HTLV-III serology in hemophilia: relationship with immunologic abnormalities

We investigated the relationship of the presence of antibodies to HTLV-III and immunologic abnormalities in patients with hemophilia. Serum antibodies to HTLV-III were analyzed by ELISA assay, immunoprecipitation of labeled cell extracts, and immunoprecipitation of purified HTLV-III p24. Thirty-four (61%) of the total group (n = 56) had antibody to HTLV-III; 34 (76%) of 45 patients given commercial factor VIII preparations were seropositive, compared with none of 11 patients treated exclusively with cryoprecipitate obtained from volunteer blood donors. Of patients who were seropositive for HTLV-III antibody, 94% had abnormal T4/T8 ratios, and 33% of those whose serum was antibody negative had abnormal T4/T8 ratios; five patients, each antibody positive, have lymphadenopathy syndrome. Sequential studies in a subset of patients indicate that there is a changing pattern of antibody production to HTLV-III antigens after seroconversion.     

Bone marrow features in children with HIV infection and peripheral blood cytopenias

HIV infection is associated with numerous abnormalities affecting both the myeloid and lymphoid lineages. We studied the features associated with peripheral cytopenias as the first sign of HIV infection in children. Peripheral blood (PB) counts, PB and bone marrow (BM) lymphocyte subsets, as well as viral load and serum levels of ferritin, vitamin B12, and folic acid were determined. Five children were naive of treatment (Group 1) and three were under HAART (Group 2). In Group 1 all patients had anemia of chronic disease. One had a bone marrow culture positive for Mycobacterium avium intracellulare and pancytopenia. Besides this, neutropenia and thrombocytopenia were seen in one patient each. In Group 2 anemia was found in all, neutropenia in one, and thrombocytopenia in two patients. Peripheral blood cytopenias were due to HAART toxicity in one patient. In the other two they were due to iron or folate deficiency. Bone marrow cytology showed cell abnormalities mainly in granulocytic precursors and megakaryocytes. All except two (taking HAART) patients had a high viral load. There was a straight correlation between viral load in PB and bone marrow. Viral load was correlated with peripheral CD4 but not with CD8 lymphocytes. A decrease in bone marrow B lymphocytes was seen in all patients. The introduction of HAART improved peripheral cytopenias. Bone marrow examination was useful for determining the etiology of the cytopenias and for detection of opportunistic infection. Hemopoietic cell abnormalities were similar to those seen in adults and indicative of HIV infection.     

Hematologic findings associated with thrombocytopenia during the acute phase of exanthem subitum confirmed by primary human herpesvirus-6 infection

PURPOSE: To elucidate the mechanism underlying thrombocytopenia during the acute phase of exanthem subitum (ES), the associated hematological findings were investigated. PATIENTS AND METHODS: Five infants with thrombocytopenia during the acute phase of ES serologically confirmed by primary human herpesvirus-6 (HHV-6) were examined and followed-up. RESULTS: Thrombocytopenia was accompanied by neutropenia, leukopenia, and decreased reticulocyte fraction during the acute phase. These changes were self-limiting, and the sequential changes of platelet, neutrophil count, and reticulocyte fraction were closely linked. Slight but significant decreases in hemoglobin in the convalescent phase and mild increases in atypical lymphocytes after subsidence of the fever were observed. Hemophagocytosis and increase in atypical lymphocytes in the bone marrow suggested that bone marrow cells were influenced by primary HHV-6 infection. Platelet-associated immunoglobulin G and indirect antiplatelet antibody were negative. Plasma levels of fibrinogen and D-dimer of fibrinogen degradation products were within normal ranges. CONCLUSIONS: Thrombocytopenia is a complication of ES, and this may result from bone marrow suppression rather than from immune-mediated peripheral consumption seen in acute idiopathic thrombocytopenic purpura or from disseminated intravascular coagulation.     

