Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study

Prophylactic blood transfusion has come to be regarded as necessary in the treatment of patients with sickle cell disease during pregnancy. Because of the risks associated with blood products and reports of successful outcomes without the use of blood transfusion, we conducted a prospective randomized controlled study of this issue. Seventy-two pregnant patients with sickle cell anemia were randomly assigned to one of two treatment groups: 36 received prophylactic transfusions of frozen red cells, and 36 received red-cell transfusions only for medical or obstetric emergencies. Twenty-eight patients with sickle cell anemia who did not qualify for randomization (mainly because they had other medical disorders), 66 with sickle cell-hemoglobin C disease, and 23 with sickle cell-beta-thalassemia were also followed and received transfusions only for emergencies. There was no significant difference in perinatal outcome between the offspring of mothers with sickle cell disease who were assigned to treatment with prophylactic transfusions and those who were not (15 vs. 5 percent). The occurrence of a perinatal death in a previous pregnancy and the presence of twins in the present pregnancy were two major risk factors for an unfavorable outcome; when they were present, perinatal mortality was 50 percent. Perinatal mortality was somewhat higher in the two groups that were randomized than in the three groups that were not. Prophylactic transfusion significantly reduced the incidence of painful crises of sickle cell disease (P less than 0.01) and substantially reduced the cumulative incidence of other complications of this disorder (P = 0.07). Other medical and obstetric complications occurred with nearly equal frequency in the two randomized groups. Increases in costs, the number of hospitalizations, and the risk of alloimmunization were disadvantages of prophylactic transfusion. We conclude that the omission of prophylactic red-cell transfusion will not harm pregnant patients with sickle cell disease or their offspring.     

Pattern of Serum Cytokine Expression and T-Cell Subsets in Sickle Cell Disease Patients in Vaso-Occlusive Crisis

