressed proliferation to PHA, 11/20 (55%) to Candida and 6/20 (30%) to Tetanus. When comparisons were made by mean counts per minute, the most striking finding was the PHA response in the SCD crisis state. In this group the mean counts per minute were significantly less than the three other groups by statistical analysis. This data suggests that the crisis state, especially when associated with infection, profoundly effects blastogenic responses to PHA and to a lesser degree in vitro antigenic stimulation. The normal group with infection showed similar diminished blastogenesis to antigen, but no effect on mitogen response.     

Lymphocyte Blastogenic Responses in Sickle Cell Disease

Cell mediated immunity (CMI) has not been extensively studied in sickle cell disease (SCD). In this study, an assessment of lymphocyte blastogenic responses was done in 62 SCD patients (62 steady state and 25 in crisis with or without documented infection), along with 30 normal controls in good health and 10 with various infections. The mitogen phytohemagglutinin (PHA) and two antigens (Candida albicans and Tetanus Toxoid) were used as stimuli. An evaluation of these results by stimulation index revealed 53/62 (86%) of SCD steady state patients, 30/30 (100%) of healthy controls and 7/10 (70%) of normal control patients with infection positively responded to all three stimuli. Conversely, only 5/25 (20%) of SCD crisis state patients had totally normal blastogenic responses. Analysis of the data from SCD crisis patients revealed 14/20 (70%) with abnormally depressed proliferation to PHA, 11/20 (55%) to Candida and 6/20 (30%) to Tetanus. When comparisons were made by mean counts per minute, the most striking finding was the PHA response in the SCD crisis state. In this group the mean counts per minute were significantly less than the three other groups by statistical analysis. This data suggests that the crisis state, especially when associated with infection, profoundly effects blastogenic responses to PHA and to a lesser degree in vitro antigenic stimulation. The normal group with infection showed similar diminished blastogenesis to antigen, but no effect on mitogen response.     

琼脂石蜡双包埋切片在胸水细胞学诊断中的应用

目的探讨胸水琼脂石蜡双包埋切片在肿瘤细胞学诊断中的意义。方法对44例胸水沉淀物分别进行常规涂片和琼脂石蜡双包埋切片,并对琼脂石蜡双包埋切片进行免疫组织化学染色。结果在确诊的21例恶性胸水中,琼脂石蜡双包埋切片法检测癌细胞的阳性率为90.48%,显著高于涂片法52.38%。11例涂片法阳性的标本中,只有5例做出了病理分类诊断,而19例琼脂石蜡双包埋切片阳性的标本均做出了病理分类诊断。琼脂石蜡双包埋切片的免疫组织化学染色阳性结果定位准确,图象背景清晰。结论琼脂石蜡双包埋切片细胞集中,可以提高恶性胸水癌细胞的检出率和做出分型的病理诊断,利于以及进行免疫组织化学检测。     

Ethical Challenges in Hematopoietic Cell Transplantation for Sickle Cell Disease

Hematopoietic cell transplantation (HCT) using an HLA-identical sibling donor offers a very high likelihood of cure with good outcomes for patients with sickle cell disease (SCD), and alternative donor HCT for SCD is an area of active clinical research. Thus, HCT is a potential option for a growing number of patients with SCD. This expanded use of HCT has raised several ethical questions. Who is eligible for HCT, in terms of both disease severity and psychosocial factors? Should affected children with matched sibling donors undergo HCT only when they have declared themselves as having significant symptomatology? Regarding donors, special ethical challenges include the use of preimplantation genetic diagnosis to conceive an HLA-identical sibling. In this review, we critically analyze various ethical challenges related to HCT for SCD, and offer recommendations to guide clinical care.     

Diffuse Pleural Rhabdomyosarcoma with Persistent Pleural Effusion

A unique case of embryonal rhabdomyosarcoma arising at the left pleura of a 7-year-old Japanese girl is reported. The present case was characterized by persistent pleural effusion, and the malignant cells incidentally found in it were the first diagnostic clue. The tumor showed a rare growth pattern involving diffuse thickening of the parietal pleura. Biopsy of the thickened parietal pleura upon thoracotomy revealed embryonal rhabdomyosarcoma largely composed of immature mesenchymal cells. Immunohistochemical demonstration of creatinine phos-phokinase MM was most helpful among several types of immunostain for the histopathological diagnosis. Ultra-structurally, thin filaments with primitive Z bands could be seen in some tumor cells. Intensive clinical examination revealed only diffuse thickening of the parietal pleura, which was reduced by chemotherapy. This is the first documented case of rhabdomyosarcoma arising at the pleura. Previous reports of rhabdomyosarcoma arising at unusual sites are reviewed and the histogenesis of this tumor is briefly discussed. Acta Pathol Jpn 39: 803 809, 1989.     