Management of human immunodeficiency virus-associated thrombocytopenia with intravenous gamma globulin

A 17-month-old boy in whom immune-mediated thrombocytopenia (ITP) was the presenting manifestation of infection with human immunodeficiency virus (HIV) is being successfully managed with intermittent high-dose intravenous gamma globulin (IVIG) allowing maintenance of hemostatic platelet counts while avoiding the immunosuppression associated with other therapeutic modalities used to treat ITP. He continues to demonstrate marked responsiveness to IVIG, and has been maintained on weekly or bimonthly infusions for 12 months. The serendipitous documentation of HIV infection prior to IVIG therapy for immune-mediated thrombocytopenia in this child documents the importance of HIV testing prior to IVIG therapy to prevent erroneous assignment of IVIG as the vehicle responsible for transmission of HIV infection. This case history also documents the importance of HIV testing in the diagnostic evaluation of immune-mediated thrombocytopenias.     

Selective defect of precursor T cells associated with apparently normal B lymphocytes in severe combined immunodeficiency disease.

Two patients, one with an autosomal and the other a sex-linked form of severe combined immunodeficiency, had more than 95% B cells in their peripheral blood. Despite an increased absolute number of B lymphocytes, the patients were unable to produce serum antibodies. In each patient, geno- or pheno-identical bone marrow transplantation was followed by the visualization of a thymus shadow and the appearance of both cellular and humoral functions. Chromosome of allotype studies showed that the T cell originated from the donor whereas serum immunoglobulins were synthesized by host B cells. In these patients the pathogenesis appears to be a selective defect of bone marrow precursor T cells without concomitant intrinsic B cell defect. The successful outcome of the graft in these two patients, who are now, respectively, 5 years and 11 months of age and free of infections, indicates that the preferred form of therapy in such patients is transplantation of bone marrow stem cells, which populate the thymus and mature slowly into T cells that cooperate fully with host B cells in synthesis of antibody.     

Agenesis of the Corpus Callosum and Neural Crest Tumors

Hematologic abnormalities, including thrombocytopenia, are seen in HIV infection. Mi have previously reported elevated platelet-associated IgG (PAIgG) in thrombocytopenia in children associated with human immunodeficiency virus (HIV). In this study we prospectively monitored 40 HIV-infected infants and children to determine the significance of elevated PAIgG levels as they relate to thrombocytopenia. We also examined platelet eluatesfor the presence of HIV antibody and antigen. Of 16 patients with thrombocytopenia, 15 (93.7%) had elevated PAIgG. Of 24 patients with normal platelet counts, 21 (87.5%) had elevated PAIgG. On follow-up, none of the children with normal platelet counts and elevated PAIgG levels developed thrombocytopenia. Examination of the platelet eluates was negative for HIV antibody or P24 antigen. Although the sensitivity of an elevated PAIgG level in predicting thrombocytopenia is 93%, its specificity is only 13%. Elevated PAIgG levels are therefore not causally related to the development of thrombocytopenia in children.     

Platelet-Associated IgG in Pediatric HIV Infection

Hematologic abnormalities, including thrombocytopenia, are seen in HIV infection. Mi have previously reported elevated platelet-associated IgG (PAIgG) in thrombocytopenia in children associated with human immunodeficiency virus (HIV). In this study we prospectively monitored 40 HIV-infected infants and children to determine the significance of elevated PAIgG levels as they relate to thrombocytopenia. We also examined platelet eluatesfor the presence of HIV antibody and antigen. Of 16 patients with thrombocytopenia, 15 (93.7%) had elevated PAIgG. Of 24 patients with normal platelet counts, 21 (87.5%) had elevated PAIgG. On follow-up, none of the children with normal platelet counts and elevated PAIgG levels developed thrombocytopenia. Examination of the platelet eluates was negative for HIV antibody or P24 antigen. Although the sensitivity of an elevated PAIgG level in predicting thrombocytopenia is 93%, its specificity is only 13%. Elevated PAIgG levels are therefore not causally related to the development of thrombocytopenia in children.     