The pathogenesis of sickle vaso-occlusive crisis (VOC) in sickle cell disease (SCD) patients involves the accumulation of rigid sickle cells and the stimulation of an ongoing inflammatory response, as well as the stress of infections. The immune response, via cytokine imbalances and deregulated T-cell subsets, also has been proposed to contribute to the development of VOC. In this study, a panel of high-sensitivity cytokine kits was used to investigate cytokines in the sera of SCD patients in VOC. The results were compared primarily with those for stable SCD patients and secondarily with those for normal healthy people who served as controls. The cytokines studied included interleukin-2 (IL-2), IL-4, and IL-10. Lymphocyte subsets of patients with VOC were also studied and were compared with those of both control groups (20 stable patients without crisis [SCD group] and 20 normal healthy controls [NHC]). The VOC group was notable for remarkably elevated levels of IL-4, among the three cytokines tested, compared with those for the SCD and NHC groups. Patients with VOC also differed from stable SCD patients and NHC by having notably lower IL-10 levels, as well as the lowest ratio of CD4⁺ to CD8⁺ T cells (0.7). The patterns of the proinflammatory cytokine IL-2 did not differ between VOC and stable SCD patients, but NHC had significantly lower IL-2 levels than both the VOC and SCD groups. Our results demonstrate coexisting levels, both high and low, of TH1- and TH2-type cytokines, as well as diminished levels of T-cell subsets in VOC. These results are discussed in an effort to better understand the importance of the immune system profile in the pathogenesis of sickle cell VOC. Since the possibility that a cytokine imbalance is implicated in the pathogenesis of sickle cell crisis has been raised, our results should prompt further investigation of the host immune response in terms of TH1 and TH2 balance in sickle cell crisis.Sickle cell disease (SCD) is a chronic, incurable condition presenting primarily as anemia (sickle cell anemia [SCA]) in people homozygous for hemoglobin S (HbS). This abnormal hemoglobin, resulting from the replacement of glutamic acid at position 6 of the β-globin chain by valine, is responsible for erythrocyte distortion and fragility in these patients, as well as for thrombosis, fever, splenomegaly, joint pain, lethargy, and weakness. Sickle cell crises refer to the sudden attacks of pain, at various levels of severity, that occur during the lifetime of the patient with sickle cell disease (1, 3). Of these, the painful vaso-occlusive crisis (VOC) is the most common and is characterized by fever, leukocytosis, joint effusions, and tenderness, which occur in about 50% of patients at initial presentation (2), as well as by susceptibility to infection. It is a medical emergency and an acute crisis state. Patients in a state of well-being between these episodes are referred to as “steady-state” SCD patients.The sequence of pathophysiological events that lead to the sickle cell VOC is not well understood. Several authors (8, 13, 27, 28) have outlined a sequence of steps occurring in the microcirculation that culminate in this painful sickle cell crisis. Polymerization of HbS, decreased blood red cell flexibility, microvascular occlusion, hypoxia of tissue involved with the occluded microvascular network, and tissue damage triggering painful stimuli have been mentioned (26), although the precise dynamics of these events and their interrelationships are poorly understood. Tissue ischemia due to vascular occlusion causing infarctive tissue damage, which in turn initiates secondary inflammatory responses, has also been mentioned (3, 4). Ischemic events produced by the occlusion of both large and small blood vessels are stressful and involve intricate interactions between red blood cells, the endothelium, and leukocytes (7). These interactions are known to be regulated by cytokines secreted by T cells as well as by adhesion molecules, and consequently, the immune response is implicated in the initiation and development of the sickle cell crisis. Indeed, studies now show that immune subsets are operative in sickle cell disease (9, 14, 16, 25), and the susceptibility of sickle cell disease patients in crisis to infections that specifically require the help of T cells to be cleared, such as Salmonella enterica serovar Typhimurium osteomyelitis (14), is suggestive.CD4⁺ T cells, subdivided based on their associated cytokines, play a crucial role in inflammatory responses and the elimination of infection. TH1 cells provide immunity against intracellular pathogens by secreting the cytokines interleukin-2 (IL-2), IL-12, and gamma interferon (IFN-γ), whereas commitment to the TH2 lineage programs the clearance of extracellular pathogens and the secretion of cytokines such as IL-10, IL-4, and IL-13. This balance of TH1/TH2 cytokine responses is believed to play an important role in coordinating an effective immune response, even under inflammatory conditions, although very limited data exist on their roles in sickle cell VOC.This study thus hypothesizes that the balance between TH1- and TH2-type cytokines might explain the differences in clinical outcomes in sickle cell disease. It was undertaken with patients with SCD in VOC in Zaria, Nigeria, a town in the zone of sickle cell endemicity of West Africa (17). The study analyzed numerical values for CD3⁺, CD4⁺, and CD8⁺ T cells and levels of selected serum cytokines in patients in VOC, and it compared these values with those obtained for steady-state SCD patients and unaffected hemoglobin AA homozygotes who served as normal healthy controls (NHC). This was done in an effort to understand if any imbalance in the immune response is important in the pathogenesis of sickle cell disease.     

Benign monoclonal expansion of CD8+ lymphocytes in HIV infection

BACKGROUND: A transient expansion of the CD8+ T cell pool normally occurs in the early phase of HIV infection. Persistent expansion of this pool is observed in two related settings: diffuse infiltrative lymphocytosis syndrome (DILS) and HIV associated CD8+ lymphocytosis syndrome. AIM: To investigate a group of HIV infected patients with CD8+ lymphocytosis syndrome with particular emphasis on whether monoclonality was present. METHODS: A group of 18 patients with HIV-1 infection and persistent circulating CD8+ lymphocytosis was compared with 21 HIV positive controls. Serum samples were tested for antinuclear antibodies, antibodies to extractable nuclear antigens, immunoglobulin levels, paraproteins, human T lymphotropic virus type 1 (HTLV-1), Epstein-Barr virus, and cytomegalovirus serology. Lymphocyte phenotyping and HLA-DR typing was performed, and T cell receptor (TCR) gene rearrangement studies used to identify monoclonal populations of T cells. CD4+ and CD8+ subsets of peripheral blood lymphocytes were purified to determine whether CD8+ populations inhibited HIV replication in autologous CD4+ cells. RESULTS: A subgroup of patients with HIV-1 infection was found to have expanded populations of CD8+ T cell large granular lymphocytes persisting for 6 to 30 months. The consensus immunophenotype was CD4- CD8+ DRhigh CD11a+ CD11c+ CD16- CD28+/- CD56- CD57+, consistent with typical T cell large granular lymphocytes expressing cellular activation markers. Despite the finding of monoclonal TCR gene usage in five of 18 patients, there is evidence that the CD8+ expansions are reactive populations capable of mediating non-cytotoxic inhibition of HIV replication. CONCLUSIONS: A subgroup of HIV positive patients has CD8+ lymphocytosis, but despite the frequent occurrence of monoclonal TCR gene usage there is evidence that this represents an immune response to viral infection rather than a malignant disorder.     