Evaluation of Clinical Severity in Sickle Cell Disease

For a severity classification of sickle cell disease to be accepted, it is necessary that clinicians agree upon relative disease severity between patients. This condition was shown to be satisfied for a randomly selected group of patients evaluated by four persons. All rank correlation coefficients between observer pairs were highly significant. Representative severity indices based on history and recent hospital events also correlated significantly with evaluator ranking. The results show that, in principle, a classification of sickle cell disease patients by severity is possible. Such a classification would be most useful to evaluate the prognostic significance of particular signs or symptoms, or the success of various treatments in affecting severity of disease.     

Pneumococcal Sepsis in Children With Sickle Cell Disease

Pneumococcal sepsis has contributed to increased morbidity and mortality in patients with sickle cell disease. This case highlights the urgency of early diagnosis and the necessity of aggressive intervention in the management of sickle cell patients, particularly during the years of early childhood. It cannot be overemphasized that sickle cell patients, especially those less than 5 years of age, should be monitored carefully and institution of parenteral antibiotics should be undertaken early, even in those patients that present with mild symptoms.     

Depression, Disease Severity, and Sickle Cell Disease

The present study examined depressive symptomatology in 440 adults with sickle cell disease (SCD). Participants completed the Center for Epidemiologic Studies-Depression scale (CES-D) as part of their yearly routine visits to the Duke University-University of North Carolina Comprehensive Sickle Cell Center. They also completed questions regarding demographics, disease severity, pain, and health care use. Data analyses revealed that the percentage of patients with SCD exhibiting significant depressive symptomatology dropped from 43 to 18% when a more stringent cutoff was used on the CES-D, suggesting that future studies should determine the most valid cutoff score for identifying depression in patients with SCD. Gender and family income were positively and significantly associated with depressive symptomatology. Also, patients who reported more frequent painful episodes were more likely to report depressive symptoms. Implications for assessment and treatment of depression in adults with SCD are discussed.     

Efficient Clinical Counseling for Sickle Cell Disease

Sickle cell anemia (SCA) is a chronic illness that requires frequent health care visits for preventative management. Adherence to national guidelines such as the National Heart Lung and Blood Institute (NHLBI) Expert Panel Report on the Evidence-Based Management of Sickle Cell Disease can be challenging to both the clinician and the patient. Utilizing effective communication strategies with patients and their families can improve clinician/patient relationships, as well as adherence to national guidelines. Aims of this overview are to review challenges faced in outpatient subspecialty medicine and describe evidence-based techniques for more effective communication for patients with sickle cell anemia.     

The role of blood transfusion in Sickle Cell Disease

Blood transfusion is used in the treatment and prevention of acute and chronic complications of Sickle Cell Disease. Blood may be administered in the emergency situation as a simple top-up transfusion or as an automated or manual exchange procedure. Elective transfusions may be offered as an one-off procedure, often pre-operatively or as a long-term regime, usually for primary or secondary stroke prevention. The main complications of transfusion in this patient population are alloimmunization, hyperhaemolytic transfusion reactions and iron overload.     

Pruritus in Sickle Cell Disease: Response to Cholestyramine

Even in the absence of gallstones, the hepatobiliary system should be suspected as a possible cause of pruritus in a patient with sickle cell disease. A case is presented in which a sickle cell patient had severe pruritus relieved by cholestyramine therapy.     

Granulocyte Colony-Stimulating Factor-Based Stem Cell Mobilization in Patients with Sickle Cell Disease

Granulocyte colony-stimulating factor (G-CSF) has been reported to exacerbate vaso-occlusive crises in sickle cell disease. It has been recommended to avoid its use for stem cell mobilization in this population, yet autologous transplant is the standard of care and at times a life-saving treatment for patients with various hematologic malignancies such as relapsed aggressive lymphoma or multiple myeloma. We report 5 cases of patients with sickle cell disease and related hemoglobinopathies who underwent granulocyte-colony stimulating factor (G-CSF)-mobilization of peripheral blood stem cells (PBSC). Three of them developed manageable vaso-occlusive pain symptoms requiring parenteral narcotics alone. The 2 others had no complications. These cases demonstrate that stem cell mobilization using G-CSF, although complicated and not without risk, is feasible in patients with sickle cell syndromes.