Mucosal cell-mediated immunity to varicella zoster virus: role in protection against disease

By indirect immunofluorescence, enzyme-linked immunosorbent assay, and in vitro lymphocyte proliferation, we studied the antibody and cell-mediated immune response to varicella zoster virus (VZV) in serum, peripheral blood lymphocytes, and tonsillar lymphocytes in 49 children before and after tonsillectomy and adenoidectomy. Among the naturally infected patients seropositive for VZV antibody, most demonstrated VZV-specific proliferation in the peripheral blood and tonsillar lymphocytes, with activity consistently higher in the tonsillar lymphocytes. Several patients seronegative for VZV antibody and without a prior history of clinical chickenpox also manifested VZV-specific proliferation in the tonsillar lymphocytes, and less frequently in peripheral blood lymphocytes. Of these, six children with high levels of activity in tonsillar lymphocytes, with or without high levels in the peripheral blood lymphocytes, failed to develop disease after intimate exposure to VZV in family settings. On the other hand, three other subjects with little or no VZV-specific proliferative activity in the tonsillar lymphocytes developed disease after similar exposure to VZV. These observations suggest the development of VZV-specific mucosal cellular immunity after overt or inapparent exposure to VZV. The appearance of such immunity appears to have a protective role against reinfection even in the absence of detectable serum antibody.     

Misdiagnosis of chronic thrombocytopenia in childhood

PURPOSE: Children ultimately diagnosed with nonimmune chronic thrombocytopenia are often referred to pediatric hematology clinics with a provisional diagnosis of autoimmune thrombocytopenic purpura (AITP). The authors' aim was to establish in these patients the features characterizing the mechanism of thrombocytopenia. PATIENTS AND METHODS: The authors performed a retrospective review of the case records of seven children (three boys and four girls, aged 5 months to 7 years) with misdiagnosed chronic AITP referred to a single pediatric hematology center between 1990 and 2000. RESULTS: In the seven children, the suspected diagnosis on referral was AITP and the final diagnosis was inherited thrombocytopenia. Abnormalities of platelets and/or leukocyte morphology were present in all of them. Other features suggestive of inherited thrombocytopenia included a history of familial thrombocytopenia (2/7), failure of steroids and/or intravenous immunoglobulins to raise the platelet count to normal levels (5/7), and moderate increase of Indium-111 platelet turnover in the two patients tested. Platelet-associated IgG (PaIgG) was above the normal threshold in the four children tested; the direct monoclonal antibody immobilization of platelet antigens (MAIPA) test was negative in the four children tested and the serum test was positive in two boys. Bone marrow examination revealed either a normal (4/7) or an elevated (3/7) number of megakaryocytes. CONCLUSIONS: Family history and blood cell morphology analysis in experienced hands are the first steps in discriminating AITP from inherited thrombocytopenia in children with isolated chronic thrombocytopenia. In contrast, bone marrow examination and search for specific autoantibodies using the MAIPA test are of little help. An isotopic platelet life span study, when available, should be performed before considering splenectomy to exclude the diagnosis of inherited thrombocytopenia, especially when steroids and/or IgG IV administration failed to raise the platelet count.     

Analysis of circulating T gamma/delta lymphocytes and CD16/56 cell populations in children and adolescents with Graves' disease