T lymphocyte subsets and NK cell cytotoxicity in chronic hemodialysis patients. The effect of recombinant human erythropoietin (rHu-EPO) treatment.

We investigated subpopulations of T lymphocytes, NK cell number and cytotoxic activity in 14 chronic uremic patients on regular hemodialysis treatment. We observed a significantly decreased absolute lymphocyte number and percentage of CD3 cells. Relative numbers of CD16 cells were significantly elevated, but NK cell cytotoxic activity was within a normal range. Nine patients with chronic renal anemia on maintenance hemodialysis were enrolled in rHu-EPO treatment trial. The treatment was continued till the hematocrit level reached 30%. Each of the patients had corrected anemia and well-being. After 12 weeks of the treatment we observed in these patients decreases in CD3, CD4, CD8 and CD16 cell numbers and elevation of CD4/CD8 ratio. Cytotoxic activity of NK cells did not change significantly. Presented results indicate that chronic hemodialysis patients have significantly diminished lymphocyte number. rHu EPO treatment affects the T lymphocyte subsets inducing a deep decrease of CD8 and CD16 cell percentage leading to normalisation of the CD4/CD8 ratio.     

Studies of the kallikrein-kinin system in patients with sickle cell anemia

Eight patients with sickle cell anemia (SS hemoglobin) were found to have decreased plasma levels of prekallikrein compared to normal control subjects or patients with other types of anemia. The prekallikrein levels in the patients with sickle cell anemia were found to decrease further during a sickle cell crisis. These results suggest that components of the kallikrein-kinin system are profoundly affected in patients with sickle cell anemia, and during crises may play a role in the clinical presentation of patients.     

CD8 lymphocytosis in primary cytomegalovirus (CMV) infection of allograft recipients: expansion of an uncommon CD8+ CD57- subset and its progressive replacement by CD8+ CD57+ T cells.

Allograft recipients undergoing cytomegalovirus infection present increased proportions of circulating CD8+ lymphocytes. A longitudinal study of 11 kidney and five liver allograft recipients with primary CMV infection but no other etiological factor of graft dysfunction revealed selective imbalances of peripheral blood CD8+ T cell subsets. Initially, CMV viraemia is associated with elevated CD8+bright T cell numbers and T cell activation. Activation markers fall to normal when viral cultures become negative (before the end of the first month). During the second to sixth month, most (12/16) patients keep up high CD8+ T cell counts (1050-2900 CD8+ cells/mm3), comprising an uncommon CD8+ T cell subset, as 45-73% of CD8+bright lymphocytes were CD3+ and TCR alpha beta+, but were not stained by anti-CD28, CD11b, CD16, CD56, and CD57 antibody. Unexpectedly, CD8+CD57+ T cells, a hallmark of CMV infection, do not appear until the second to sixth month of primary CMV infection, and their numbers increase progressively thereafter. They become the predominant CD8+ T cell subset after 6 months of infection and their persistence for several (up to 4) years is strongly correlated (r = 0.87) with expansion of CD8+ cells. By analysis with MoAbs, there was no bias towards the use of particular TCR-V beta gene families at any time of primary CMV infection. Persistence of CD8 lymphocytosis is thus directly related to the rate of expansion of an uncommon CD8+CD57- subset and its progressive replacement by CD8+CD57+ T cells that are chronically elicited by CMV.     

Activated T cells and eosinophilia in bronchoalveolar lavages from subjects with asthma correlated with disease severity.

Activated T-lymphocytes may regulate the eosinophilic inflammation of bronchial asthma. In the present study, we investigated T cell activation and eosinophilia in blood and bronchoalveolar lavage (BAL) in 17 patients with asthma not receiving steroid treatment. Compared to normal individuals, BAL from patients with asthma contained significantly increased numbers of both lymphocytes and eosinophils (EOSs). The lymphocytosis consisted of increased numbers of both CD4+ and CD8+ T cells, and these T cell populations expressed elevated levels of T cell activation markers (interleukin-2 receptor [CD25], HLA-DR, and very late activation antigen 1). Close correlation was found between numbers of BAL CD4+ IL-2R+ T cells and numbers of EOSs. Moreover, the numbers of activated T cells and EOSs were related to the severity of asthma as measured by impairment of FEV1 and increased methacholine bronchial responsiveness. We demonstrate in both blood and BAL a close correlation between T cell activation, eosinophilia, and severity of asthma, suggesting that recruitment and activation of lymphocytes and EOSs are fundamental to the pathogenesis of bronchial asthma.     

Red cell distribution width in sickle cell disease

Red cell distribution width (RDW), an electronically determined index of anisocytosis, was examined in 60 patients with sickle cell anemia (Hb SS), 28 patients with hemoglobin sickle cell (SC) disease, and seven patients with sickle cell-beta(+) thalassemia (S-thal). All patients were adults and in the steady state of their disease. The RDW was greater in sickle cell patients than in 39 healthy, age and race matched controls without hemoglobinopathy (Hb AA). Patients with sickle cell anemia had higher mean RDW than those with Hb SC disease or with S-thal. The mean RDWs in the latter two disorders were not significantly different. In SS patients, the RDW correlated significantly with the degree of anemia and reticulocytosis. A group of 18 SS patients was studied while in acute painful crisis. Their mean RDW was not different from that in the steady state. Mean WBC and red cell volume, however, were significantly higher during pain crisis.     

Alternate Pathway Activation in Sickle Cell Disease and ��-Thalassemia Major

Total hemolytic complement activity (CH50), immuno-electrophoretic conversion of Factor B (C3PA), and of C3 were studied in 16 patients with sickle cell disease in a steady state, eight patients in crisis, and ten patients with β-thalassemia major anemia maintained on a constant transfusion regimen. Patients with sickle cell disease in a steady state have moderatley 56 (percent) depressed conversion of Factor B in addition to markedly decreased conversion of C3 in four of ten patients. One of the three sickle cell patients and two of the four thalassemia patients with low C3 conversion levels have died subsequent to the studies. The combination of chronically decreased Factor B conversion in the face of markedly decreased C3 conversion may make these patients occasionally vulnerable to overwhelming infection analagous to the situation seen in postsplenectomy cases.     

Clinicobiological, immunophenotypic, and molecular characteristics of monoclonal CD56-/+dim chronic natural killer cell large granular lymphocytosis