The aim of the present study was to determine the proportion of gamma/delta T-lymphocytes and CD16/CD56 (CD3- and CD3+) cells in the peripheral blood of children and adolescents with Graves' disease (GD; n = 27; mean age, 15.5 +/- 5.1 y) and nontoxic nodular goiter (NTNG; n = 25; mean age, 15.2 +/- 5.7 y), in comparison with sex- and age-matched healthy control subjects (n = 25; mean age, 15.9 +/- 2.4 y). In addition, in patients with GD, we investigated the effect of methimazole therapy on the proportion of these cells. We also looked for associations among the parameters investigated. The percentages of gamma/delta TCR+CD3+ lymphocytes and CD3+, CD16/56+CD3+, and natural killer (NK) cells were analyzed by the three-color flow cytometry using a Coulter EPICS XL cytometer. In patients with untreated GD, we observed a significant decrease in gamma/delta T (CD3+) (p < 0.002), CD16/56(CD3+) (p < 0.001), and NK (p < 0.001) cells in comparison with the healthy control subjects. After 2-6 mo of methimazole therapy, the percentages of gamma/delta TCR+CD3+ and CD16/56(CD3+) cells in peripheral blood of hyperthyroid patients returned to the normal values, whereas the percentages of NK cells normalized after 18-24 mo of therapy. These abnormalities were absent in children and adolescents with NTNG. Furthermore, there was no difference in the percentage of CD3+ lymphocytes in all of the groups. In the patients with untreated GD, we found a negative correlation between free thyroxine concentration in blood serum and the percentages of CD16/56 (CD3-) and gamma delta T cells (r = -0.5, p < 0.035; r = -0.4, p < 0.02). No such correlation was detected in patients with NTNG. We conclude that the abnormal distribution of CD16/CD56 (CD3- and CD3+) cells and gamma/delta T lymphocytes in the peripheral blood in children and adolescents with untreated GD suggests their role in the development of autoimmunity.     

Transfusion-acquired human immunodeficiency virus infection in twelve neonates: epidemiologic, clinical and immunologic features

Twelve neonates in 3 cohorts received blood transfusions from two donors who were infected with human immunodeficiency virus (HIV). All 12 infants developed laboratory and/or clinical evidence of HIV infection, usually in the first year of life. Ten of 12 infants had serum antibody to HIV when tested between 9 and 42 months of age. The two seronegative infants were severely hypogammaglobulinemic when they were tested. Nine infants developed a variety of illnesses attributable to HIV infection, but only 2 fulfilled criteria for the diagnosis of acquired immunodeficiency syndrome. In follow-up ranging from 2 1/2 to 4 years 5 patients (42%) have died. Four patients had HIV-associated illnesses but recovered and now have few if any symptoms attributable to HIV infection. Three children have never had signs or symptoms attributable to HIV. Immunologic abnormalities were present in all patients; the most consistent finding was a decrease in the proportion of T helper cells. Three patients had severe panhypogammaglobulinemia. The hypogammaglobulinemic infants had significantly lower numbers and percentages of T helper cells compared to the remaining patients (P less than 0.01). We conclude that exposure to HIV via transfusion in the neonatal period results in an extremely high rate of infection with substantial mortality and morbidity, but clinical recovery occurs in some patients. Also hypogammaglobulinemia may be more common in infants with HIV infection than previously appreciated.     

Danaparoid sodium (Orgaran) in four children with heparin-induced thrombocytopenia type II

We report on four children with heparin-induced thrombocytopenia type II. In three patients, therapy with unfractionated heparin was associated with development of cardiac thrombi or with thrombosis progression up to the inferior vena cava or with aggravation of peripheral arterial occlusion. In the fourth child, the disease was recognized early on, and no complication occurred. Heparin-induced thrombocytopenia type II was confirmed by heparin-induced platelet activation assay and/or heparin/platelet factor 4-ELISA. Concomitant elevated antiphospholipid antibodies were seen in all patients. Danaparoid sodium applied at a dosage of between 1.2 and 7.1 U/kg/h stopped the disease progression in each patient. Three children had a clinical recovery with partial recanalization, but for the child with peripheral arterial occlusion disease, amputation of some of the toes became necessary. Conclusion: Our data indicate that heparin-induced thrombocytopenia type II is a potential lifethreatening disease in children and danaparoid sodium is beneficial in this age group.     

Immunological spectrum of childhood tuberculosis.