Indolent natural killer (NK) cell lymphoproliferative disorders include a heterogeneous group of patients in whom persistent expansions of mature, typically CD56(+), NK cells in the absence of any clonal marker are present in the peripheral blood. In the present study we report on the clinical, hematological, immunophenotypic, serological, and molecular features of a series of 26 patients with chronic large granular NK cell lymphocytosis, whose NK cells were either CD56(-) or expressed very low levels of CD56 (CD56(-/+dim) NK cells), in the context of an aberrant activation-related mature phenotype and proved to be monoclonal using the human androgen receptor gene polymerase chain reaction-based assay. As normal CD56(+) NK cells, CD56(-/+dim) NK cells were granzyme B(+), CD3(-), TCRalphabeta/gammadelta(-), CD5(-), CD28(-), CD11a(+bright), CD45RA(+bright), CD122(+), and CD25(-) and they showed variable and heterogeneous expression of both CD8 and CD57. Nevertheless, they displayed several unusual immunophenotypic features. Accordingly, besides being CD56(-/+dim), they were CD11b(-/+dim) (heterogeneous), CD7(-/+dim) (heterogeneous), CD2(+) (homogeneous), CD11c(+bright) (homogeneous), and CD38(-/+dim) (heterogeneous). Moreover, CD56(-/+dim) NK cells heterogeneously expressed HLA-DR. In that concerning the expression of killer receptors, CD56(-/+dim) NK cells showed bright and homogeneous CD94 expression, and dim and heterogeneous reactivity for CD161, whereas CD158a and NKB1 expression was variable. From the functional point of view, CD56(-/+dim) showed a typical Th1 pattern of cytokine production (interferon-gamma(+), tumor necrosis factor-alpha(+)). From the clinical point of view, these patients usually had an indolent clinical course, progression into a massive lymphocytosis with lung infiltration leading to death being observed in only one case. Despite this, they frequently had associated cytopenias as well as neoplastic diseases and/or viral infections. In summary, we describe a unique and homogeneous group of monoclonal chronic large granular NK cell lymphocytosis with an aberrant activation-related CD56(-/+dim)/CD11b(-/+dim) phenotype and an indolent clinical course, whose main clinical features are related to concomitant diseases.     

Transient stress lymphocytosis: an immunophenotypic characterization of the most common cause of newly identified adult lymphocytosis in a tertiary hospital

We prospectively evaluated 52 consecutive cases of newly identified absolute lymphocytosis to determine the hematologic and immunophenotypic features of transient stress lymphocytosis (TSL). The lymphocytosis in all cases was associated with an acute stressful event and ranged from 4,000 to 10,400/microL (4.0-10.4 x 10(9)/L). Compared with healthy individuals, patients with TSL showed an increase in the total WBC, absolute lymphocyte (ALC), absolute neutrophil (ANC), and platelet counts with no difference in hemoglobin levels. Immunophenotypic analyses of 38 cases revealed increases in absolute numbers of T B, and natural killer cells. Both CD4+ and CD8+ T cells were increased, predominantly accountedfor by an increase in memory cell subsets, with no change in gamma/delta T cells. Follow-up studies showed a significant reduction in the ALC with a concurrent increase in the ANC and reduction in hemoglobin values. The reduction in lymphocytes at resolution was accompanied by reduction in all broad Iymphocyte subsets. However, naive and memory subsets showed different patterns of alteration within the CD4+ and CD8+ populations, suggesting that acute stress differentially affects the in vivo distribution of these subsets.     

Alloimmunization in sickle cell anemia and transfusion of racially unmatched blood

Transfusion therapy for sickle cell anemia is limited by the development of antibodies to foreign red cells. To evaluate the frequency and risk factors associated with such alloimmunization, we determined the transfusion history, red-cell phenotype, and development of alloantibodies in 107 black patients with sickle cell anemia who received transfusions. We compared the results with those from similar studies in 51 black patients with sickle cell disease who had not received transfusions and in 19 nonblack patients who received transfusions for other forms of chronic anemia. We assessed the effect that racial differences might have on the frequency of alloimmunization by comparing the red-cell phenotypes of patients and blood-bank donors (n = 200, 90 percent white). Although they received transfusions less frequently, 30 percent of the patients with sickle cell anemia became alloimmunized, in contrast to 5 percent of the comparison-group patients with other forms of anemia (P less than 0.001). Of the 32 alloimmunized patients with sickle cell anemia, 17 had multiple antibodies and 14 had delayed transfusion reactions. Antibodies against the K, E, C, and Jkb antigens accounted for 82 percent of the alloantibodies. Comparison of red-cell phenotypes in the three study groups (the patients with sickle cell anemia, the patients with other forms of anemia, and the blood donors) revealed statistically significant differences between the patients with sickle cell anemia and the donors but not between the patients with other forms of anemia and the donors. These differences are most likely racial. We conclude that alloimmunization is a common, clinically serious problem in sickle cell anemia and that it is partly due to racial differences between the blood-donor and recipient populations.     