Fifty-three children with tuberculous meningitis (TBM) were studied in parallel with 37 children with primary pulmonary complex (PPC), 32 tuberculin skin positive controls, and 38 skin negative controls for immunological evaluation. Proportions of peripheral blood total T and T helper lymphocytes (TH) were significantly reduced in TBM (T55, TH32) as compared to PPC (T70, TH40) and Controls (T74, TH43), but response to mitogens phytohaemagglutinin (PHA) and poke weed mitogen (Pwm) were comparable in all the groups. Response of peripheral blood lymphocytes to tubercular protein (PPD) was significantly greater in the tuberculin skin positive group, but comparable between the TBM and PPC groups. There was no significant difference in the proportion of B lymphocytes, antibody levels to PPD and monocyte capacity to release hydrogen peroxide in the four groups. These findings suggest that there is no definite immunological spectrum in childhood tuberculosis.     

抗胸腺细胞球蛋白治疗儿童再生障碍性贫血的临床研究

目的 总结以抗胸腺细胞球蛋白(ATG)为主的免疫抑制疗法治疗儿童再生障碍性贫血时,ATG的实施方法、不良反应防治和长期随访措施等与远期疗效的相关性.方法 儿童再生障碍性贫血35例,其中极重型再生障碍性贫血(VSAA)6例,急性再生障碍性贫血(SAA)11例,慢性重型SAA8例,中型再生障碍性贫血(MAA)10例.ATG治疗期间措施包括:治疗前清除感染灶;积极防治过敏反应;密切观察和处理因ATG相关血小板减少所致严重出血和免疫抑制所致严重感染;积极防治血清病;重视长期随访中的治疗措施与质量.结果 ATG治疗后,所有病例均出现60%以上的淋巴细胞绝对计数下降.平均随访28个月,总有效率为77.14%(27/35),显效率为57.14%(20/35).VSAA、SAA和MAA间疗效无明显差异.ATG不良反应观察结果:①48.6%出现轻度类过敏反应;②血清病发生率42.9%,平均病程3.6 d;③9例(25.7%)外周血小板(BPC)计数＜10×109/L;④8例(22.9%)在ATG治疗后1个月内发生感染;⑤未发生ATG治疗相关死亡.Genzyme和Fresenius两种不同ATG制剂,在疗效和ATG相关不良反应发生率方面差异均无统计学意义.结论 ATG治疗儿童SAA和MAA疗效显著,但需积极预防和控制ATG不良反应,避免治疗相关死亡.长期辅助治疗和提高随访质量,也是确保疗效的重要环节.

Abstract：

Objective To evaluate the efficacy of antithymocyte globulin (ATG) based immunosupression therapy for childhood aplastic anemia, to reduce the adverse effects and to observe the long-term outcome. Method Thirty-five children with aplastic anemia (AA) were enrolled in this study.Six of the cases had very severe AA (VSAA), 11 had severe AA (SAA)-Ⅰ, 8 had SAA-Ⅱ and 10 had moderate AA (MAA). All these patients were treated with ATG plus Cyclosporin A (CSA). The following measures were taken during the ATG therapy: infection of the patients had been controlled before ATG treatment. Comprehensive anti-allergic measures were implemented. Close attention was paid to the hemorrhage related with platelet reduction caused by ATG and severe infection of the patients. Result Shortly after the ATG usage, all the patients had a significant decrease of absolute peripheral lymphoblast count by more than 60 percent. With a mean follow-up time of 28 months, the total effective rate was 77.14％ ( 27/35), significant response rate was 57.14％(20/35). There was no significant difference among VSAA, SAA and MAA groups in the response rate. Adverse reactions included the following:① 48.6％ (17/35) patients presented mild anaphylactoid reaction during the first day of ATG treatment; ②42.9％(15/35) cases presented serum sickness 5-11 days after the last dose of ATG with a mean duration of 3. 6 days, all the patients were cured effectively with methylprednisolone; ③25.7％ (9/35)patient's peripheral blood platelet count was reduced, might be caused by ATG, to below 10 × 109/L, but no patient had severe hemorrhagic complication after platelet transfusion was performed; ④22.9％ ( 8/35 ) of patients got infection within a month after ATG therapy, including 3 cases with clinical septicemia, all the 3 cases recovered after antibiotics treatment. There was no ATG treatment-related death in this series. Conclusion ATG is a very effective therapy for children with SAA and MAA. Comprehensive measures are needed to prevent and handle the side effects to avoid treatment-related death. Long-term supportive therapy and proper follow up contribute to the favourable outcomes of the patients.     