Preferential reductions in lymphocyte sub-populations induced by monthly pulses of chlorambucil: studies in patients with chronic progressive multiple sclerosis.

Thirty-three patients with chronic progressive multiple sclerosis (MS) were assigned to intervention groups receiving monthly pulses of chlorambucil (CB) for about one year. The monthly doses ranged from 0.4 to 1.5 mg/kg. Administration of CB resulted in preferential reduction in different lymphocyte subsets which was dose- and time-dependent. The number of B-cells (CD20) decreased more rapidly than NK-cells (CD16, CD56, CD16+CD56+) or T-cell (CD3) and T-cells subsets (CD4 and CD8). At 1.2 mg/kg, CB administration resulted in a preferential drop of T-suppressor/cytotoxic cells (CD8) compared with T-helper cells (CD4), and of the less mature "virgin" CD4 cells (CD4+CD45RA+) compared with "memory" CD4 cells (CD4+CD45RA-). The expression of activation markers (transferrin receptor, CALLa, HLA-Dr and CD38[OKT10]) within CD4, CD8 or CD20 lymphocytes was not altered by CB administration. Our data, which show that CB administration results in a preferential fall in B-cell numbers, contrast with the effects of long-term administration of the related immunosuppressive drugs, azathioprine and cyclophosphamide.     

侵袭性NK细胞白血病的临床病理学特点

目的 探讨侵袭性NK细胞白血病(ANKL)的临床病理学特点.方法 回顾性分析10例ANKL患者的临床病理档案资料,全部病例均行全血细胞计数以及外周血涂片、骨髓穿刺与骨髓活检标本的形态学观察.用流式细胞学(FCM)及免疫组织化学(EliVision法)进行免疫表型分析.聚合酶链反应(PCR)法检测T细胞受体(TCR)γ基因重排.结果 10例患者中,最常见的血液学异常为贫血(7例)与血小板减少(9例).6例外周血涂片可见大颗粒淋巴细胞.骨髓穿刺涂片示8例淋巴细胞比例增高(＞20.0％).6例可见大颗粒淋巴细胞.骨髓活检切片示轻度浸润5例,中度浸润3例,重度浸润2例.骨髓切片中8例为间质型浸润,2例呈弥漫型浸润,4例可见噬血现象(吞噬成熟红细胞).免疫表型方面,FCM检测示全部病例为CD2+sCD3- CD4- CD56+CD57-.9例CD7、5例CD16、4例CD8和1例CD5阳性.8例行免疫组织化学相关抗原检测:cCD3 4例、CD566例、T细胞内抗原1(TIA-1)6例、颗粒酶B4例和穿孔素2例阳性.10例TCRγ基因重排检测均为胚系构型.结论 ANKL是一种NK细胞来源的高度侵袭性淋巴组织肿瘤,需进行全面的外周血与骨髓的形态学、免疫表型以及分子遗传学检测才能确诊,需注意与多种NK细胞与T细胞淋巴瘤鉴别.     

CD4/Leu7 and CD8/Leu7 large granular lymphocytosis: comparative studies between NK cells and T cells

Lymphocytes, co-expressing CD4/Leu7 and CD8/Leu7 markers respectively, taken from two patients having large granular lymphocytosis taking an indolent clinical course have been comparatively studied for function as NK cells and T cells. Both large granular lymphocytes (LGLs) were acid phosphatase positive and showed a beta-glucuronidase reaction in their cytoplasmic granules. Studies on case 1 indicated that the CD4/Leu7 lymphocytosis with LGL morphology takes a benign clinical course with mild neutropenia as well as those of CD8/Leu7 LG lymphocytosis. Both CD4/Leu7 and CD8/Leu7 LGLs behave similarly in their lack of NK activity, and manifest decreased IL-2 production in vitro and show a low IL-2 receptor expression unrelated to their T cell phenotype, but behave differently in influencing the immunoglobulin production in vitro and the ADCC activity, depending on their T cell phenotype and on the expression of Fc receptor, respectively. Furthermore, the altered Fc receptors which were undetectable by the Leul 1 antibody but were still effective for ADCC activity might be present in case 2 LGLs.     