Profile of dengue patients admitted to a tertiary care hospital in Mumbai

Dengue is a mosquito-borne arboviral infection that has become a public health concern in India and particularly Mumbai, where endemicity is on the rise. Fifty-seven children having dengue infection admitted over 12 months (2008) in a child health unit of a teaching hospital in Mumbai and who were positive for IgM antibodies by ELISA test were retrospectively studied for clinical profile and outcome. Common clinical findings were fever (100%), hepatomegaly (66.6%), vomiting (40.3%), and significant bleeding manifestations (38.5%). Common laboratory findings were thrombocytopenia (platelet < 100,000/mm3 in 96.5% patients), increased liver enzymes (59.6%), hypoalbuminemia (50.8%), hyponatremia (40.3%), and deranged prothrombin time/partial thromboplastin time (PT/PTT) (33.3%). Third spacing in the form of ascites and pleural effusion was present in 15.7% and 31.5% of patients, respectively. There was no correlation between platelet count and bleeding manifestation. Patients with dengue shock syndrome required more supportive therapy with blood products and inotropes and had a longer recovery time. Mortality in the study was 3.5%. PT/PTT, serum sodium, albumin, and white blood cell (WBC) counts were predictors of severity of dengue. To summarize, fever, hemorrhagic manifestations, hepatomegaly, thrombocytopenia, and evidence of plasma leakage (hemoconcentration, pleural effusion, ascites or hypoproteinemia) should lead a clinician to suspect dengue infection.     

裂隙淋巴细胞辅助诊断儿童百日咳的临床价值

目的 探讨外周血涂片裂隙淋巴细胞对辅助早期诊断百日咳的意义。方法 采集107例疑似百日咳患儿的鼻咽拭子和外周血。采用PCR-流式荧光杂交法进行百日咳杆菌核酸检测,根据结果分为百日咳组（n=52）和非百日咳组（n=55）;百日咳组按年龄分为<1岁组（n=42）和≥1岁组（n=10）,按病情严重程度分为轻症组（n=45）和重症组（n=7）。采用全自动血细胞分析仪对各组患儿进行外周血细胞计数测定,血涂片后行瑞氏染色和过氧化物酶染色,显微镜下观察各组患儿裂隙淋巴细胞并计数。结果 裂隙淋巴细胞呈圆形,胞体较小,胞浆量少,胞核上有切迹或裂缝;过氧化物酶染色为阴性,细胞学分类属淋巴细胞。百日咳组患儿白细胞计数、淋巴细胞比例、血小板计数、裂隙淋巴细胞比例均明显高于非百日咳组（P < 0.001）。百日咳组患儿中,<1岁组患儿淋巴细胞比例、血小板计数、裂隙淋巴细胞比例均明显高于≥1岁组患儿（P < 0.05）;重症组患儿上述血细胞计数及细胞比例均高于轻症组,但差异无统计学意义（P > 0.05）。结论 裂隙淋巴细胞联合外周血细胞计数可为百日咳患儿的早期诊断提供新思路。     