An unusual association of Felty syndrome and TCR gamma delta lymphocytosis.

Felty syndrome, comprised of neutropenia, rheumatoid arthritis and splenomegaly, occurs in approximately 1% of patients with rheumatoid arthritis. Up to one third of these patients have an increased number of large granular lymphocytes. The usual immunophenotype of these cells is CD3+, CD8+, CD57+, T cell receptor (TCR) alpha beta. A patient with Felty syndrome and large granular lymphocytosis, who had an unusual immunophenotype CD3+, CD4-, CD8-, TCR gamma delta, is described. Her neutropenia responded to treatment with granulocyte colony stimulating factor (G-CSF), which was given in order to raise her neutrophil count prior to bilateral knee replacement surgery. Thus, Felty syndrome with large granular lymphocytosis is a heterogeneous condition, one in which TCR gamma delta large granular lymphocytosis may be found, and also shows a response to treatment with G-CSF.     

Effectiveness of treatment during osteoarticular pain crises in drepanocytosis; based on the example of pentoxifylline

Many drugs have been used for prevention and treatment of vaso-occlusive attacks in sickle cell anemia. Pentoxifylline is one of the most recent. It increases deformability and filtrability of normal or sickled red cells. In this double-blind study it is compared with a placebo for treatment of 20 osteoarticular crisis during SS or SC sickle cell anemia in Mali. Pentoxifylline did not decrease intensity nor duration of crisis. On the other hand the clinical assessment used for testing drugs efficiency over pain seemed effective and reproducible.     

Assessment of the use of transfusion therapy and complications in orthopedic surgery in patients with sickle-cell anemia: retrospective study]

Red blood cells (RBCs) transfusion is a common practice in the treatment or for the prevention of complications of patients with sickle-cell disease. In surgery, pre-operative transfusions are frequently given to prevent peri-operative complications. There is no consensus however on the best regimen of transfusion for this purpose. The transfusion techniques are muliple. In addition, pre-operative transfusion therapy is reported to be largely responsible for an increased morbidity and mortality in patients with sickle cell anemia undergoing surgery. During the period 1990-2000, 16 patients (4 men and 12 women) with a mean age of 37 years and various major sickle cell hemoglobinopathies underwent 32 total hip arthroplasty for femoral head necrosis. Nine patients with sickle-cell trait were included as control group. Twelve of them had haemoglobin SS (HbSS), 2/16 had HbSC, 2/16 had HbS/betathalassemia. Operative transfusion were given in only 12/32 procedures, 4 were performed pre-operatively and 8 intra-operatively. Simple transfusion (mean: 2.5 packed red cells) were administered in all the procedures. The main complications observed in our patients were anemia by hemolysis and haemorrhagic shock, vaso-occlusive crisis and chest syndrome. Anemia requiring transfusions was significatively related to the procedures with pre-operative transfusion. In the light of our result, we would like to propose transfusional protocol--if needed--only intra-operatively.     

再生障碍性贫血T细胞亚群及NK细胞活性测定与疗效的关系

本文报道２０例再障患者ＣＤ３＋、ＣＤ４＋、ＣＤ４＋／ＣＤ８＋比值显著低于正常对照组（Ｐ＜０．０５），ＮＫ细胞活性显著低于正常对照组（Ｐ＜０．０１）。其中１０例经ＣＳＡ和ＡＯｄｒｉｏｌ（安雄）治疗，８例疗效好的患者ＣＤ４＋／ＣＤ８＋比值和ＮＫ细胞活性显著升高，提示测定ＣＤ４＋／ＣＤ８＋比值及ＮＫ细胞活性可作为观察再障疗效的一个指标。