Anti-HPA-3A induces severe neonatal alloimmune thrombocytopenia

OBJECTIVE: Fetal and neonatal alloimmune thrombocytopenia (AIT) caused by feto-maternal incompatibility at the HPA-1a (PLA-1) locus is well characterized. Alloimmunization and disease caused by HPA-3a is rare. STUDY DESIGN: We conducted a retrospective analysis of all known cases of AIT caused by HPA-3a incompatibility identified at 3 major reference laboratories from 1986 to 1996. Platelet antigen typing and antibody specificity were determined by serologic evaluation. In some cases confirmatory genotyping was performed. RESULTS: Fourteen cases of anti-HPA-3a-induced AIT in 11 families were identified. Five patients had a previous affected sibling, and 2 cases were firstborn children. All patients had severe thrombocytopenia at birth (platelet count <20 x 10(9)/L). Regardless of therapy, the median time to platelet recovery was 6 days (range, 3 to 23 days). Two (15%) patients had documented intracranial hemorrhage, 1 with severe sequelae including apnea and convulsions. A literature review describing 16 additional patients corroborates the finding of severe thrombocytopenia and a significant incidence of intracranial hemorrhage caused by HPA-3a incompatibility. CONCLUSION: AIT caused by incompatibility of HPA-3a is similar in severity to disease caused by incompatibility of HPA-1a. Affected families should be appropriately counseled and considered for antenatal therapy.     

Epstein-Barr virus infection in a child with acquired immunodeficiency syndrome

A 3-year-old girl, born to an intravenous-drug-dependent mother, had protracted diarrhea, failure to thrive, generalized lymphadenopathy, and recurrent fevers during the first six months of life. At 7 months of age, the Epstein-Barr virus (EBV) genome was detected in her saliva by DNA dot-blot hybridization using a cloned EBV probe. Spontaneous EBV+ lymphoblastoid cell lines had repeatedly developed from her peripheral blood lymphocytes over the subsequent 2 1/2 years. At 11 months of age, persistent tachypnea and a diffuse pulmonary infiltrate developed. Lung biopsy demonstrated a florid, peribronchiolar lymphocytic infiltrate and the EBV genome was identified in the lung tissue. Serum anti-EBV antibodies remained undetectable until 14 months of age. She had a T4+/T8+ ratio of less than 0.8 and serum antibody to human T-cell lymphotropic virus type III. The delayed seroresponse of this patient to symptomatic EBV infection suggests that reliance on EBV serology to diagnose EBV infection in immunocompromised hosts may be inappropriate, and other methods such as DNA probes should be used.     

Dysfunction of the monocyte-macrophage system in the idiopathic minimal change nephrotic syndrome

We studied the function of phagocytes and the distribution of lymphocyte subpopulations in 23 patients with Idiopathic Minimal Change Nephrotic Syndrome. All the patients were in relapse at the time of the study. The latter was performed before specific therapy was started. Our control group consisted of 26 normal children who were studied while undergoing routine analysis prior to plastic surgery. Polymorphonuclear leukocytes from the patients showed no alterations in their ability to ingest and to kill candidas. On the contrary, peripheral blood monocytes had a normal phagocytic function with a decreased candidacidal activity when compared to normal controls (p less than 0.001). No correlation was found between serum immunoglobulin levels and the monocyte lytic function. The absolute number of B lymphocytes was significantly increased (p less than 0.05), whereas the absolute number of total lymphocytes, T lymphocytes and T4+ and T8+ cell subsets did not differ from those of the age-matched normal controls. Natural killer cells were functionally normal.     

Large granular lymphocyte leukemia (LGL) in a child with hyper IgM syndrome and autoimmune hemolytic anemia

We describe a female with a history of autosomal recessive hyper-IgM (HIGM) syndrome along with a history of autoimmune hemolytic anemia and intermittent lymphadenopathy. She subsequently developed neutropenia, lymphocyostosis and mild thrombocytopenia. Flow cytometry of the peripheral blood revealed the presence of a marked predominance of cytotoxic T lymphocytes, shown to be clonal, with concomitant natural killer (NK) antigen expression. She responded to weekly methotrexate therapy.